What percentage of symptomatic ICH occurs after IV TPA?
5-6%
What is the risk of asymptomatic ICH with intra-arterial thrombolysis?
33%
What is the mechanism of action of mirtazapine?
Presynaptic alpha-2 antagonist, postsynaptic antagonist at 5HT2, 5HT3, and H1 receptors
What are the etiologies of lateral periodic discharges?
Stroke, intracranial mass, subdural hematoma, encephalitis
What is sodium oxybate used for?
Reduce excessive sleepiness in narcolepsy and cataplexy
What is sodium oxybate?
A sodium salt of gamma hydroxybutyrate
What lab test is used for cerebrotendinous xanthomatosis?
Serum cholestanol
How many hypomelanotic nodules are needed for major criteria for TSC?
3 or more
How many angioblastomas are needed for major criteria for TSC?
3 or more
How many ungual fibromas are needed for major criteria for TSC?
2 or more
What are lab findings for narcolepsy?
Hypothalamic dysfunction with decreased CSF levels of orexins
What disease is characterized by 'peroxisomal ghosts'?
Zellweger syndrome
What is not recommended for treating fibromyalgia?
Alternate hot/cold therapy
What are the 3 major groups of cortical development?
Group 1 - abnormal cell proliferation or apoptosis; Group 2 - defects in cell migration; Group 3 - development defects after cell migration
Where is norepinephrine made?
Locus ceruleus
Where is dopamine made?
Substantia nigra, ventral tegmental area
Where is histamine made?
Tuberomammillary nucleus
Where is acetylcholine made?
Nucleus basalis of Meynert
Where is serotonin made?
Raphe nuclei
What is negative punishment?
Removal of desirable stimulus to decrease specific behavior
What is positive reinforcement?
Addition of desirable stimulus to increase specific behavior
What is positive punishment?
Undesirable stimulus to reduce a specific behavior
What is negative reinforcement?
Removal of undesirable stimulus to increase a specific behavior
What is extinction in behavior?
Loss of previously learned operant response after it is no longer reinforced
What are findings in S1 radiculopathy?
Lateral foot paresthesia and diminished Achilles reflex
What causes foot dorsiflexion?
Tibialis anterior, innervated by deep peroneal nerve (L5/L5)
What causes knee extension?
Quadriceps, innervated by femoral nerve (L2, L3, L4)
What causes foot inversion?
Tibialis posterior, innervated by tibial nerve (L4-5)
What can you see with 'string of pearls'?
Fibromuscular dysplasia
What occurs in myoclonic-astatic epilepsy?
Myoclonic jerks followed by atonic seizures; neurocognitive development usually normal prior to seizures
What do hydroxyamphetamine 1% eye drops do?
Localize Horner's syndrome
What is the pharmacological function of barbiturates?
Increased duration of GABA Cl- channel opening
What do barbiturates do for the brain?
Decrease cerebral metabolic activity, blood flow, and intracranial pressure
What is the pharmacological function of benzodiazepines?
Increase frequency of GABA Cl- channel opening
What is paroxysmal torticollis?
Benign intermittent self-limited episodes of head tilting lasting hours to days
What does dissection of the internal carotid artery cause?
Symptoms related to carotid artery disruption
What syndrome is characterized by excruciating headache and facial pain?
Horner’s syndrome
What layer does the dissection of the internal carotid artery affect?
Intimal layer
What are common triggers for RCVS?
Triptans, SSRIs, marijuana
What MRI findings are associated with RCVS?
Hypoperfusion in watershed regions, classical dot sign
What does prolonged hyperkalemia cause?
Partial depolarization, decreased nerve excitability
What does CMV cause in immunocompromised patients?
Peripheral neuropathy, cauda equina syndrome
What are mitochondrial encephalomyopathies due to?
Reduced oxidative metabolism
What is the term for inability to identify faces?
Prosopagnosia
What is central achromatopsia?
Inability to recognize color
What is Anton’s syndrome?
Inability to identify one's own blindness
Where do brain metastases tend to go?
Gray-white matter junction
What is Pendred syndrome?
Sensorineural hearing loss, goiter, enlarged vestibular aqueducts
What gene causes Pendred syndrome?
SLC26A4
What is the most common initial manifestation in neurocysticercosis?
Seizure
What cardiac issue is associated with DMD?
Supraventricular arrhythmia, conduction block
What neuropathy does CIDP cause?
Tingling/sensory loss in a stocking-glove distribution
What causes affective motor aprosodia?
Lesion to non-dominant frontal lobe
What is aprosodia?
Inability to express nonaffective or affective intonations
What causes receptive aprosodia?
Nondominant posterosuperior temporal gyrus lesion
What is Wernicke's aphasia associated with?
Posterosuperior temporal gyrus lesion
What does a lesion of the mesial frontal lobe cause?
Transcortical motor aphasia
What is thoracoabdominal polyradiculopathy?
Nonradicular truncal pain, abdominal bulging, sensorimotor changes
Where is the artery of Adamkiewicz located?
Between T8-L1 on left side of aorta
What are endocrine causes of increased intracranial pressure?
Hypoparathyroidism, Addison's, Cushing's, pregnancy
What antibody is associated with paraneoplastic cerebellar degeneration?
Anti-yo antibody
What does anti-Ma1 and anti-Ma2 cause?
Limbic encephalitis, brainstem encephalopathy
Who is usually affected by subcortical heterotopia?
Girls
What is a somatic mutation?
Not inherited, arises from mutation in body's cells
What does DCX mutation cause?
X-linked mutation affecting girls
What causes subcortical band heterotopia?
Inappropriate neuronal migration during fetal development
What artery supplies the pituitary?
Internal carotid artery branches
What do vertebral arteries supply?
Spinal cord, brainstem, cerebellum
What does early peripheral polyneuropathy cause?
Dysfunction in small peripheral nerve fibers
How does anti-LGI1 present?
Memory difficulty, myoclonus, faciobrachial dystonic seizures
Can you have MS with only optic neuritis?
Need dissemination in time evidence
What is memantine's mechanism of action?
Mild-moderate antagonist of NMDA receptors
Can you have MS with only optic neuritis?
Yes
What is an example of dissemination in time for MS?
Abnormal MRI, oligoclonal bands
What is memantine's mechanism of action?
Mild-moderate antagonist of NMDA receptors
What helps with REM sleep atonia?
High doses of melatonin at bedtime
In what condition is alien limb seen?
Corticobasal degeneration
Percentage of females with specific learning disorder in reading?
60-80%
What is the treatment for trigeminal neuralgia?
Carbamazepine
What percentage of children experience sleepwalking?
15%
At what age is sleepwalking greatest?
11-12 years old
Where does ocular bobbing localize?
Pons
Where does convergence retraction nystagmus localize?
Dorsal midbrain
Where does downbeat nystagmus localize?
Cervicomedullary junction (Chiari 1)
What artery supplies the thalamus?
PCA
What does damage to the postcentral gyrus cause?
Loss of proprioception, astereognosis, loss of vibratory sense, loss of 2-point discrimination
What is lamotrigine's mechanism of action?
Block Na and Ca channels
What is Keppra's mechanism of action?
Bind SV2A, decrease Ca influx, affect GABA
What is topiramate's mechanism of action?
Block Na and Ca channels, enhance GABA
What is perampanel's mechanism of action?
AMPA glutamate receptor antagonist
What drug causes nystagmus and AMS?
PCP
What condition is associated with cavernous sinus infection and mucormycosis?
Diabetic patients
When should aspirin be avoided?
Samter’s triad (asthma, nasal polyps, urticaria)
Who is most affected by Reye syndrome?
Children/teens recovering from chickenpox or flu-like symptoms
What are signs of Reye syndrome?
Confusion, seizures, loss of consciousness
What percentage of patients with Down syndrome have autism?
7%
Why do patients with Down syndrome develop Alzheimer's?
Increased expression of beta amyloid precursor protein
What genetic disorder is holoprosencephaly most common in?
Trisomy 13
What variant is tumefactive giant perivascular spaces of the cerebral white matter?
Benign variant
What is mild intellectual disability range?
55-70
What is severe intellectual disability range?
25-40
What is the mainstay treatment for primary CNS lymphoma?
High dose methotrexate
When is temozolomide used?
Glioblastoma after resection and chemo
When is bevacizumab used?
Recurrent glioblastoma
What part of the trigeminal nerve is common for HSV-1 latency?
V3 (mandibular division)
What is the next step for lithium poisoning with severe symptoms?
Emergency hemodialysis
What infantile spasms have the best prognosis?
Cryptogenic infantile spasms
What is the workup for infantile spasms?
MRI brain, genetics, metabolic, LP
What are positive predictors for infantile spasms?
Diagnosis over age 4 months, short time from onset to treatment, early response to treatment
Does mirtazapine affect weight?
Yes, can cause weight gain
What is delayed sleep phase disorder?
Fall asleep by 2-6am, wake up between 10-1pm
What is advanced sleep phase disorder?
Fall asleep between 6-9pm, wake up between 2-5am
What is hereditary hemorrhagic telangiectasia associated with?
AVMs
What neurological issues are associated with Ehlers Danlos?
Intracranial aneurysms, carotid-cavernous fistulas, arterial dissection
What describes partial complex seizures?
Behavioral arrest, staring spell, automatism
What supplies the lateral geniculate nucleus?
Cistern portion of anterior choroidal artery
What are assimilation and accommodation?
Piaget's concepts of how individuals create understanding
What supplies the lateral geniculate nucleus and lateral thalamus?
CISTERNAL portion of anterior choroidal artery
What is assimilation?
How individuals take in new information and incorporate it into existing ideas
What is accommodation?
Reinterpreting a new experience to fit with previously acquired information
What are the baby temperaments according to Chest and Thomas?
Easy, slow to warm up, difficult
What is 'easy' temperament in babies?
40% of babies; calm and adaptable
What is 'slow to warm up' temperament in babies?
15% of babies; cautious but warms up
What is 'difficult' temperament in babies?
10% of babies; fussy and less adaptable
Where do brain teratomas usually occur?
Pineal, suprasellar, cerebellar regions; can go to lateral ventricles
What percentage of congenital brain tumors are intracranial teratomas?
50%
What conditions increase RLS?
Pregnancy, uremia, anemia, rheumatoid arthritis; increases with age
What disease is associated with partial visual seizures and cognitive decline?
Lafora disease
What is the most common form of progressive myoclonic epilepsy?
Unverricht-Lundborg
What is the treatment for Wilson's disease?
Penicillamine
What is the treatment for opioid overdose?
Naloxone
What is the most common complication of serotonin syndrome?
Rhabdomyolysis; myoglobin present
What tumors are associated with high AFP?
Yolk sac tumors
What tumors are associated with high Beta HCG?
Choriocarcinomas
What is growing teratoma syndrome?
Occurs in 21% of nongerminomatous germ cell tumors during treatment; requires surgery/radiation
What can a colloid cyst cause?
Obstructive hydrocephalus
What is central neurocytoma?
Lesion arising from septum pellucidum
What are transient motor tics?
Tics lasting less than 6 months; 10% of children experience tics
What is Tourette syndrome?
Motor and phonic tics lasting at least 12 months
What are chronic motor tics?
Tics lasting at least 6 months
When does absence epilepsy usually start?
Between 4-10 years of age
How long do absence episodes usually last?
Less than 15 seconds
What usually causes an epidural hematoma?
Trauma; 95% skull fractures, 85% tear of middle meningeal artery
Where does the middle meningeal artery travel through?
Foramen spinosum
What bug causes lethargy and rash in toxic shock syndrome?
Staphylococcus aureus
What does the piriform cortex contribute to?
Odor discrimination
What defective enzyme is associated with PKU?
Phenylalanine hydroxylase
What is the MOA for natalizumab?
Binds to alpha 4 subunit; decreases inflammation
What is the MOA of teriflunomide?
Inhibits dihydroorotate dehydrogenase; targets pyrimidine synthesis
What is the MOA for alemtuzumab?
Targets CD52 on T and B lymphocytes
What is the MOA for fingolimod?
Sphingosine-1-phosphate receptor blocker; prevents lymphocyte circulation into CNS
What is the MOA for ocrelizumab?
Targets CD20 (B cell marker)
Can optic gliomas decrease color vision?
Yes
What findings indicate central sleep apnea?
Repetitive cessation or decrease of both airflow and ventilatory effort during sleep
What findings indicate obstructive sleep apnea?
Repetitive cessation or decrease of airflow but NOT of ventilatory effort during sleep
What is Cheyne Stokes respiration?
Cyclic breathing pattern; apnea followed by increasing then decreasing respiratory frequency
What is hypopnea?
Abnormally slow or shallow breathing
What does mutation in HFE gene cause?
Hemochromatosis
Intracranial hypertension can cause injury to which nerve?
CN VI
What are the side effects of fingolimod?
Not specified in the text
What is shallow breathing?
A type of breathing that is not deep or full.
What does mutation in HFE gene cause?
Hemochromatosis
What nerve is affected by intracranial hypertension?
CN VI
What are side effects of fingolimod?
Bradycardia, macular edema
What causes lateral medullary syndrome?
PICA infarction
What are symptoms of lateral pontine syndrome?
Ipsi facial palsy, Ipsi hearing loss, vertigo, nystagmus, contra loss pain and temp, ipsi ataxia
What causes medial medullary syndrome?
Occlusion of perforating branches of the vertebral artery or basilar artery
What is Weber syndrome?
Ipsilateral oculomotor palsy and contralateral hemiparesis
What is ventral pontine syndrome (Millard-Gubler)?
Unilateral facial palsy, ipsi abducens palsy, contralateral hemiparesis
What causes VMA?
NE from locus coeruleus
What does unilateral lateral lemniscus infarction cause?
No auditory deficits due to bilateral representation
What is infantile neuroaxonal dystrophy?
Causes axonal swelling in cerebral and anterior horn cell gray matter
What is the similarity between ICA lesions and what artery?
MCA
What does occlusion of anterior choroidal artery lead to?
Lacunar infarct
What is a common gender for fibromyalgia?
Women
How does ADEM differ from MS?
Patients recover more rapidly from ADEM
What med is used if acyclovir is not available?
Ganciclovir
What is propriospinal myoclonus?
Sudden jerks affecting abdomen, neck, and trunk
What is periodic limb movement on polysomnography?
Leg movement >/= 8 uv in EMG lasting 0.5-10 seconds
What is Parsonage Turner syndrome?
Rapid onset of severe upper arm and shoulder girdle pain
What sensory area is usually affected in Parsonage Turner syndrome?
Lateral shoulder, arm, and or hand
What are risk factors for Parsonage Turners?
Viral infection, bacterial infections, immunizations, stress, meds, radiation
What is idiopathic hypertrophic brachial neuritis?
Usually painless hypertrophy of brachial plexus
What are MRI findings in chronically schizophrenic patients?
Reduced volume of prefrontal cortex, superior temporal gyrus
What is agoraphobia?
Fear of public places
What are hypnic jerks?
Jerks that occur after falling asleep
What is large fiber neuropathy?
Loss of distal proprioception and vibratory sensation
What is small fiber neuropathy?
Affects touch, pain, temperature
What is mononeuritis multiplex?
Asymmetric involvement of >/=2 named nerves
What is diabetic amyotrophy?
Pain, weakness in proximal muscles
What is distal symmetric diabetic sensory polyneuropathy?
A type of nerve damage related to diabetes.
What is mononeuritis multiplex?
Asymmetric involvement of >/=2 named nerves in separate parts of the body.
What is diabetic amyotrophy?
Pain and weakness and atrophy of the pelvifemoral muscles.
What can help with sialorrhea?
Botox A type A.
What endocrine disorders can lead to proximal myopathy?
Hyperparathyroidism, Hypothyroidism, Hyperthyroidism (CK usually normal).
What are the 5 stages of grief?
Denial, Anger, Bargaining, Depression, Acceptance.
What is a Hirano body?
Associated with Alzheimer's disease.
What are neurofibrillary tangles?
Pathological aggregates in neurons, associated with Alzheimer's disease.
What is amyloid angiopathy?
A condition characterized by the deposition of amyloid in the walls of blood vessels.
What is Chiari I malformation?
Displacement of cerebellar tonsils below foramen magnum.
What are symptoms of Chiari I malformation?
Occipital headache, Neck pain, Myelopathy, Gait abnormalities, Cranial neuropathy, Brainstem compression, Syringomyelia.
What is hydromyelia?
A cavity that results in enlargement of the central canal.
What is Chiari II malformation?
Myelomeningocele, Displacement of cerebellar tonsils, Inferior cerebellar vermis, Medulla into the upper cervical canal, May have hydrocephalus.
What is Chiari III malformation?
Downward displacement of cerebellum from a small posterior fossa into cervical or occipital encephalocele.
What ages are covered for IDEA?
Birth to 21 years.
What is the benign paroxysmal vertigo of childhood?
Migraine equivalent typically remits by age 5, Short episodes, Nystagmus, nausea, vomiting.
What is vestibular paroxysmia?
Very brief episodes of vertigo, may be caused by compression of vestibular nerve, usually responds to carbamazepine.
What can sevoflurane cause?
Malignant hyperthermia; remove offending agent and can give dantrolene if needed.
How do TCAs work for pain?
Enhance descending pain inhibition via increased serotonin and NE to inhibit nociceptive pathways.
What is the most common embolic stroke etiology in older patients?
Atrial fibrillation (A fib).
What can congenital aqueduct of Sylvius cause?
Hydrocephalus.
What scale can differentiate delirium from dementia?
Delirium rating scale-revised-98.
What are known side effects of topiramate?
Impaired cognition and kidney stones.
When do seizures with BECTS usually remit?
During puberty.
What is a side effect of clozapine?
Seizures (increases with increasing doses) and agranulocytosis.
What has a better prognosis for medulloblastoma?
Monosomy 6, Positive staining for beta catenin, Total or near total resection, Localized disease.
What has a worse prognosis for medulloblastoma?
MYC amplification, Subtotal resection, Anaplasia on pathology, Positive CSF cytology and disease dissemination.
What gene is implicated in congenital neuronal ceroid lipofuscinosis?
Ceroid lipofuscinosis neuronal 10 (CLN10).
What are symptoms for ceroid lipofuscinosis?
Neonatal period, Pass away in first week, Seizures, microcephaly, lack of reflexes.
What can be used for pediatric acute migraines in children 12 years and older?
Combo sumatriptan and naproxen.
Decreased taste sensation in Bell's palsy suggests involvement of?
Chorda tympani.
What may nitrous oxide inhalation unmask?
Vitamin B12 deficiency; can cause paresthesias in hands and feet.
What is Hoover’s sign?
Involuntary extension of the stationary leg when flexing the other leg; absence suggests conversion disorder.
What is somatic symptom disorder?
Presents with >/= 1 physical symptom causing disproportionate emotional distress.
When to consider antifibrinolytic therapy for delayed aneurysm?
If repair > 72 hours from onset is anticipated.
What causes emotional distress in patients?
Disproportionate degree of emotional distress
When to consider antifibrinolytic therapy for delayed aneurysm?
If repair > 72 hours from onset
When do vasospasms occur after SAH?
Usually peaks 7-8 days
What medication reduces the risk of vasospasm?
Nimodipine
What is cocaine's mechanism of action?
Inhibits dopamine removal from the synaptic cleft
What is cocaine classified as?
Dopamine reuptake inhibitor
What causes lacunar infarcts?
Small vessel hyalinosis due to uncontrolled hypertension
What causes a mixed sensorimotor stroke?
Lesion in the posterior limb of internal capsule
What is the most common side effect of clozapine?
Sedation
What is the medial geniculate nucleus involved in?
Hearing
What does a lesion to the right lateral geniculate body cause?
Left homonymous hemianopia
What is an example of a COMT inhibitor?
Entacapone
What is the mechanism of action of COMT inhibitors?
Prevents COMT from metabolizing L'DOPA into 3OMD
What is decorticate posturing?
Flexion of arms, feet extended turned inward
What causes decorticate posturing?
Disinhibition of the red nucleus
What does a lesion below the red nucleus cause?
Decerebrate posturing
What is kleptomania?
Impulse control to steal items
What are the 3 infant development stages by Margaret Mahler?
Autistic phase, Symbiotic phase, Separation individuation
What are the phases of separation individuation?
Hatching, Practicing, Rapprochement
How is Rett syndrome inherited?
X linked dominant
What are findings in cold water caloric testing?
Slow component toward ipsilateral side, fast component opposite
What to do if parents decline treatment in an emergency?
Must initiate treatment if life threatening
What indicates obstructive sleep apnea in children?
25% total sleep with PaCO2 >50 mmHg and child snores
What is the requirement for sleep-related hypoxemia?
Arterial oxygen saturation ≤ 90% for at least 5 minutes
What is the PaCO2 criteria for obesity hypoventilation syndrome?
PaCO2 during wakefulness > 45 mmHg
What paraneoplastic syndrome is associated with peripheral neuropathy?
Anti-Hu, ANNA-1
What paraneoplastic syndrome is associated with memory difficulty?
Anti-LG1 (VGKC ab)
What paraneoplastic syndrome is associated with cerebellar degeneration?
Anti-Yo ab or Purkinje cell cytoplasmic ab type 1
What is oculopharyngeal muscular dystrophy?
AD, late onset, ocular and pharyngeal involvement
What do parasympathetic fibers for CN III control?
Pupillary constriction
What are Betz cells?
Upper motor neurons, large cells in primary motor cortex
What are the 6 layers of the neocortex?
Molecular layer, External granular, External pyramidal, Internal granular, Internal pyramidal, Multiform layer
What cells are the largest number of cortical neurons?
Pyramidal cells
What cells function predominantly as cortical interneurons?
Granular or stellate cells
What cells give rise to efferents in cortical areas?
Amidal cells
What cells function predominantly as cortical interneurons?
Granular or stellate cells
What cells are found in higher numbers in the sensory cortex?
Granular cells
Variations in the SLCO1B1 gene affect susceptibility to what myopathy?
Statin myopathy
Patients with polymyositis have a risk of what cancer?
Non-Hodgkin lymphoma
Acute inflammatory demyelinating polyradiculopathy is also known as?
GBS
What vitamin deficiency causes inverted v sign?
Vitamin B12
What stage do children understand conservation of mass?
Concrete operations (Age 7-12)
What risk is associated with Dalfampridine?
Seizures (increased risk with renal impairment)
For what purpose is Dalfampridine used?
Increase walking speeds in MS (K channel blockade)
Which cranial nerve is not part of the PNS?
CN II
What are the boxed warnings for valproic acid?
Teratogenicity, Pancreatitis, Hepatotoxicity
What phenomenon causes transient blurring of vision in MS patients?
Uhthoff phenomenon
What is the Pulfrich phenomenon?
Visual illusion in MS; moving object appears to travel in an elliptical orbit
What is the genetic cause for NARP?
Heteroplasmic mutation (70-90% leads to NARP)
What type of destruction of ACH receptors occurs in MG?
Complement-mediated
What are symptoms of paroxysmal hemicrania?
Pain 20-30 min, >5x/day, triggered by alcohol, unilateral
What is the treatment for paroxysmal hemicrania?
Indomethacin
What side effect is associated with lithium?
Hyperparathyroidism
Where are subclinical rhythmic electrographic discharges maximal?
Parietal or posterior temporal regions
What are the diagnostic criteria for CIDP?
1) Progressive or relapsing dysfunction of >1 limb, 2) Develops over 8 weeks, 3) Areflexia or hyporeflexia
How to test for C. botulinum?
Stool culture
What do fragile X sperm cells carry?
Premutation alleles
What MRI findings are seen in fragile X tremor ataxia syndrome?
Hyperintensities in cerebellum, corpus callosum, middle cerebellar peduncle on T2
What supplies sensation to the lower lip?
Inferior alveolar nerve
What are symptoms of hereditary sensory and autonomic neuropathy type 2?
AR, loss of pain/temp/touch/pressure, recurrent infections, fractures
What is the treatment for optic neuritis refractory to steroids?
Plasmapheresis or plasma exchange
What treatment supports nocturnal hypoventilation due to neuromuscular disease?
Bilevel (spontaneously timed mode)
What is the equilibrium potential for potassium in a neuron?
-102 mv
What is the resting membrane potential in a neuron?
-60 mv
What is the equilibrium potential of sodium in a neuron?
+56 mv
Buspirone works primarily on which receptor?
5HT1A partial agonist
What is a good prognostic sign for oligodendroglioma?
1p/19q co-deletion
What is the most common finding in chronic lead poisoning in children?
Neuropsych symptoms
What is the effect of chronic lead poisoning in adults?
Motor neuropathy
What causes migraine aura?
Depolarization of neuronal cells at 2-5 mm/min
What are signs of chronic exposure to organic mercury?
Ataxia, paresthesias, visual field defects
Which areas are vulnerable to loss of oxygen?
CA1 region of hippocampus, cortical pyramidal neurons, cerebellar purkinje cells, brainstem nuclei
What is not a side effect of amitriptyline?
Hypertension (causes orthostatic hypotension)
What imaging is used for ipsilateral Horner syndrome?
MRI
What are cerebellar purkinje cells?
Type of neurons in the cerebellum
What brain regions are involved with neurons?
Brainstem nuclei, thalamus, striatum, amygdala
What is not a side effect of amitriptyline?
Hypertension
What imaging is used for ipsilateral Horner syndrome with severe headache?
MRA head and neck
When does trust vs. mistrust occur?
Birth to 18 months
What type of neuropathy is diabetic lumbosacral radiculoplexus neuropathy?
Vasculitic axonal neuropathy
What are the symptoms for inclusion body myositis?
Weak wrist and finger flexors, weak quadriceps, weak anterior tibial muscles
What is a symptom of dysphagia in inclusion body myositis?
Dysphagia 1/3rd
What enzyme converts tyrosine to L-DOPA?
Tyrosine hydroxylase
What enzyme converts L-DOPA to dopamine?
DOPA-decarboxylase
What enzyme converts dopamine to norepinephrine?
Dopamine B-hydroxylase
What enzyme converts intracellular dopamine to HVA?
Monoamine oxidase
What to do for augmentation to ropinirole?
Switch to another med with different MOA
What is diabetic neuropathic cachexia?
Painful, acute, sensory, motor, autonomic, often bilateral thighs, severe weight loss
What is the pathology of palatal myoclonus?
Mostly brainstem lesions of central tegmental tract or dentate-olivary pathways
What mineral can be low in Wernicke's besides thiamine?
Magnesium
Order of thoracolumbar spinal injuries by increasing severity?
Compression fracture -> Burst fracture -> Translational/rotational -> Distraction
What is a case control study?
Analyze a known outcome by looking back in time to assess exposure
What is a cohort study?
Follow people over time to determine risk of disease
What is a cross-sectional study?
Analyze population at a single point in time
What is a crossover study?
Longitudinal study, subjects receive different treatment
Which antidepressant to avoid in hypertension and acute closure glaucoma?
Venlafaxine
Which antidepressant to avoid in hepatic/renal impairment?
Duloxetine
Difference between hemorrhagic and ischemic strokes?
Hemorrhagic strokes cause severe headache, nausea, vomiting, decreased LOC
Findings in pineoblastoma?
Parinaud syndrome, supranuclear upgaze palsy, light near dissociation
What gene is associated with lissencephaly?
DCX gene
What WHO grade are SEGAs?
WHO grade 1 tumor
What is musculocutaneous neuropathy?
Weakness in flexion of forearm, absent biceps reflex, sensory loss on lateral forearm
What is a finding in the ophthalmic vein in carotid cavernous fistula?
Dilated superior ophthalmic vein by CT
What is the flow in carotid cavernous fistula?
High flow
Is cyclobenzaprine helpful for spasticity?
False
What does adrenoleukodystrophy lead to?
Build up of very long chain fatty acids
Inheritance pattern of adrenoleukodystrophy?
X linked
What nuclei provide parasympathetic innervation?
Edinger Westphal nucleus, Superior salivatory nucleus
What helps decrease the possibility of stroke after SAH?
Nimodipine
What is palatal myoclonus due to?
Spasms of tensor veli palatini
What causes objective tinnitus?
Vascular issues
Can aspirin overdose cause tinnitus?
Yes, bilateral subjective tinnitus
What is the MOA of sumatriptan?
5HT1B agonist and 5HT1D agonist
What does spelling 'world' test?
Attention
What do clock drawing tests assess?
Executive function
What do recalling objects tests assess?
Recall
What is paramyotonia congenita?
A genetic neuromuscular disorder
What do spelling 'world' tests assess?
Attention
What do clock drawing tests evaluate?
Executive function
What do recalling objects tests measure?
Recall
What mutation causes paramyotonia congenita?
AD Na channel mutation
What is the AR neurodegenerative disorder in early childhood?
Giant axonal neuropathy
What characterizes multifocal acquired demyelinating neuropathy?
Asymmetric motor and sensory loss with conduction block
What is a symptom of Stage II Lyme disease?
Bilateral facial palsy
What are symptoms of Behcet's disease?
Recurrent oral and genital ulcers, ophthalmic and skin lesions
What CNS findings are associated with Behcet's disease?
Relapsing, remitting aseptic meningoencephalitis
What nerve is damaged by midshaft humerus injury?
Radial nerve
What nerve is affected by medial epicondyle injury?
Ulnar nerve
What nerve is affected by distal end of humerus injury?
Median nerve
What is spondylosis?
Nonspecific degeneration of spinal column
What is spondylitis?
Inflammation of vertebrae
What is a treatment for chronic insomnia?
Good sleep hygiene, CBT, Melatonin
Do most patients with juvenile absence epilepsy experience GTC?
True
Is twitching a side effect of pyridostigmine?
True
What are neuro findings in Sturge-Weber?
Meningeal angiomas, hydrocephalus, tram tracking calcifications
Is a patient unable to maintain fixation upon rapid head turn to the left in vestibular neuritis?
True
What does a brain biopsy in PML show?
Oligodendrocyte enlargement with glassy magenta stain
Where does the mandibular nerve enter?
Foramen ovale
What does Rinne test indicate in conductive hearing loss?
Bone > air
What is the genetics of spinobulbar muscular atrophy?
X-linked, CAG
What are findings in spinobulbar muscular atrophy?
Progressive weakness and atrophy in facial, bulbar, limb muscles
What are EEG findings in HSV encephalitis?
PLEDS
When to insert an intraventricular catheter for elevated ICP?
Comatose (<8), measure ICP and relieve it
What is the Marburg variant of MS?
Large acute tumor-like demyelinating lesions in one hemisphere
What does WNT indicate in medulloblastoma?
Good prognosis
What are findings for granulovacuolar degeneration?
Seen in hippocampal neurons in Alzheimer's disease
What nerve injury occurs at the shaft of the humerus?
Radial nerve injury
What nerve is affected at the neck of the humerus?
Axillary nerve
What is the ventrolateral preoptic nucleus involved in?
Wakefulness and sleep
What is orthostatic tremor?
Postural tremor occurs when standing
What lies anterior to the spinal cord?
Posterior longitudinal ligament
What mutation causes myotonic dystrophy type 1?
DMPK gene
What mutation causes myotonic dystrophy type 2?
CNBP gene
Where do most motor fibers in the brachial plexus originate?
C5-C6
What are symptoms of fibromyalgia?
Tingling, muscle spasms, weakness, pain, sleep disturbances, fatigue
What is the GEFS+ gene?
SCN1A, AD
What is a prophylactic medication for cluster headaches?
Verapamil
What bug causes Lyme disease?
Borrelia burgdorferi
What medications are contraindicated for migraine in CAD?
Triptans, ergots, NSAIDs
What can be used instead of contraindicated meds for migraine in CAD?
Intranasal lidocaine
What is required by McDonald criteria for MS diagnosis?
1 lesion (spinal cord and left eye) with enhancing
What is contraindicated in CAD?
Triptans, ergots, NSAIDs
What can be used instead in CAD?
Intranasal lidocaine
What are the McDonald criteria for MS?
1 lesion (spinal cord and left eye), enhancing and non-enhancing lesions on brain MRI
What is the marker for thymoma in MG?
Anti-striated muscle antibodies
What is the most common acute hepatic porphyria?
Acute intermittent porphyria
What enzyme is associated with acute intermittent porphyria?
Porphobilinogen deaminase
What causes left neglect?
Right parietal lobe lesion
What does carbidopa inhibit?
Peripheral conversion of levodopa to dopamine
What is the BMI threshold for anorexia?
< 18.5
What are symptoms of a lower trunk lesion?
Excessive arm abduction, fall from height, loss of sensation medial forearm, weakness of intrinsic hand muscles
What can neurosyphilis lead to?
General paresis
What are bipolar findings in neuroimaging?
Increased intensity in frontal white matter, decreased volume right caudate, increased volume in hippocampus
What are A waves in ICP monitoring?
Pathologic, plateau waves, loss of intracranial compliance, elevations into 30-50 mmHg
What are B waves in ICP monitoring?
May be related to respiratory cycle
What are C waves in ICP monitoring?
Purely physiological, related to cardiac cycle
What is the most superficially prominent cervical vertebra?
C7
What is the toxicity of nitrous oxide?
Inactivates cobalamin (vitamin B12), causes sensory neuropathy, myelopathy, encephalopathy
What is the REM latency in GAD?
Normal REM latency
What is the REM latency in depression?
Reduced REM latency
What is porencephaly?
Not lined by gray matter
What is the mechanism of action of Galantamine?
Reversible acetylcholinesterase inhibitor, nicotinic Ach receptor modulator
What is the genetic cause of Dravet syndrome?
SCN1A mutation
What is the most common incomplete syndrome from traumatic spinal cord injury?
Central cord syndrome
What are symptoms of supranuclear vertical gaze palsy?
Myoclonic jerks, pendular vergence ocular movements, concurrent masticatory muscle contraction
What organism is associated with Whipple's disease?
Tropheryma whipplei
What structures provide input to the caudate?
Cortex, thalamus, substantia nigra pars compacta
What type of imaging is associated with choroid plexus papilloma?
MRI findings
What is the pathogenesis of Tourette's syndrome?
Hyperdopaminergic stimulation of limbic system and ventral striatum
What is the risk of tardive dyskinesia with risperidone?
Higher rate for atypical antipsychotics
What indicates a positive prognosis for conversion disorder?
Acceptance of diagnosis, short duration of symptoms
What are symptoms of Meige syndrome?
Forced blinking, tongue contractions, tongue spasms
What type of drug is LSD?
Serotonin agonist
Which chromosome is associated with CJD?
Chromosome 20
What is a side effect of fingolimod?
Macular edema
What can occur in left temporal lobe epilepsy?
Hypergraphia, hyposexuality, intense emotional responses, hyperreligiosity, hypermorality
What is the genetic cause of Duchenne muscular dystrophy?
Recessive X-linked gene
What are symptoms of perilymphatic fistula?
Fluctuating vertigo, hearing loss, often preceded by head injury or barotrauma, Tulio phenomenon
What does elevated PCO2 cause?
Cerebral vasodilation, increased cerebral blood flow
What does decreased PCO2 cause?
Cerebral vasoconstriction, decreased blood flow
What is the genetic cause of Prader-Willi syndrome?
Paternal deletion on chromosome 15
What is the ABCD1 gene mutation associated with?
Increased very long chain fatty acids, adrenoleukodystrophy
What does biotinidase deficiency cause?
Hearing and vision loss
What is the developmental origin of the hypothalamus?
Diencephalon
What is the developmental origin of the thalamus?
Diencephalon
What is the developmental origin of the limbic system?
Telencephalon
What is the developmental origin of the substantia nigra?
Mesencephalon
What is the developmental origin of the pons and cerebellum?
Metencephalon
What is the developmental origin of the myelencephalon?
Medulla
Which antipsychotics have the lowest risk of EPS?
Chlorpromazine and thioridazine
Which antipsychotics are more likely to cause EPS?
Haloperidol and fluphenazine
What are rimmed vacuoles associated with?
Intracellular B-amyloid deposition in vacuoles
What characterizes inclusion body myositis?
Painless weakness and atrophy in fingers
What drugs are more likely to cause EPS?
Haloperidol and fluphenazine
What is associated with intracellular B-amyloid deposition?
Rimmed vacuoles
What condition features painless weakness and atrophy in finger flexors?
Inclusion body myositis
Which brain area is targeted for DBS in seizures?
Anterior nucleus of thalamus
What lesion affects mood fluctuations?
Anterior and medial thalamus
What is the function of the anterior thalamic nucleus?
Memory storage and emotion
What inputs does the mediodorsal thalamic nucleus receive?
Temporal lobe and hypothalamus
What is impaired in Alzheimer's dementia?
Recent memory
What is the action of fluvoxamine?
Affects serotonin in raphe nuclei
What are the features of Cri du chat syndrome?
Mood facies, hypertelorism, low set ears, hypotonia, high pitched cry
What is a side effect of phenytoin?
Hirsutism
What are the features of Walker-Warburg syndrome?
Muscular dystrophy, cerebral and eye malformations, cobblestone lissencephaly
What does elevated acetylcholinesterase in amniotic fluid indicate?
Increased sensitivity/specificity of neural tube defect screening
What is the first line treatment for mania in pregnant bipolar disorder?
Haloperidol
What is the most common cause of spinal epidural abscess?
Staph aureus
What are the CNS manifestations of SLE?
Episodic psychiatric changes
Which SSRIs are FDA approved for PTSD?
Paroxetine and Sertraline
What does the anterior nucleus of hypothalamus regulate?
Body temperature
What initiates the heat maintenance reflex?
Posterior hypothalamus
What innervates the gluteus medius and tensor fascia lata?
Superior gluteal nerve
What is the mechanism of action of Topamax?
Blocks Na channels, inhibits carbonic anhydrase and glutamic acid, stimulates GABA-A
What are the symptoms of argininosuccinate lyase deficiency?
Vomiting, lethargy, seizures, coma, respiratory failure
What is trichorrhexis nodosa?
Brittle hair with nodules along hair shaft
What are the symptoms of arginase deficiency?
Spastic diplegia and dystonia
What happens with an infarct in the medial pons?
Contralateral weakness and dysarthria
What percentage of children with meningitis have seizures?
25%
What does St. John's Wort do to warfarin and digoxin levels?
Lowers levels
What is the most common form of cerebral palsy?
Spastic
What is Kearns-Sayre syndrome?
Muscle fibers with ragged red fibers
What is the mechanism of action of Memantine?
NMDA receptor antagonist
What is SAH associated with?
Polycystic kidney disease
What do reduced serum iron stores affect?
Dopamine-receptor function
What hematoma thickness requires surgery regardless of GCS?
10 mm
What midline shift requires surgery regardless of GCS?
5 mm
What are congenital disorders of glycosylation primarily?
Most AR
What are Lisch nodules associated with?
NF1
What does dermatomyositis EMG show?
Increased insertional activity, fibrillations, positive sharp waves, proximal weakness
What is zero order kinetics?
Constant amount of drug eliminated per set amount of time
What BMI is associated with bulimia nervosa?
Normal BMI or increased weight
What is critical illness polyneuropathy?
Inactivation of Na ion channels, low motor and sensory amplitudes on NCS
What is the mechanism of action of Selegiline?
MAO-B isoform
How is serotonin terminated?
By reuptake by 5HTT
What converts tryptophan to serotonin?
Tryptophan hydroxylase
What mutation is involved in congenital myasthenia gravis?
Nicotinic ACh receptor
What conditions are associated with mutations in CACNA1A?
Episodic ataxia 2, spinocerebellar ataxia type 6, childhood absence epilepsy
What is episodic ataxia 1?
Potassium channelopathy
What drugs can cause tinnitus?
Aspirin, quinine, furosemide, vancomycin, neomycin
What is leukocoria?
Initial feature in retinoblastoma
What is central core myopathy associated with?
Risk of malignant hyperthermia, muscle weakness
What is vancomycin?
An antibiotic used to treat bacterial infections.
What is neomycin?
An antibiotic used to prevent infections.
What is leukocoria?
An initial feature in retinoblastoma.
What is central core myopathy?
A condition with muscle weakness and risk of malignant hyperthermia.
What are the muscle fiber characteristics in central core myopathy?
Single or multiple central or slightly eccentric cores in at least 20% of muscle fibers.
What is benign myoclonic epilepsy of infancy?
A type of epilepsy characterized by myoclonic seizures in infants.
What does EEG show in benign myoclonic epilepsy of infancy?
Polyspike waves.
What should be done in case of anaphylaxis to natalizumab?
Do not use it again.
What is convergence-retraction nystagmus?
Not a true nystagmus.
When should a cervical spine radiograph be obtained in Down syndrome?
Only if symptoms of atlantoaxial subluxation develop.
How does the cortex develop?
Inside-out via cell migration.
What is the prevalence of migraine in children 7 and younger?
2.5%.
What percentage of headaches in young children are migraines?
75%.
What can anterior shoulder dislocation injure?
Axillary nerve.
What are the symptoms of axillary neuropathy?
Diminished sensation over lateral upper arm, loss of arm abduction, and weakness of deltoid and teres minor.
What is Lambert-Eaton myasthenic syndrome associated with?
Low CMAP and increment with high-frequency repetitive stimulation.
What increases the risk of SDH?
Chronic alcohol use disorder.
What are the features of congenital toxoplasmosis?
Hydrocephalus, chorioretinitis, and intracranial calcifications.
What is the characteristic of axonal loss peripheral neuropathy?
Low amplitude with preserved conduction velocity.
What is associated with Miller Fisher syndrome?
GQ1b antibodies.
What is Leber congenital amaurosis?
Retinal dystrophy and the most common genetic cause of congenital visual impairment.
What does the digit span test assess?
Immediate recall, more affected in acute psych disorders than early neurodegenerative disease.
What does routine screening for dementia include?
Depression screening, BUN, creatinine, electrolytes, glucose, LFTs, thyroid, B12, CBC.
What are common associations with carpal tunnel syndrome?
Diabetes, amyloidosis, thyroid disease.
What are spinocerebellar ataxias?
AD, 40 types, usually present in middle age.
What is the lumbar plexus derived from?
Anterior rami of L1-L5 nerve roots.
What is exploding head syndrome?
Sudden loud noise or sensation of explosion in head at wake-sleep transition.
What are the symptoms of facioscapulohumeral muscular dystrophy?
Asymmetric weakness of face and shoulder girdle muscles, scapular winging.
What is the risk associated with SSRI and MAOI?
Don't take SSRI within 2 weeks washout of MAOI.
What are the risks for spina bifida?
Affected siblings, inadequate folic acid, maternal B12 deficiency, maternal hyperthermia, pregestational maternal diabetes, valproic or carbamazepine use.
What is acute brachial plexus neuritis?
Severe shoulder and upper arm pain followed by weakness in absence of trauma.
What are the symptoms of necrotizing vasculitis?
Headaches, altered mental status, stroke.
What does pure motor deficit indicate?
Internal capsule lacunar infarct.
What is posterior interosseous neuropathy?
Weakness of wrist extension, sensory loss to dorsal wrist capsule.
What are Freud's psychosexual stages?
Oral, anal, phallic, latency, genital.
What are carbon monoxide findings on MRI?
Globus pallidus on DWI.
What is Ullrich’s congenital muscular dystrophy?
Distal hypermobility in collagen disorder, neonatal weakness, and contractures.
What are the effects of acute arsenic ingestion?
Hypovolemic shock.
What is chronic arsenic exposure associated with?
Palmar and plantar hyperkeratosis.
What is the ulnar nerve derived from?
Anterior rami of C8 and T1 with contributions from C7.
What is Usher syndrome?
Autosomal recessive, hearing loss, retinitis pigmentosa, vestibular areflexia, gene - myosin VIIA.
Where are otoliths located?
In the utricle and saccule of the vestibular apparatus.
What type of inheritance is Usher syndrome?
AR
What are the main symptoms of Usher syndrome?
Hearing loss, Retinitis pigmentosa, Vestibular areflexia
Which gene is associated with Usher syndrome?
myosin VIIA
Where are otoliths located?
Utricle and saccule of vestibular apparatus
What is the most common symptom of MS?
Fatigue
Which chromosome is associated with Huntington's disease?
Chromosome 4
What effect does tramadol have on synapses?
Increases 5HT
What type of sleep is confusional arousal associated with?
Non-REM
What disease causes changes in the corpus callosum?
Marchiafava-Bignami disease
What are the CSF findings in Alzheimer's disease?
Increased CSF tau, Decreased A-B
What can temporal lobe epilepsy affect?
Sexuality
What is the concordance rate of schizophrenia in monozygotic twins?
30-40%
What is a symptom of calpain-3 deficiency?
Scapular winging
What causes incomplete ring enhancement in imaging?
Demyelination
What is the mechanism of action of tramadol?
Mu-opioid receptor, weakly inhibits reuptake of NE and serotonin
What are symptoms of an epidermoid cyst?
Hearing loss, headaches, recurrent aseptic meningitis
What nerve supplies the knee extensors?
L4
Which nerves are affected in weakness of the hamstrings?
Sciatic nerve (L5, S1, S2)
What causes numbness of the dorsum of the foot?
Sciatic nerve L4-S3 or Common peroneal nerve L4-S2
What causes weakness of hip flexors?
Femoral nerve (L2-L4)
What syndrome is associated with pyruvate dehydrogenase deficiency?
Leigh syndrome
Which antibodies are associated with Miller Fisher syndrome?
anti-GQ1B
What are the potential side effects of statins?
Direct toxic effect on muscle, immune mediated
What is the most common tumor of the conus medullaris?
Myxopapillary ependymoma
What can corticosteroids cause in myasthenia gravis?
Transient worsening of symptoms
What chromosome is associated with TSC1?
Chromosome 9
What chromosome is associated with TSC2?
Chromosome 16
What syndrome is associated with Parinaud syndrome?
Dorsal midbrain lesion
What are the mutations associated with frontotemporal dementia?
MAPT, GRN, CHMP2B, DCTN1
What is a side effect of valproic acid in children?
Decreased IQ
What are common side effects of lamotrigine?
Headaches, Diplopia, Dizziness
What is the mechanism of action of lamotrigine?
Enhances slow inactivation of voltage-dependent Na channels
What causes neonatal myasthenia gravis transiently?
Due to mothers
What is associated with broad aseptate hyphae?
Mucormycosis
What is the WHO grade of choroid plexus papilloma?
Grade 1
What is the treatment for CRPS?
Steroids, Bone loss preventive med, Antidepressants, Analgesic - NSAID
What can VZV affect?
Large and small arteries, causing cerebral hemorrhage and ischemia
What is the family history association with RLS?
Usually first degree relatives have RLS
Which disease may show symptoms of Kluver-Bucy syndrome?
Pick's disease
What characterizes Marburg's MS?
Fulminant MS, rapid progression, severe disease course, death within 1 year
What tests are used for fibromyalgia diagnosis?
No specific test, can do CBC, ESR, CRP
What chromosome is associated with NF1?
Chromosome 17
What enzyme is deficient in galactosemia?
Galactose 1 phosphate uridyl transferase
What is Chiari 1 associated with?
Syringomyelia (40-75%)
What is a symptom of McArdle's disease?
Muscle cramps
What is methadone's mechanism of action?
MU-receptor agonist and NMDA receptor
What is yophosphorylase associated with?
Muscle cramps, myoglobinuria, second wind phenomenon, electrical silence during muscle contracture
What type of receptor does methadone act on?
MU-receptor agonist
What is another action of methadone?
NMDA receptor antagonist
What is the function of the ciliary body?
Helps with accommodation
What is the treatment for glioblastoma?
Resection followed by radiation with chemo (temozolomide)
What condition is associated with bamboo spine?
Ankylosing spondylitis
What genetic test is related to ankylosing spondylitis?
HLA-b27
What characterizes demyelination in certain conditions?
Sparing of u fibers
What deficiency is associated with Krabbe disease?
Galactosylceramidase deficiency
What does an EEG look like in catatonia?
Normal EEG
What is spasmus nutans?
Usually occurs between 6-12 months, no intervention needed
What is the most common location for chordomas?
Sacrococcygeal region
What is a preventive treatment for cluster headaches?
Verapamil
What type of disorder is Emery-Dreifuss muscular dystrophy?
X-linked disorder
What are the symptoms of Emery-Dreifuss muscular dystrophy?
Absence of emrin, contractures, muscle weakness
What injury is associated with orbitofrontal cortex damage?
Disinhibition syndrome
What symptoms arise from bilateral anterior cingulate lesions?
Apathy, indifference, reduction of movement
What are the effects of dorsolateral prefrontal cortex lesions?
Anhedonia, impaired judgment, planning, problem solving
What does prolonged P100 latency indicate?
Multiple sclerosis
What deficiency is associated with Niemann-Pick A?
Sphingomyelinase deficiency
What are the symptoms of Niemann-Pick B?
Hepatosplenomegaly, lung disease, not CNS
What characterizes Niemann-Pick C?
Cerebellar involvement in middle to late childhood
What is associated with transverse myelitis?
Numerous T2/Flair hyperintense lesions
What is a useful marker for thymoma?
Anti-striated muscle antibodies
What is the first line treatment for stiff person syndrome?
Benzodiazepines
What brain areas show decreased activity in Alzheimer's?
Temporal, parietal, some frontal lobe
What is the greatest risk associated with clozapine?
Seizures
What cells clean debris in the CNS?
Microglia
What is central sensitization?
Wind up phenomenon from repeated stimulation of C fibers
What are the components of Miller Fisher triad?
Ophthalmoplegia, areflexia, ataxia
How does glucose cross the blood-brain barrier?
Through GLUT1 protein
What does locked-in syndrome affect?
Pons bilaterally
What is the likely diagnosis for a 10-year-old with seizures and hypomelanotic nodules?
Tuberous sclerosis complex (TSC)
Is it true that nightmares allow clear recall of dreams?
True
Why are carbidopa and levodopa given together?
Carbidopa inhibits peripheral conversion of levodopa
What characterizes anorexia nervosa restricting type?
Excessive dieting, exercise, fear of weight gain, BMI < 18.5
What are the symptoms of neurosyphilis general paresis?
10-30 years post-infection, hyperreflexia, personality changes
What imaging findings are associated with bipolar disorder?
Increased intensity in frontal white matter, decreased right caudate volume
What sleep behavior is associated with Parkinson's disease?
REM sleep behavior disorder
What do A waves in ICP indicate?
Pathological elevations typically in 30-50s
What is hemimegalencephaly?
A condition with enlarged cerebral hemisphere
What is task-specific dystonia?
Associated with voluntary muscle action and overflow activation
What are the symptoms of fibromyalgia?
Chronic widespread pain, fatigue, heightened pain response
What gene is associated with GEFS+ seizures?
SCN1A
What can be used for migraine if triptans are contraindicated?
Intranasal lidocaine
What lesion is associated with hemiballismus?
Contralateral subthalamic nucleus
What is prosopagnosia?
Facial recognition impairment due to fusiform lesion
What is a characteristic of left frontocentral seizures?
Evolution of frequency
What is the lesion associated with Hemiballismus?
Contralateral subthalamic nucleus
What condition is characterized by facial recognition issues?
Prosopagnosia
Where is the lesion in prosopagnosia located?
Fusiform
What type of seizure is associated with the left frontocentral area?
Left frontocentral seizure
What condition may a neonate with septo optic dysplasia have?
Empty sella
What antibodies are associated with Polymyositis?
Antibodies to extractable nuclear antigens (anti-ENA)
What is the risk factor for malignancy in Polymyositis?
Negative risk factor
What fibers are responsible for 'fast pain'?
A-delta fibers
What fibers are responsible for 'slow pain'?
C-fibers
Which allele is associated with a lower risk of Alzheimer's disease?
APOE2 allele
What condition is characterized by congenital absence of cerebellar vermis?
Dandy Walker malformation
What are common symptoms of Dandy Walker malformation?
Seizures or signs of hydrocephalus including ataxia
What is the risk of depression in elderly patients with CVA?
Higher risk with left hemisphere lesions
What distinguishes Inclusion Body Myositis from Polymyositis?
Slow progressive weakness and wasting of both distal and proximal muscles
What antibodies are associated with Miller Fisher syndrome?
GQ1b antibodies
What is the mainstay treatment for ADEM?
IV steroids with oral taper
What are common signs of PRES?
Decreased LOC and seizure, history of HTN, history of headache
Which cranial nerves pass through the superior orbital fissure?
CN III, IV, VI, superior ophthalmic vein, branch of inferior ophthalmic vein
What is a common feature of MSA?
Sleep apnea
What gene is associated with Holoprosencephaly?
Sonic hedgehog gene
What are the symptoms of Progressive Muscular Atrophy?
Progressive muscle weakness, usually distal, hyporeflexia/areflexia
What happens to Carbamazepine after initiation?
It autoinduces itself
What is the reaction time for Disulfiram with alcohol?
Can occur immediately and last > 2 hours after 1 alcohol beverage
What skull opening allows passage for the middle meningeal artery?
Foramen spinosum
What is the most common cause of delirium in advanced age?
Multiple medications
What type of aphasia is associated with a hypointensive event from LMCA/ACA watershed?
Broca's aphasia with intact repetition - transcortical motor aphasia
What lab test is used for NTD in fetuses?
AFP in maternal serum
What is a side effect of antipsychotics that can be treated with beta blockers?
Akathisia
What is the mechanism of action of Aspirin?
Inhibits synthesis of thromboxane A2
What is the fact about port wine stains?
90% infants with facial port wine stains do not have any intracranial lesion
What are the findings of Sturge-Weber syndrome?
Glaucoma, ID, focal neuro deficits, seizures
What causes loss of consciousness from injury?
Injury to bilateral thalamus, bilateral hemispheres, bilateral RAS, bilateral pons
What is classical conditioning?
Conditioned stimulus elicits conditioned response due to proximity of unconditioned stimulus
What is operant conditioning?
Reinforcement or punishment is used to modulate frequency of a particular behavior
Are iris hamartomas found in TSC?
No, they are found in NF1
What is the treatment for vestibular paroxysmia?
Antiepileptics
What can help with spasticity reduction in lower limb?
High sciatic phenol nerve block
What causes myelomeningocele?
Failure of posterior neuropore closure at 26 days
What is not seen in frontotemporal dementia?
Visual hallucinations
What is the mechanism of action of Carbamazepine?
Stabilize inactivated voltage-gated Na channels
What is the mechanism of action of lidocaine?
Fast sodium-channel blockade
What is the mechanism of action of ziconotide?
Blockade of N-type Ca channel
What herb is taken for tinnitus?
Ginkgo biloba
What artery is the medial orbitofrontal artery a branch of?
Anterior cerebral artery
What artery is the anterior hippocampal artery a branch of?
Posterior cerebral artery
What artery is the anterior choroidal artery a branch of?
Internal carotid artery
How is Alzheimer's diagnosed?
Clinical diagnosis only
What is a major risk factor for NMS?
History of catatonia
What is the anterior choroidal artery a branch of?
Internal carotid artery
How is Alzheimer's diagnosed?
Clinical diagnosis only
What is a major risk factor for NMS?
History of catatonia
What is the most common gene associated with FtD?
C9ORF72 gene
Which gene is also associated with familial ALS?
C9ORF72 gene
Which 2nd generation antipsychotic is likely to cause hyperglycemia and obesity?
Olanzapine
Which TCA can also be used for BPH?
Desipramine
What type of amine is Desipramine?
Secondary amine TCA
What side effects is Desipramine less likely to cause?
Anticholinergic side effects
What is the mechanism of action of mirtazapine?
Presynaptic alpha-2 antagonist, postsynaptic antagonist at 5HT2, 5HT3, and H1 receptors
What are the etiologies of lateral periodic discharges?
Stroke, intracranial mass, subdural hematoma, encephalitis
What are the 3 major groups of cortical development?
Group 1 - abnormal cell proliferation or apoptosis; Group 2 - defects in cell migration; Group 3 - development defects after cell migration
What is extinction in behavior?
Loss of previously learned operant response after it is no longer reinforced
What occurs in myoclonic-astatic epilepsy?
Myoclonic jerks followed by atonic seizures; neurocognitive development usually normal prior to seizures
What is the pharmacological function of barbiturates?
Increased duration of GABA Cl- channel opening
What do barbiturates do for the brain?
Decrease cerebral metabolic activity, blood flow, and intracranial pressure
What is the pharmacological function of benzodiazepines?
Increase frequency of GABA Cl- channel opening
What is paroxysmal torticollis?
Benign intermittent self-limited episodes of head tilting lasting hours to days
What does dissection of the internal carotid artery cause?
Symptoms related to carotid artery disruption
What is thoracoabdominal polyradiculopathy?
Nonradicular truncal pain, abdominal bulging, sensorimotor changes
What are endocrine causes of increased intracranial pressure?
Hypoparathyroidism, Addison's, Cushing's, pregnancy
What does damage to the postcentral gyrus cause?
Loss of proprioception, astereognosis, loss of vibratory sense, loss of 2-point discrimination
Who is most affected by Reye syndrome?
Children/teens recovering from chickenpox or flu-like symptoms
Why do patients with Down syndrome develop Alzheimer's?
Increased expression of beta amyloid precursor protein
What are positive predictors for infantile spasms?
Diagnosis over age 4 months, short time from onset to treatment, early response to treatment
What neurological issues are associated with Ehlers Danlos?
Intracranial aneurysms, carotid-cavernous fistulas, arterial dissection
What supplies the lateral geniculate nucleus and lateral thalamus?
CISTERNAL portion of anterior choroidal artery
What is assimilation?
How individuals take in new information and incorporate it into existing ideas
Where do brain teratomas usually occur?
Pineal, suprasellar, cerebellar regions; can go to lateral ventricles
What is growing teratoma syndrome?
Occurs in 21% of nongerminomatous germ cell tumors during treatment; requires surgery/radiation
What usually causes an epidural hematoma?
Trauma; 95% skull fractures, 85% tear of middle meningeal artery
What is the MOA of teriflunomide?
Inhibits dihydroorotate dehydrogenase; targets pyrimidine synthesis
What is the MOA for fingolimod?
Sphingosine-1-phosphate receptor blocker; prevents lymphocyte circulation into CNS
What findings indicate central sleep apnea?
Repetitive cessation or decrease of both airflow and ventilatory effort during sleep
What findings indicate obstructive sleep apnea?
Repetitive cessation or decrease of airflow but NOT of ventilatory effort during sleep
What is Cheyne Stokes respiration?
Cyclic breathing pattern; apnea followed by increasing then decreasing respiratory frequency
What are symptoms of lateral pontine syndrome?
Ipsi facial palsy, Ipsi hearing loss, vertigo, nystagmus, contra loss pain and temp, ipsi ataxia
What causes medial medullary syndrome?
Occlusion of perforating branches of the vertebral artery or basilar artery
What is ventral pontine syndrome (Millard-Gubler)?
Unilateral facial palsy, ipsi abducens palsy, contralateral hemiparesis
What does unilateral lateral lemniscus infarction cause?
No auditory deficits due to bilateral representation
What is infantile neuroaxonal dystrophy?
Causes axonal swelling in cerebral and anterior horn cell gray matter
What is periodic limb movement on polysomnography?
Leg movement >/= 8 uv in EMG lasting 0.5-10 seconds
What sensory area is usually affected in Parsonage Turner syndrome?
Lateral shoulder, arm, and or hand
What are risk factors for Parsonage Turners?
Viral infection, bacterial infections, immunizations, stress, meds, radiation
What are MRI findings in chronically schizophrenic patients?
Reduced volume of prefrontal cortex, superior temporal gyrus
What is distal symmetric diabetic sensory polyneuropathy?
A type of nerve damage related to diabetes.
What is mononeuritis multiplex?
Asymmetric involvement of >/=2 named nerves in separate parts of the body.
What endocrine disorders can lead to proximal myopathy?
Hyperparathyroidism, Hypothyroidism, Hyperthyroidism (CK usually normal).
What are neurofibrillary tangles?
Pathological aggregates in neurons, associated with Alzheimer's disease.
What is amyloid angiopathy?
A condition characterized by the deposition of amyloid in the walls of blood vessels.
What are symptoms of Chiari I malformation?
Occipital headache, Neck pain, Myelopathy, Gait abnormalities, Cranial neuropathy, Brainstem compression, Syringomyelia.
What is Chiari II malformation?
Myelomeningocele, Displacement of cerebellar tonsils, Inferior cerebellar vermis, Medulla into the upper cervical canal, May have hydrocephalus.
What is Chiari III malformation?
Downward displacement of cerebellum from a small posterior fossa into cervical or occipital encephalocele.
What is the benign paroxysmal vertigo of childhood?
Migraine equivalent typically remits by age 5, Short episodes, Nystagmus, nausea, vomiting.
What is vestibular paroxysmia?
Very brief episodes of vertigo, may be caused by compression of vestibular nerve, usually responds to carbamazepine.
What can sevoflurane cause?
Malignant hyperthermia; remove offending agent and can give dantrolene if needed.
How do TCAs work for pain?
Enhance descending pain inhibition via increased serotonin and NE to inhibit nociceptive pathways.
What has a better prognosis for medulloblastoma?
Monosomy 6, Positive staining for beta catenin, Total or near total resection, Localized disease.
What has a worse prognosis for medulloblastoma?
MYC amplification, Subtotal resection, Anaplasia on pathology, Positive CSF cytology and disease dissemination.
What gene is implicated in congenital neuronal ceroid lipofuscinosis?
Ceroid lipofuscinosis neuronal 10 (CLN10).
What are symptoms for ceroid lipofuscinosis?
Neonatal period, Pass away in first week, Seizures, microcephaly, lack of reflexes.
What can be used for pediatric acute migraines in children 12 years and older?
Combo sumatriptan and naproxen.
What may nitrous oxide inhalation unmask?
Vitamin B12 deficiency; can cause paresthesias in hands and feet.
What is Hoover’s sign?
Involuntary extension of the stationary leg when flexing the other leg; absence suggests conversion disorder.
What is somatic symptom disorder?
Presents with >/= 1 physical symptom causing disproportionate emotional distress.
When to consider antifibrinolytic therapy for delayed aneurysm?
If repair > 72 hours from onset is anticipated.
What is the mechanism of action of COMT inhibitors?
Prevents COMT from metabolizing L'DOPA into 3OMD
What are the 3 infant development stages by Margaret Mahler?
Autistic phase, Symbiotic phase, Separation individuation
What are findings in cold water caloric testing?
Slow component toward ipsilateral side, fast component opposite
What to do if parents decline treatment in an emergency?
Must initiate treatment if life threatening
What indicates obstructive sleep apnea in children?
25% total sleep with PaCO2 >50 mmHg and child snores
What is the requirement for sleep-related hypoxemia?
Arterial oxygen saturation ≤ 90% for at least 5 minutes
What paraneoplastic syndrome is associated with cerebellar degeneration?
Anti-Yo ab or Purkinje cell cytoplasmic ab type 1
What are the 6 layers of the neocortex?
Molecular layer, External granular, External pyramidal, Internal granular, Internal pyramidal, Multiform layer
What is the Pulfrich phenomenon?
Visual illusion in MS; moving object appears to travel in an elliptical orbit
What are symptoms of paroxysmal hemicrania?
Pain 20-30 min, >5x/day, triggered by alcohol, unilateral
Where are subclinical rhythmic electrographic discharges maximal?
Parietal or posterior temporal regions
What are the diagnostic criteria for CIDP?
1) Progressive or relapsing dysfunction of >1 limb, 2) Develops over 8 weeks, 3) Areflexia or hyporeflexia
What MRI findings are seen in fragile X tremor ataxia syndrome?
Hyperintensities in cerebellum, corpus callosum, middle cerebellar peduncle on T2
What are symptoms of hereditary sensory and autonomic neuropathy type 2?
AR, loss of pain/temp/touch/pressure, recurrent infections, fractures
What treatment supports nocturnal hypoventilation due to neuromuscular disease?
Bilevel (spontaneously timed mode)
Which areas are vulnerable to loss of oxygen?
CA1 region of hippocampus, cortical pyramidal neurons, cerebellar purkinje cells, brainstem nuclei
What type of neuropathy is diabetic lumbosacral radiculoplexus neuropathy?
Vasculitic axonal neuropathy
What are the symptoms for inclusion body myositis?
Weak wrist and finger flexors, weak quadriceps, weak anterior tibial muscles
What is diabetic neuropathic cachexia?
Painful, acute, sensory, motor, autonomic, often bilateral thighs, severe weight loss
What is the pathology of palatal myoclonus?
Mostly brainstem lesions of central tegmental tract or dentate-olivary pathways
Order of thoracolumbar spinal injuries by increasing severity?
Compression fracture -> Burst fracture -> Translational/rotational -> Distraction
Difference between hemorrhagic and ischemic strokes?
Hemorrhagic strokes cause severe headache, nausea, vomiting, decreased LOC
What is musculocutaneous neuropathy?
Weakness in flexion of forearm, absent biceps reflex, sensory loss on lateral forearm
What is a finding in the ophthalmic vein in carotid cavernous fistula?
Dilated superior ophthalmic vein by CT
What nuclei provide parasympathetic innervation?
Edinger Westphal nucleus, Superior salivatory nucleus
What characterizes multifocal acquired demyelinating neuropathy?
Asymmetric motor and sensory loss with conduction block
What are symptoms of Behcet's disease?
Recurrent oral and genital ulcers, ophthalmic and skin lesions
What CNS findings are associated with Behcet's disease?
Relapsing, remitting aseptic meningoencephalitis
What are neuro findings in Sturge-Weber?
Meningeal angiomas, hydrocephalus, tram tracking calcifications
Is a patient unable to maintain fixation upon rapid head turn to the left in vestibular neuritis?
True
What are findings in spinobulbar muscular atrophy?
Progressive weakness and atrophy in facial, bulbar, limb muscles
When to insert an intraventricular catheter for elevated ICP?
Comatose (<8), measure ICP and relieve it
What are findings for granulovacuolar degeneration?
Seen in hippocampal neurons in Alzheimer's disease
What are symptoms of fibromyalgia?
Tingling, muscle spasms, weakness, pain, sleep disturbances, fatigue
What is required by McDonald criteria for MS diagnosis?
1 lesion (spinal cord and left eye) with enhancing
What are the McDonald criteria for MS?
1 lesion (spinal cord and left eye), enhancing and non-enhancing lesions on brain MRI
What are symptoms of a lower trunk lesion?
Excessive arm abduction, fall from height, loss of sensation medial forearm, weakness of intrinsic hand muscles
What are bipolar findings in neuroimaging?
Increased intensity in frontal white matter, decreased volume right caudate, increased volume in hippocampus
What are A waves in ICP monitoring?
Pathologic, plateau waves, loss of intracranial compliance, elevations into 30-50 mmHg
What is the toxicity of nitrous oxide?
Inactivates cobalamin (vitamin B12), causes sensory neuropathy, myelopathy, encephalopathy
What is the mechanism of action of Galantamine?
Reversible acetylcholinesterase inhibitor, nicotinic Ach receptor modulator
What is the most common incomplete syndrome from traumatic spinal cord injury?
Central cord syndrome
What are symptoms of supranuclear vertical gaze palsy?
Myoclonic jerks, pendular vergence ocular movements, concurrent masticatory muscle contraction
What is the pathogenesis of Tourette's syndrome?
Hyperdopaminergic stimulation of limbic system and ventral striatum
What indicates a positive prognosis for conversion disorder?
Acceptance of diagnosis, short duration of symptoms
What can occur in left temporal lobe epilepsy?
Hypergraphia, hyposexuality, intense emotional responses, hyperreligiosity, hypermorality
What are symptoms of perilymphatic fistula?
Fluctuating vertigo, hearing loss, often preceded by head injury or barotrauma, Tulio phenomenon
What is the ABCD1 gene mutation associated with?
Increased very long chain fatty acids, adrenoleukodystrophy
What are the features of Cri du chat syndrome?
Mood facies, hypertelorism, low set ears, hypotonia, high pitched cry
What are the features of Walker-Warburg syndrome?
Muscular dystrophy, cerebral and eye malformations, cobblestone lissencephaly
What does elevated acetylcholinesterase in amniotic fluid indicate?
Increased sensitivity/specificity of neural tube defect screening
What is the mechanism of action of Topamax?
Blocks Na channels, inhibits carbonic anhydrase and glutamic acid, stimulates GABA-A
What are the symptoms of argininosuccinate lyase deficiency?
Vomiting, lethargy, seizures, coma, respiratory failure
What does dermatomyositis EMG show?
Increased insertional activity, fibrillations, positive sharp waves, proximal weakness
What is critical illness polyneuropathy?
Inactivation of Na ion channels, low motor and sensory amplitudes on NCS
What conditions are associated with mutations in CACNA1A?
Episodic ataxia 2, spinocerebellar ataxia type 6, childhood absence epilepsy
What are the muscle fiber characteristics in central core myopathy?
Single or multiple central or slightly eccentric cores in at least 20% of muscle fibers.
What is benign myoclonic epilepsy of infancy?
A type of epilepsy characterized by myoclonic seizures in infants.
When should a cervical spine radiograph be obtained in Down syndrome?
Only if symptoms of atlantoaxial subluxation develop.
What are the symptoms of axillary neuropathy?
Diminished sensation over lateral upper arm, loss of arm abduction, and weakness of deltoid and teres minor.
What is Lambert-Eaton myasthenic syndrome associated with?
Low CMAP and increment with high-frequency repetitive stimulation.
What are the features of congenital toxoplasmosis?
Hydrocephalus, chorioretinitis, and intracranial calcifications.
What is the characteristic of axonal loss peripheral neuropathy?
Low amplitude with preserved conduction velocity.
What is Leber congenital amaurosis?
Retinal dystrophy and the most common genetic cause of congenital visual impairment.
What does the digit span test assess?
Immediate recall, more affected in acute psych disorders than early neurodegenerative disease.
What does routine screening for dementia include?
Depression screening, BUN, creatinine, electrolytes, glucose, LFTs, thyroid, B12, CBC.
What is exploding head syndrome?
Sudden loud noise or sensation of explosion in head at wake-sleep transition.
What are the symptoms of facioscapulohumeral muscular dystrophy?
Asymmetric weakness of face and shoulder girdle muscles, scapular winging.
What are the risks for spina bifida?
Affected siblings, inadequate folic acid, maternal B12 deficiency, maternal hyperthermia, pregestational maternal diabetes, valproic or carbamazepine use.
What is acute brachial plexus neuritis?
Severe shoulder and upper arm pain followed by weakness in absence of trauma.
What is posterior interosseous neuropathy?
Weakness of wrist extension, sensory loss to dorsal wrist capsule.
What is Ullrich’s congenital muscular dystrophy?
Distal hypermobility in collagen disorder, neonatal weakness, and contractures.
What is Usher syndrome?
Autosomal recessive, hearing loss, retinitis pigmentosa, vestibular areflexia, gene - myosin VIIA.
What are the main symptoms of Usher syndrome?
Hearing loss, Retinitis pigmentosa, Vestibular areflexia
What is the mechanism of action of tramadol?
Mu-opioid receptor, weakly inhibits reuptake of NE and serotonin
What is the mechanism of action of lamotrigine?
Enhances slow inactivation of voltage-dependent Na channels
What is the treatment for CRPS?
Steroids, Bone loss preventive med, Antidepressants, Analgesic - NSAID
What characterizes Marburg's MS?
Fulminant MS, rapid progression, severe disease course, death within 1 year
What is yophosphorylase associated with?
Muscle cramps, myoglobinuria, second wind phenomenon, electrical silence during muscle contracture
What are the symptoms of Emery-Dreifuss muscular dystrophy?
Absence of emrin, contractures, muscle weakness
What symptoms arise from bilateral anterior cingulate lesions?
Apathy, indifference, reduction of movement
What are the effects of dorsolateral prefrontal cortex lesions?
Anhedonia, impaired judgment, planning, problem solving
What is the likely diagnosis for a 10-year-old with seizures and hypomelanotic nodules?
Tuberous sclerosis complex (TSC)
What characterizes anorexia nervosa restricting type?
Excessive dieting, exercise, fear of weight gain, BMI < 18.5
What are the symptoms of neurosyphilis general paresis?
10-30 years post-infection, hyperreflexia, personality changes
What imaging findings are associated with bipolar disorder?
Increased intensity in frontal white matter, decreased right caudate volume
What antibodies are associated with Polymyositis?
Antibodies to extractable nuclear antigens (anti-ENA)
What condition is characterized by congenital absence of cerebellar vermis?
Dandy Walker malformation
What are common symptoms of Dandy Walker malformation?
Seizures or signs of hydrocephalus including ataxia
What is the risk of depression in elderly patients with CVA?
Higher risk with left hemisphere lesions
What distinguishes Inclusion Body Myositis from Polymyositis?
Slow progressive weakness and wasting of both distal and proximal muscles
Which cranial nerves pass through the superior orbital fissure?
CN III, IV, VI, superior ophthalmic vein, branch of inferior ophthalmic vein
What are the symptoms of Progressive Muscular Atrophy?
Progressive muscle weakness, usually distal, hyporeflexia/areflexia
What is the reaction time for Disulfiram with alcohol?
Can occur immediately and last > 2 hours after 1 alcohol beverage
What type of aphasia is associated with a hypointensive event from LMCA/ACA watershed?
Broca's aphasia with intact repetition - transcortical motor aphasia
What is the fact about port wine stains?
90% infants with facial port wine stains do not have any intracranial lesion
What causes loss of consciousness from injury?
Injury to bilateral thalamus, bilateral hemispheres, bilateral RAS, bilateral pons
What is classical conditioning?
Conditioned stimulus elicits conditioned response due to proximity of unconditioned stimulus
What is operant conditioning?
Reinforcement or punishment is used to modulate frequency of a particular behavior
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