Flashcards in this deck (63)

• What age does Motor Neuron Disease generally begin in adults?

  Between 30 and 60 years
  disease age
• What is the average annual incidence of Motor Neuron Disease?

  Approximately 2 per 100,000
  disease incidence
• What is the male predominance in Motor Neuron Disease?

  Yes, there is a male predominance
  disease gender
• What percentage of Motor Neuron Disease cases may be familial?

  5–10%
  disease genetics
• What is characterized by degeneration of anterior horn cells?

  Motor Neuron Disease
  disease symptoms
• What are the five types of Motor Neuron Disease?

  1. Progressive Bulbar Palsy 2. Pseudobulbar Palsy 3. Progressive Spinal Muscular Atrophy 4. Primary Lateral Sclerosis 5. Amyotrophic Lateral Sclerosis
  disease classification
• What is the most common form of Motor Neuron Disease?

  Amyotrophic Lateral Sclerosis
  disease common
• What initial symptoms occur in 20% of ALS patients?

  Weakness of bulbar muscles
  disease symptoms
• What are the symptoms of bulbar involvement?

  Difficulty in swallowing, chewing, coughing, breathing, and speaking
  disease symptoms
• What are the limb involvement symptoms in ALS?

  Fatigability, weakness, stiffness, twitching, wasting, muscle cramps, weight loss
  disease symptoms
• What does examination reveal in ALS?

  Upper or lower motor neuron signs
  disease examination
• What is required for a definitive diagnosis of Motor Neuron Disease?

  Combined upper and lower motor neuron signs in bulbar region + two spinal regions
  disease diagnosis
• What is the approved drug treatment for Motor Neuron Disease?

  Currently, no specific drug treatment is approved
  treatment drugs
• What is the effect of Riluzole in ALS treatment?

  Slightly slows disease progression, prolongs life by 2-3 months
  treatment riluzole
• What is Edaravone used for in ALS?

  It is a free-radical scavenger, infused IV over 10 days per month
  treatment edaravone
• What are the symptoms of Motor Neuron Diseases in children?

  Proximal and distal wasting, fasciculation, weakness of muscles
  disease children
• What is Infantile Spinal Muscular Atrophy?

  A genetically determined disorder affecting spinal and cranial motor neurons
  disease children
• What type of inheritance is associated with Hoffmann Disease (SMA-I)?

  Autosomal recessive
  genetics sma-i
• When does Hoffmann Disease (SMA-I) typically manifest?

  Within the first 3 months of life
  disease sma-i
• What is a common symptom of Hoffmann Disease (SMA-I)?

  The infant is floppy (hypotonic)
  symptoms sma-i
• What difficulties are associated with Hoffmann Disease (SMA-I)?

  Difficulty with sucking, swallowing, or ventilation
  symptoms sma-i
• What is the progression of Hoffmann Disease (SMA-I)?

  It is a rapidly progressive disease
  progression sma-i
• What is the typical outcome for infants with Hoffmann Disease (SMA-I)?

  Death from respiratory complications by about 3 years of age
  outcome sma-i
• What type of inheritance is associated with Intermediate Spinal Muscular Atrophy (SMA-II)?

  Autosomal recessive
  genetics sma-ii
• When does Intermediate Spinal Muscular Atrophy (SMA-II) begin?

  In the 2nd half of the first year
  disease sma-ii
• What symptoms are seen in Intermediate Spinal Muscular Atrophy (SMA-II)?

  Wasting and weakness of the extremities
  symptoms sma-ii
• How does the progression of Intermediate Spinal Muscular Atrophy (SMA-II) compare to SMA-I?

  Progresses slowly & its course is more benign
  progression sma-ii
• What is the typical survival outcome for patients with Intermediate Spinal Muscular Atrophy (SMA-II)?

  Many patients survive into adulthood
  outcome sma-ii
• What type of inheritance is associated with Juvenile Spinal Muscular Atrophy (SMA-III)?

  Autosomal recessive
  genetics sma-iii
• When does Juvenile Spinal Muscular Atrophy (SMA-III) develop?

  In childhood or early adolescence
  disease sma-iii
• What muscles are primarily affected in Juvenile Spinal Muscular Atrophy (SMA-III)?

  Proximal limb muscles
  symptoms sma-iii
• What is the course of Juvenile Spinal Muscular Atrophy (SMA-III)?

  It follows a gradually progressive course
  progression sma-iii
• What treatment can slow or halt disease progression in spinal muscular atrophy?

  Intrathecal Nusinersen (Spinraza)
  treatment sma
• What is syringomyelia?

  Cavitation of the spinal cord
  condition syringomyelia
• What are the two types of syringomyelia?

  Communicating and Non-communicating
  types syringomyelia
• What is associated with communicating syringomyelia?

  Arnold-Chiari malformation
  conditions syringomyelia
• What can cause non-communicating syringomyelia?

  Trauma, intramedullary spinal tumors, spinal arachnoiditis
  causes syringomyelia
• What is a clinical feature of syringomyelia?

  Dissociated sensory loss at the level of the lesion
  symptoms syringomyelia
• What sensory loss is typically seen in syringomyelia?

  Impaired pinprick and temperature appreciation, preserved light touch
  symptoms syringomyelia
• What are some trophic changes in the upper limbs due to syringomyelia?

  Painless skin ulcers, scars, edema, hyperhidrosis
  symptoms syringomyelia
• What is the prognosis for syringomyelia after surgical treatment?

  Prognosis is bad because surgical treatment is disappointing
  prognosis syringomyelia
• Which imaging techniques confirm the diagnosis of syringomyelia?

  MRI or CT scanning
  diagnosis syringomyelia
• What differentiates avitation inside the cord from intramedullary spinal tumors?

  Avitation inside the cord does not take enhancement.
  medicine neurology
• What is the treatment for avitation inside the cord?

  Surgical treatment.
  medicine treatment
• What is the prognosis for avitation inside the cord?

  Bad; condition frequently reoccurs after surgery.
  medicine prognosis
• What does MRI show in patients with avitation inside the cord?

  Syringomyelia in 2 patients.
  medicine imaging
• How is Familial Spastic Paraplegia (FSP) transmitted?

  Usually transmitted as an autosomal trait.
  genetics fsp
• What type of inheritance is most common in adult-onset FSP cases?

  Dominantly inherited.
  genetics inheritance
• When does FSP typically arise?

  In the third or fourth decade of life.
  medicine fsp
• What degeneration occurs in FSP?

  Degeneration of the corticospinal tracts.
  medicine neurology
• What are the initial symptoms of FSP?

  Progressive spastic paraparesis beginning in distal lower extremities.
  medicine symptoms
• What urinary issues may occur late in FSP?

  Urinary urgency and incontinence; sometimes fecal incontinence.
  medicine symptoms
• How is sexual function affected in FSP?

  Sexual function tends to be preserved.
  medicine fsp
• What is the treatment for FSP?

  Symptomatic treatment.
  medicine treatment
• What type of disease is Multiple Systems Atrophy (MSA)?

  Sporadic disease.
  medicine msa
• What age group is usually affected by MSA?

  Middle-aged and elderly individuals.
  medicine age
• What are the symptoms of MSA?

  Parkinsonism without tremor, autonomic failure, cerebellar involvement, pyramidal tract dysfunction.
  medicine symptoms
• What syndrome is characterized by parkinsonism with autonomic failure?

  Shy-Drager syndrome.
  medicine syndrome
• How does degeneration in MSA compare to Parkinson's disease?

  Degeneration is more widespread than in Parkinson's disease.
  medicine comparison
• What is the response to levodopa in MSA?

  Disappointing response to levodopa and other anti-parkinsonian drugs.
  medicine treatment
• What is a common consequence of MSA compared to idiopathic Parkinson's disease?

  Falls are much more common in MSA.
  medicine symptoms
• How is life expectancy affected in MSA?

  Life expectancy is considerably reduced.
  medicine prognosis
• What is the treatment approach for MSA?

  Symptomatic treatment.
  medicine treatment