Neurology: Step 2 Flashcards

1
Q

What are the symptoms associated with Parkinson Disease?

A

Tetrad: Resting tremor, bradykinesia, rigidity, and postural instability (asymmetrical)

Other: masked facies, memory loss, and micrographia

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2
Q

What are the possible etiologies of Parkinsonism (bradykinesia and rigidity)?

A

Vascular (multiple infarcts), NPH, Metabolic, Toxins, Infection, Drugs (Reserpine/tetrabenazine, metoclopramide/prochloperazine, atypical antipsychotics)

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3
Q

If a suspected Parkinson patient fails to respond to Levodopa, what diagnoses are more likely?

A
  1. Multiple system atrophy: Often has autonomic or cerebellar component; MRI for diagnosis
  2. Progressive supranuclear palsy: Inability to look down (can’t go down stairs)
  3. Corticobasal ganglionic degeneration: Often has component of limb apraxia or dystonia
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4
Q

What are the treatment options for Parkinson disease?

A
  1. Carbidopa/levodopa (DA mimetic)
  2. Ropinirole/Pramipexole/Bromocriptine (DA agonists)
  3. Selegiline (MAO-B inhibitor)
  4. Entacapone/tolcapone (COMT inhibitors)
  5. Amantadine (NMDA antagonist)
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5
Q

What are the non-pharmacologic treatment options for Parkinson disease?

A
  1. DBS (deep brain stimulation)

2. Surgical pallidotomy

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6
Q

What is the pathophysiology of Huntington Disease?

A
  1. A hyperkinetic, autosomal dominant trinucleotide repeat (CAG) repeat disease involving the HD gene on chromosome 4 and showing “anticipation”
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7
Q

What are the symptoms associated with Huntington Disease?

A

Triad: Chorea (sudden onset of purposeless, involuntary, dance-like movements), Altered behavior, and Dementia (antisocial, irritability, clumsiness, etc)

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8
Q

What is a reasonable differential diagnosis for choreiform movements?

A
  1. Hereditary (HD, Spinocerebellar ataxia, pseudohypoparathyroidism)
  2. Autoimmune (vasculitus, SLE)
  3. Neoplasm (tumor, paraneoplastic syndrome)
  4. Metabolic (hyperthyroidism, etc)
  5. Toxins/drugs
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9
Q

What is the gold standard for diagnosis of Huntington Disease?

A
  1. DNA analysis for number of CAG repeats (>29 is abnormal)

2. CT/MRI shows caudate atrophy and ex vacuo hydrocephalus (Not Gold Standard)

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10
Q

What are the treatment options available for patients with Huntington Disease?

A
  1. Chorea with Reserpine/tetrabenazine
  2. Psychosis with atypical antipsychotics
  3. Depression/anxiety with SSRI
  4. Provide genetic counseling
    All symptomatic relief!
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11
Q

What is the pathophysiology of Spinocerebellar ataxia? Diagnostic evaluation? Treatment?

A
  1. AD inheritance of cerebellar atrophy that leads to progressive ataxia/gait disturbances as well as various other neurological features
  2. Diagnosis is made by DNA analysis
  3. Treatment is supportive
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12
Q

What is the pathophysiology of primary dystonia? Diagnostic evaluation? Treatment?

A
  1. AD deletion of torsin A gene that leads to sustained muscle contractions, twisting movements, and abnormal postures; The disorder is aggravated by DA antagonists (Like PD)
  2. Clinical (i.e. no signs of secondary etiology)
  3. DBS of GPi ( best) or trihexyphenidyl
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13
Q

What is the pathophysiology of Parkinson Disease? When does Parkinson Disease classically present?

A
  1. Hypo kinetic disorder caused by loss of dopaminergic neurons in the Substantia Nigra; It is characterized by the presence of eosinophilic cytoplasmic inclusion bodies (Lewy)
  2. The 5th and 6th decades of life
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14
Q

What is the pathophysiology of Essential tremor? Diagnostic evaluation? Treatment?

A
  1. AD inheritance of BILATERAL hand tremor with movement and fine motor skill tasks that spreads to the legs, larynx, and HEAD
  2. Rule out other etiologies; PET scan shows increased thalamic uptake
  3. Primidone and Beta-blockers for symptom relief OR Botox injections OR DBS
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15
Q

What is a reasonable DDx for tremor?

A
  1. Physiologic
  2. Essential
  3. Parkinson Disease
  4. Hyperthyroidism
  5. Multiple sclerosis
  6. Head trauma (cerebellum or red nucleus)
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16
Q

What is a reasonable differential diagnosis for seizure?

A

VITAMINS (Vascular, Infectious, Trauma, Autoimmune, Metabolic, Idiopathic, Neoplasm, pSychiatric); Eclampsia, liver failure (ammonemia), renal failure (uremia)

17
Q

What are the “red flags” for secondary etiologies of seizures?

A
  1. Age of onset (Infant or Adult)

2. Focal neurological deficit(s)

18
Q

What is the difference between partial and generalized seizures?

A

Partial: involves a focus on 1 hemisphere, with no associated loss of consciousness
Generalized: involves both hemispheres and results in loss of consciousness

19
Q

What is the difference between simple partial and complex partial seizures?

A

Simple: no altered/impaired consciousness with motor, sensory, or autonomic features; postictal neurological deficits (resolve in 24 hours)
Complex: altered/impaired consciousness with automatisms, hallucinations, and Déjà vu; postictal confusion is common

20
Q

What are the diagnostic evaluations available for seizures?

A
  1. EEG
  2. MRI (If focal signs are present, r/o lesion)
  3. Blood screening for secondary etiologies
21
Q

What are the various types of generalized seizures?

A
  1. Tonic-clonic (grand mal): tonic contraction, followed by rhythmic muscle contractions
  2. Myoclonic
  3. Atonic (drop)
  4. Tonic
  5. Clonic
22
Q

What are the EEG findings associated with partial, generalized, and absence seizures?

A
  1. Partial: epileptogenic focus with spread
  2. Generalized: 10 Hz activity followed by slow waves
  3. Absence: 3 per second spike and wave discharges
23
Q

What are the preferred treatment options for partial seizures? Children?

A

Phenytoin, Carbamazepine, Valproate, and phenobarbital (1st line in children)

24
Q

What are the preferred treatment options for primary generalized seizures? Absence?

A

Absence: Ethosuximide or Valproate
Primary generalized: Phenytoin and Valproate
With Topiramate or Lamotrigine as adjuncts
Secondary generalized: same as partial

25
Q

What is the definition of status epilepticus? Evaluation? Treatment?

A

> 10 minutes of constant or repetitive seizures without a return to baseline consciousness; CT head to r/o hemorrhage then tailored to find likely etiology; ABCs, IV benzodiazepines plus loading dose of fosphenytoin, naloxone, thiamine+glucose, and Phenobarbital if symptoms do not improve

26
Q

A male infant presents with generalized seizure and has a positive family history. Which disorder is most likely? How is the diagnosis confirmed? Sequelae? Treatment?

A
  1. West syndrome (infantile spasms)
  2. Hypsarrhythmia on EEG
  3. Mental retardation and arrested cognitive development
  4. Hormonal therapy: ACTH, prednisone, and valproate or clonazepam (does not improve prognosis)
27
Q

Why is hypsarrhythmia?

A

High amplitude and irregular waves and spikes on a background of chaotic or disorganized activity

28
Q

A child presents with recurrent seizures despite the use of multi-drug therapy. What is the most likely etiology? Diagnosis? Sequelae? Treatment?

A
  1. Lennox-Gastaut syndrome
  2. Slow spike and wave appearance on EEG
  3. Mental retardation and delayed psychomotor development
  4. Resistant
29
Q

What is the difference between absence, atypical absence, and complex partial seizures?

A
  1. Distinct beginning/end with absent awareness for 20 seconds

Absence: children; Partial: adults