Nuerology Shelf Exam Flashcards
Alcoholic cerebellar degeneration
Epidemiology:
- > 10 years heavy alcohol use
- Degeneration of Purkinje cells (cerebellar vermis)
Manifistation:
- develops over weeks to months
- wide-based gait
- incoordination in legs
- nystagmus
- truncal ataxia (visible with walking heel to toe)
- later stage: postural tremor of the fingers & thigh, dysarthria, visual problems/diplopia)
- intact cognition
Diagnosis:
- postural incoordination (impaired tandem walking) (abnormal heel-knee-shin testing)
- preserved limb coordination (normal finger-nose testing)
- muscle hypotonia leading to pendular knee reflex (persistent swinging movements of the limb after eliciting the deep tendon reflex —> > 4 swings is abnormal)
- CT/MRI shows cerebellar atrophy
Treatment:
- stop alcohol
- nutritional supplement
- ambulatory assistance devices (walker)
Cerebellar ataxia
Autosomal recessive:
- Friedreich’s ataxia (FA): arreflexia with pathologic reflexes
- ataxia with vitamin E deficiency
Autosomal dominant:
- spinocerebellar ataxias (SCA): pure cerebellar or extra-cerebellar signs
- episodic ataxia (EA): ataxia attacks
X-linked:
- mitochondrial disorders
- fragile X-associated tremor/ataxia
- x-linked adrenoleukodystrophy
Sporadic:
-multiple systems atrophy (MSA)
Pyramidal tract disease
Manifistation:
- clonus
- Clasp-knife spasticity
- seen in hypertonia
Babiniski sign
- hyperreflexia
- upward deviation of the big toe when the sole of the foot is stroked
- evidence of UMN lesion
Bradykinesia
- slowing of movement
- A hallmark of Parkinson disease (Resting tremor, rigidity, postural instability, normal deep-tendon reflexes, cogwheel rigidity)
Diseases affecting the inner ear & vestibulo-cochlear nerve
- causes hearing loss & gait instability
- common causes: infection, trauma, Meniere disease
Acoustic shwannoma (neoplasm of the cerebellopontine angle)
-lead to cerebellar dysfunction, vestibular dysfunction, & hearing loss
Migraine therapies
Abortive:
- triptans (sumatriptan)
- NSAIDS (naproxen)
- acetaminophen
- antiemetics (metoclopramide, prochlorperazine)
- Ergotamine (dihydroergotamine)
Preventives: (prophylactics)
- Topiramate
- Divalproex sodium
- Tricyclic antidepressants (amitriptyline & venlafaxine)
- Beta blockers (propranolol)
Prophylactics medication is given to patient who:
- frequent (> 4/mnth) or long-lasting (> 12 hrs) episodes
- have disabling symptoms
- no relief with abortive drugs
Migraine
Manifistation:
- episodes of severe, one-side, throbbing headache
- associated with N/V
- preceded by aura (progressive one-sided tingling sensation followed by numbness)
Beta-interferon
- given to Multiple sclerosis ( with relapsing-remitting episodes)
- decrease relapse & brain lesion development
Glucocorticoids
Long term side effects:
- Hyperglycemia
- Osteoporosis
Levetiracetam
- seizure treatment & prophylaxis
Sertraline
- SSRI (Antidepressant)
- not used for migraine
Meningitis
- fever + headache
- focal bleeding + focal neurologic manifistation
- treatment: Lumbar puncture (when toxicology screen is negative)
Cocaine use
- sudden-onset of severe headache
- progressive one-sided weakness
- one-sided facial weakness
- slurred speech
- decrease pinprick sensation in one-side upper/lower extremity
- fever
- tachycardia
- neck is supple (can bend)
- dilated pupil (mydriasis) (sympathetic) + reactive to light
- thalamic hemorrhage with no midline shift
- -
Intracranial hemorrhage (ICH)
Sign:
- headache
- one-sided weakness & hemisensory loss
- chronic HTN
Diagnosis:
- CT
- urine toxicology screen
Treatment:
- manage HTN
- normalize ICP
- prevent further bleeding
Cocaine-use
- Young age
- Absence of chronic HTN
- Acute HTN, tachycardia, hyperthermia, mydriasis ( due to cocaine-induced vasoconstriction preventing heat dissipation)
- Most cocaine-induced ICH seen at the subcortical location (thalamus) & is associated with intraventricular hemorrhage
Alzhiemer disease
SIGNS:
1. Early & permanent memory loss
- family history increases the risk of developing the disease
- modifying medical conditions ( HTN, diabetes, obesity, inactivity) can reduce the risk
- donepezil (cholinesterase inhibitor) to treat dementia
- EEG is used to characterize & stage dementia
Locked in syndrome
- caused by: occlusion in basilar artery
- Location: infarction of bilateral ventral pons
SIGNS:
- Patient can’t move and can’t speak
- Retain consciousness, sensation, eye opening & vertical eye movement
Preserved:
- vertical gaze: superior colliculus (SC)
- sensation: lateral spinothalamic tract (LST)
- Consciousness: midbrain reticular formation (RF)
- brainstem & spinal reflexes
- sensation: dorsal column
Absent:
- horizontal gaze: paramedian pontine reticular formation (PPRF)
- Limb function (=quadraplagia): corticospinal tract (CS)
- speech: corticobulbar tract (CB)
Notes:
- right PPRF = activates right abducens & left oclumotor
Dementia with lewy body (DLB)
Parkinsonism + dementia
SIGNS:
- Confusion (Alter in consciousness)
- Early appearance of dementia
- Visual hallucination
- Parkinsonian motor symptoms (tremor, rigidity)
- Repeated falls & sleep disturbance
Diagnosis:
- lewy body ( eosinophilic intracytoplasmic inclusion = alpha-synuclein protein)
- CT shows atrophy of cortical
Treatment
- Carbidopa-levodopa (for parkinsonism)
- Cholinesterase inhibitors (for cognitive impairment)
- Low-dose second generation antipsychotic (for psychotic symptoms)
Normal-pressure hydrocephalus (NPH)
SIGNS:
- Cognitive changes ( decrease attention & concentration, apathy, dementia)
- Changes in gait
- Urinary incontinence
Caused by:
1. hydrocephalus + normal CSF
DIAGNOSIS:
- MRI shows ventriculomegaly that is out of proportion to the degree of sulcal widening (sulcal atrophy)
Parkinson’s disease
- bradykinesia + either (tremor or rigidity)
- red flags: early postural instability (recurrent falls) indicate other diagnosis than parkinsonism
Pathology:
1. Accumulation of alpha-synuclein within neuron of substania nigra (pars compacta)
Signs: (TRAP)
- Late appearance of dementia
- Rest tremor (asymmetric at distal upper extremity)
- Rigidity (cogwheel)
- Akinesia/bradykinesia (slow movement)
- Postural instability (shuffling gait)
Diagnosis:
- Clinical (TRAP)
- resting tremor: 4-6 Hz in frequency with pill-rolling
- rigidity: oscillating (cogwheel) or uniform (lead-pipe)
- akinesia/bradykinesia:- Difficulty initiating movement, such as rising from a chair, or start walking.
- Narrow-based, shuffling gait
- Micrographia ( small hand writing)
- Masked facies = decrease facial expression/ lack blinking
- Soft speech
- postural instability:
- Flexed axial posture
- Loss of balance when turning or stopping
- Loss of balance when stationary
- Frequent falls
Treatment
- Cognitive impairment: cholinesterase inhibitor ( donepezil)
- Psychotic symptoms: low potency antipsychotics ( pimavanserin, quetiapin) & clozapine
Initial workup of suspected cognitive impairment
Signs of dementia:
Cognitive testing
- MMSE (score < 24/30 suggests of mild cognitive impairment/dementia)
- Montreal cognitive assessment (score < 26/30)
- Mini-cog (abnormal 3-word recall &/or clock drawing test)
Laboratory testing
- Routine: CBC, vitamin B12, TSH , Complete metabolic panel
- Selective: folate (alcohol use), syphilis (exposure), vitamin D (celiac, CKD)
- Atypical (early onset): CSF (for infection or malignancy)
Imaging
- Routine: CT or MRI of brain
- Atypical: EEG (electroencephalogram) (for seizure)
Initial workup:
- Montreal cognitive assessment (nueropsycological testing)
- CBC, Vitamin B12, TSH, CMP
- MRI
Pediatric traumatic brain injury (PECARN rule)
High risk features age < 2:
- Altered mental status (fussy behavior)
- Loss of consciousness
- Severe mechanism of injury (fall > 0.9 m, high impact, MVC)
- Non-frontal scalp hematoma
- Palpable skull fracture
High risk feature age > 2-18:
- Altered mental status (agitation, somnolence)
- Loss of consciousness
- Severe mechanism of injury (fall > 1.5 m, high impact, MVC)
- Vomiting, severe headache
- Basilar skull fracture sign (CSF escape from ear or nose, raccoon eye, battle sign behind ear, halo sign seen in pillow/ ring with blood-csf)
Management:
- CT scan with no contrast
- or observe for 4-6 hours if mental status is normal & no sign of a basilar skull fracture