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Nuerology Shelf Exam UWORLD > Nuerology Shelf Exam > Flashcards

Nuerology Shelf Exam Flashcards

1
Q

Alcoholic cerebellar degeneration

A

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)
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2
Q

Cerebellar ataxia

A

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)

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3
Q

Pyramidal tract disease

A

Manifistation:

  • clonus
  • Clasp-knife spasticity
  • seen in hypertonia
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4
Q

Babiniski sign

A
  • hyperreflexia
  • upward deviation of the big toe when the sole of the foot is stroked
  • evidence of UMN lesion
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5
Q

Bradykinesia

A
  • slowing of movement
  • A hallmark of Parkinson disease (Resting tremor, rigidity, postural instability, normal deep-tendon reflexes, cogwheel rigidity)
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6
Q

Diseases affecting the inner ear & vestibulo-cochlear nerve

A
  • causes hearing loss & gait instability

- common causes: infection, trauma, Meniere disease

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7
Q

Acoustic shwannoma (neoplasm of the cerebellopontine angle)

A

-lead to cerebellar dysfunction, vestibular dysfunction, & hearing loss

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8
Q

Migraine therapies

A

Abortive:

  1. triptans (sumatriptan)
  2. NSAIDS (naproxen)
  3. acetaminophen
  4. antiemetics (metoclopramide, prochlorperazine)
  5. Ergotamine (dihydroergotamine)

Preventives: (prophylactics)

  1. Topiramate
  2. Divalproex sodium
  3. Tricyclic antidepressants (amitriptyline & venlafaxine)
  4. 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
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9
Q

Migraine

A

Manifistation:

  • episodes of severe, one-side, throbbing headache
  • associated with N/V
  • preceded by aura (progressive one-sided tingling sensation followed by numbness)
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10
Q

Beta-interferon

A
  • given to Multiple sclerosis ( with relapsing-remitting episodes)
  • decrease relapse & brain lesion development
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11
Q

Glucocorticoids

A

Long term side effects:

  • Hyperglycemia
  • Osteoporosis
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12
Q

Levetiracetam

A
  • seizure treatment & prophylaxis
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13
Q

Sertraline

A
  • SSRI (Antidepressant)

- not used for migraine

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14
Q

Meningitis

A
  • fever + headache
  • focal bleeding + focal neurologic manifistation
  • treatment: Lumbar puncture (when toxicology screen is negative)
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15
Q

Cocaine use

A
  • 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
  • -
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16
Q

Intracranial hemorrhage (ICH)

A

Sign:

  • headache
  • one-sided weakness & hemisensory loss
  • chronic HTN

Diagnosis:

  • CT
  • urine toxicology screen

Treatment:

  • manage HTN
  • normalize ICP
  • prevent further bleeding
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17
Q

Cocaine-use

A
  1. Young age
  2. Absence of chronic HTN
  3. Acute HTN, tachycardia, hyperthermia, mydriasis ( due to cocaine-induced vasoconstriction preventing heat dissipation)
  4. Most cocaine-induced ICH seen at the subcortical location (thalamus) & is associated with intraventricular hemorrhage
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18
Q

Alzhiemer disease

A

SIGNS:
1. Early & permanent memory loss

  1. family history increases the risk of developing the disease
  2. modifying medical conditions ( HTN, diabetes, obesity, inactivity) can reduce the risk
  3. donepezil (cholinesterase inhibitor) to treat dementia
  4. EEG is used to characterize & stage dementia
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19
Q

Locked in syndrome

A
  • caused by: occlusion in basilar artery
  • Location: infarction of bilateral ventral pons

SIGNS:

  1. Patient can’t move and can’t speak
  2. 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

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20
Q

Dementia with lewy body (DLB)

Parkinsonism + dementia

A

SIGNS:

  1. Confusion (Alter in consciousness)
  2. Early appearance of dementia
  3. Visual hallucination
  4. Parkinsonian motor symptoms (tremor, rigidity)
  5. Repeated falls & sleep disturbance

Diagnosis:

  • lewy body ( eosinophilic intracytoplasmic inclusion = alpha-synuclein protein)
  • CT shows atrophy of cortical

Treatment

  1. Carbidopa-levodopa (for parkinsonism)
  2. Cholinesterase inhibitors (for cognitive impairment)
  3. Low-dose second generation antipsychotic (for psychotic symptoms)
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21
Q

Normal-pressure hydrocephalus (NPH)

A

SIGNS:

  1. Cognitive changes ( decrease attention & concentration, apathy, dementia)
  2. Changes in gait
  3. 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)

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22
Q

Parkinson’s disease

A
  • 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)

  1. Late appearance of dementia
  2. Rest tremor (asymmetric at distal upper extremity)
  3. Rigidity (cogwheel)
  4. Akinesia/bradykinesia (slow movement)
  5. Postural instability (shuffling gait)

Diagnosis:

  1. Clinical (TRAP)
    - resting tremor: 4-6 Hz in frequency with pill-rolling
    - rigidity: oscillating (cogwheel) or uniform (lead-pipe)
    - akinesia/bradykinesia:
    1. Difficulty initiating movement, such as rising from a chair, or start walking.
    2. Narrow-based, shuffling gait
    3. Micrographia ( small hand writing)
    4. Masked facies = decrease facial expression/ lack blinking
    5. Soft speech
      • postural instability:
  2. Flexed axial posture
  3. Loss of balance when turning or stopping
  4. Loss of balance when stationary
  5. Frequent falls

Treatment

  1. Cognitive impairment: cholinesterase inhibitor ( donepezil)
  2. Psychotic symptoms: low potency antipsychotics ( pimavanserin, quetiapin) & clozapine
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23
Q

Initial workup of suspected cognitive impairment

A

Signs of dementia:

Cognitive testing

  1. MMSE (score < 24/30 suggests of mild cognitive impairment/dementia)
  2. Montreal cognitive assessment (score < 26/30)
  3. Mini-cog (abnormal 3-word recall &/or clock drawing test)

Laboratory testing

  1. Routine: CBC, vitamin B12, TSH , Complete metabolic panel
  2. Selective: folate (alcohol use), syphilis (exposure), vitamin D (celiac, CKD)
  3. Atypical (early onset): CSF (for infection or malignancy)

Imaging

  1. Routine: CT or MRI of brain
  2. Atypical: EEG (electroencephalogram) (for seizure)

Initial workup:

  1. Montreal cognitive assessment (nueropsycological testing)
  2. CBC, Vitamin B12, TSH, CMP
  3. MRI
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24
Q

Pediatric traumatic brain injury (PECARN rule)

A

High risk features age < 2:

  1. Altered mental status (fussy behavior)
  2. Loss of consciousness
  3. Severe mechanism of injury (fall > 0.9 m, high impact, MVC)
  4. Non-frontal scalp hematoma
  5. Palpable skull fracture

High risk feature age > 2-18:

    1. Altered mental status (agitation, somnolence)
  2. Loss of consciousness
  3. Severe mechanism of injury (fall > 1.5 m, high impact, MVC)
  4. Vomiting, severe headache
  5. 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
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25
Q

Preventative migraine therapy for pregnant women

A
  • 1st line: beta blocker (propranolol or metoprolol)

- calcium channel blocker ( verapamil)

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26
Q

Cluster headache

A

Signs:

  1. Recurrent severe unilateral headache at peri-orbital, mitosis, ptosis, lacrimation, tearing, rhinorrhea , Conjunctival injection

Management:

Abortive:

  • 100% oxygen by face-mask
  • or subcutaneous sumatriptan ( prevent in: ischemic cardiac disease, pregnancy)

Preventative:

  • verapamil
  • lithium

Timing:

  • occurs at sleep
  • progress rapidly for 90 min
  • up to 8 times daily for 6-8 weeks
  • period of remission
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27
Q

Carbamazepine

A
  • treat trigeminal neuralgia

Signs:

  • recurrent & sudden onset, severe, stabbing pain along the V2 (maxillary) & V3 ( mandibular) branches of trigeminal nerve
  • No miosis
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28
Q

Glucocorticoids

A
  • for temporal arteritis

Sign:

  • age > 50
  • headache with localized temporal tenderness
  • elevated erythrocyte sedimentation rate (ESR)
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29
Q

Enoxaparin

A
  • used for treatment of deep venous thrombosis (blood clot in vessels of leg)
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30
Q

Guillain- barrie syndrome

A
  • autoimmune disease leading to demeylenation of axons in the PNS

Signs:

  • ascending, symmetrical weakness (starts in legs, than spread to arms)
  • facial nerve palsy
  • respiratory paralysis
  • pain can occur
  • occurs after GI illness

Diagnosis:

  • nerve conduction
  • cerebrospinal fluid (CSF) via lumbar puncture —> elevated protein + WCC (< 10 million/L)
      • albuminocytologic dissociation = elevated CSF protein with normal CSF leukocyte count

Risk factors:

  • viral infection ( CMV, HZV, EBV, HIV)
  • surgery
  • upper respiratory tract infection
  • pneumonia
  • flu vaccine

Treatment:
- IV IG

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31
Q

Vascular dementia

A

Signs:

  1. Mild memory loss
  2. Prominent executive dysfunction
  3. Focal neurologic deficits
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32
Q

Carotid artery dissection

A

Common cause of stroke in young patients after trauma (fall) or illness

Sign:

  1. Trauma ( fall)
  2. Unilateral headache
  3. Neck pain
  4. Partial horner syndrome ( ptosis, miosis)
  5. Transient ischemic attack ( transient leg weakness)
  6. NO tinnitus & NO carotid bruits

Risk factors:

  1. Trauma or infection
  2. HTN, smoking, connective tissue disease (Ehlers Danlos), OCP

Diagnosis:
1. CT or MRI angiography

Treatment:

  1. Within first 4 hours: Thrombolysis
  2. After 4 hours: anti-platelet therapy (aspirin)+ anticoagulation
  3. For large vessels (MCA): mechanical Thrombectomy
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33
Q

ALS

A

Pathology:

  1. Sporadic mutation
  2. Combination of UMN lesion + LMN lesion + sensation intact

Signs:

  1. LMN lesion:
    - arryflexia
    - weakness
    - fasciculation
  2. UMN lesion:
    - hyperreflexia
    - Spasticity
  3. Different limbs will do different things (babiniski is different)
  4. Intact sensation

Diagnosis:
1. EMG

Treatment:

  1. Supportive (patient die because all their muscle fail & they can’t breathe) —> use non invasive positive pressure ventilation to improve atalactasis
  2. Riluzole (treatment that give patient 3 months to live)
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34
Q

Myasthenia Gravis

A

Pathology:

  1. Autoimmune disease against Acetyl-choline receptor antibody
  2. Someone over 50

Signs:

  1. Fatigue ( in the muscle of eyes, throat, and fine movement of distal extremities)
    - blurry vision
    - difficulty swallowing
    - lack of hand coordination
  2. Worse in the evening

Diagnosis:

  1. Antibodies ( Ach-receptor antibodies)
  2. EMG ( great amplitude —> fatigue off —> no contraction)
  3. CT (look for Thymoma)

Treatment:

  1. Cholinesterase inhibitors ( to increase Ach)
  2. Steroid ( decrease autoimmune or resistance)
  3. Crisis (can’t swallow, can’t breathe): IvIg (Plasmapharesis)
  4. Thymectomy (remove thymoma)
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35
Q

Lambert-Eaton

A

Pathology:

  1. Paraneoplastic syndrome
  2. Antibodies against synaptic calcium channel
  3. Patient > 50

Signs:

  1. Fatigue in proximal muscle (Muscle used the least are the worse)
  2. Improvement with use
  3. Ex: inability to rise from chair + inability to comb hair + getting things down out of a shelf

Diagnosis:

  1. Antibodies
  2. EMG ( not much contraction —> more attempts —> gets more amplitude)
  3. CT (shows small cell lung cancer)

Treatment:

  1. Treat cancer ( chemoradiation + immunosuppressant)
  2. Symptomatic therapy: Guanidine or 3,4-diaminopyridine ( both increase Ach level)
  3. Refractory therapy: IvIg or immunosuppressant (Corticosteroid, Azathioprine, 6-Mercaptopurine)
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36
Q

Guillain Barre

A

Pathology:

  1. Autoimmune disease
  2. Demyelination disease

Sign:

  1. Initial presentation —> Watery diarrhea or flu-shot pain
  2. Ascending paralysis (symmetrical)
  3. Reach diaphragm = respiratory paralysis
  4. Hypo-reflexia
  5. Sensory intact

Diagnosis:

  1. Nerve conduction
  2. Lumbar puncture = elevated protein + decreased WCC (<10 million/L)
  3. MRI
  4. EMG

Treatment:

  1. Incubation (before LP)
  2. IvIG (similar to plasmapharesis)
  3. Never give steroid (worsen outcome)
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37
Q

Multiple sclerosis

A

Pathology:

  1. Autoimmune disease
  2. Demyelinating disorder
  3. Young women 20-40

Signs:

  1. Any neuro symptoms separated by time & space (relapse then remission)
  2. Optic neuritis ( blurred vision + painful eye with movement)

Diagnosis:

1st line test:
1. MRI : plaques (opacities) at periventricular white matter (Dawson’s fingers)

If MRI was not conclusive —> Less sensitive tests:

  1. Lumbar puncture: oligoclonal IgG
  2. Evoked potential

Treatment:

  1. Flare: steroid (high dose IV-steroid for 3-5 days) (IV methyl-prednisolone)
    - note: plasmapheresis can be performed in patients with MS flare who are refractory to corticosteroid therapy
  2. Chronic:
    - interferon: delay progression
    - glatiraner
    - fingolonod
  3. Disease modifying therapies:
    - interferon beta
    - fumarate
    - biologics ( natalizumab)
  4. Complication:
    • urinary retention: bethanecol
    • urinary incontinence: amitriptyline
      - spasm: baclofen
      - neuropathic pain: gabapentin (lyrica)
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38
Q

Muscle weakness

A
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39
Q

Un-ruptured vs. ruptured intracranial aneurysm

A

Un-ruptured:
1. Headache, facial pain, third nerve palsy (pupillary dilation, ptosis, down/out eye movement)

Ruptured:

  1. Thunderclap headache + nuchal rigidity
  2. Lead to subarachnoid hemorrhage (SAH)
    • acute & severe headache + 3rd nerve palsy
    • vomiting
    • photophobia
    • neck stiffness
    • lethargy
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40
Q

Focal seizure

A

Onset:

  1. Neuronal discharge begin in 1 cerebral hemisphere
  2. Symptoms: motor (twitching), sensory (paresthesia) , or autonomic (sweating)
  3. Structural abnormality (tumor) more likely

Categories:

  1. No impairment of awareness:
    - when seizure remains localized to 1 hemisphere
  2. Impairment of awareness:
    - when seizure spreads to the other hemisphere
    - associated with repetitive automatisms (chewing, picking)
    - staring + no response to verbal or tactile stimuli

Signs:

  • child
  • head tilt + repetitive picking
  • staring + no response to verbal or tactile stimuli
  • after seizure, postictal confusion, lethargy, postictal paresis/paralysis (TODD paralysis; brief period of temporary paralysis )

Diagnosis:

  1. EEG (abnormal electrical activity) vs. interictal EEG (normal)
  2. MRI
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41
Q

Absence seizure

A
  • generalized (originated from both hemisphere)

Signs:

  • staring spell with/without automatisms (repetitive chewing, picking)
  • lasts for 10-20 seconds
  • provoked by hyperventilation
  • not associated with postictal period
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42
Q

Juvenile myoclonic epilepsy

A
  • present in adolescents with myoclonic jerks immediately on wakening
  • absence & generalized tonic-clonic seizures may be seen
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43
Q

Lennox-Gastaut syndrome

A
  • present by age of 5
  • intellectual disability + varying severe seizure (atypical, absence, or tonic)
  • diagnosis: interictal EEG shows slow spike-&-wave pattern
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44
Q

Tic disorder

A
  • sudden, brief movement (grimacing) or vocalization

- intact awareness

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45
Q

Miosis vs mydiarisis

A
  • pupil constriction: miosis

- pupil dilation: mydiarisis

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46
Q

2nd nerve palsy (optic nerve)

A
  • compression of cranial nerve 2
  • by aneurysm of internal carotid artery (ICA) or anterior communicating artery (ACA)
  • signs: unilateral vision loss or bi-temporal hemianopsia
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47
Q

3rd nerve palsy (occlumotor nerve)

A
  • compression of cranial nerve 3
  • by rupture aneurysm of posterior communicating artery (PCA)
  • signs: ptosis, mydiarisis, dow/out eye
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48
Q

4th nerve palsy (trochlear nerve)

A
  • compression of cranial nerve 4

- by aneurysm affecting superior cerebellar (SC)

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49
Q

6th nerve palsy ( abducens nerve)

A
  • compression of cranial nerve 6

- by aneurysm affecting anterior inferior cerebellar artery (AICA)

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50
Q

Cavernous sinus thrombosis

A

Signs:

  1. Fever
  2. Headache
  3. Peri-orbital swelling
  4. Palsy of CN 3, 4, 6 (opthalmoplegia = weakness of eye muscles)
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51
Q

Lateral medullary syndrome (Wallenberg syndrome)

A

Caused by:
- stroke affecting vertebral artery (VA) or anterior inferior cerebellar artery (AICA)

Sign:

  • contralateral sensory deficit of the extremity
  • ipsilateral sensory deficit of the face
  • ipsilateral Horner syndrome
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52
Q

Trigeminal neuralgia

A
  • vascular nerve root compression of V2 & V3 of CN 5 (Trigeminal) as it enters the pons —> leading to demyelination & atrophy of the nerve.
  • compression is caused by: vascular loop, neoplastic growth or Multiple sclerosis plaque.
  • demyelinating plaque (EX: Multiple sclerosis) in the pons causes TN

Signs:

  • unilateral
  • intermittent sharp pain (shock-like stabbing) of cheek & lip
  • lasts for seconds - 2 min
  • triggered by minor stimuli (brush teeth, drink cold water, talking, chewing, light touch)
  • atrophy & demyelination of CN5
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53
Q

Bells palsy

A

Sign:

  • inflammation & edema of facial nerve (CN7)
  • unilateral facial paralysis
  • not pain
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54
Q

Herpetic neuralgia

A

Sign:

  • reactivation of herpes zoster virus
  • preceded by dermatomal pain
  • followed by vesicular rash (after several days)
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55
Q

Sydenham Chorea

A

Signs:

  1. Preceded by Group A strep. Infection
  2. jerky movement (only while awake, not seen in sleeping) (descending & symmetrical limbs)
  3. hypotonia
  4. Behavioral changes (emotional liability)
  5. +/- rheumatic fever
  6. Seen in girls

Diagnosis:

  • via GAS testing with throat culture: Antistreptolysin O (ASO) + antideoxyribonueclease B (Anti-DNAse B) titers
  • investigate Rheumatic fever via: EEG, ECG, inflammatory markers.

Treatment:

  1. Penicillin G
  2. Haloperidol

Prognosis:
- spontaneous resolution + recurrence

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56
Q

Hyperthyroidism

A

Signs:

  1. Symmetrical tremor (vs. asymmetrical in PD)
  2. Heat intolerance
  3. Palpitation
  4. Weight loss
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57
Q

Wilson disease

A

Pathology:

  • autosomal recessive
  • accumulation of Copper

Signs:

  • dysarthria, dystonia, drooling, ataxia
  • parkinsonism (TRAP)
  • hepatic dysfunction (HEPATOMEGALY)
  • Kayser-fleischer rings in eye
  • abdominal pain + neuropsychiatric symptoms related to hepatitis
  • Before age of 35

Diagnosis:

  1. Slit-lamp light: evaluate kayser-fleischer rings
  2. Low serum ceruloplasmin
  3. Elevated LFT

Treatment:
- copper chelation therapy

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58
Q

Sturge-Weber syndrome

A

Signs:

  1. Facial Port-wine stain (in forehead, eyelids, cheek)
  2. Leptomeningeal capillary-venous malformation (angioma of brain & eye)
  3. Glaucoma

Diagnosis:
1. Glaucoma: via tonometry (shows elevated intraocular pressure)

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59
Q

Glaucoma

A

Pathology:

  1. Optic neuropathy: increase IOP
  2. Impaired drainage of intraocular fluid

Signs:

  1. Tearing, Photophobia, Blepharospasm (Excessive blinking due to increase eyelid muscle contraction).
  2. Enlarged globe & cornea
  3. Optic nerve cupping

Diagnosis:
1. tonometry: shows elevated intraocular pressure (IOP)

Treatment:

  1. Surgery (pediatric = to preserve vision)
  2. +/- pressure-reducing eye drops
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60
Q

Peri-orbital cellulitis

A

Signs:

  1. Eyelid swelling
  2. Erythema
  3. Warmth
  4. Tenderness

Treatment:
1. Oral antibiotics

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61
Q

Orbital cellulitis

A

Signs:

  1. Eyelid swelling, erythema, warmth, tenderness
  2. Proptosis (eye bulging)
  3. Pain with eye movement

Treatment:
1. IV antibiotics

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62
Q

Tourette syndrome

A

Signs:

  1. Combination of vocal (throat clearing) & motor ( shoulder shrugging) tics
  2. Peaks at age of 10-12
  3. Tics exacerbated by anxiety, stress, fatigue

Treatment:

  1. VMAT 2 inhibitor (tetrabenzine)
  2. Antipsychotics (receptor blocker)
  3. Alpha 2 adrenergic receptor agonist (guanfacine, clonidine) —> if ADHD presents
  4. (+) behavioral supportive therapy
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63
Q

Huntington disease

A

Pathology:

  • autosomal recessive (CAG repeats)
  • early onset & later symptoms
  • loss of GABA-ergic neurons
  • atrophy of caudate nucleus + putamen

Sign:

  1. Motor
    - chorea + delayed saccade
  2. Psychiatry:
    - depression + irritability + psychosis + Obsessive-compulsive symptoms
  3. Cognitive:
    - executive dysfunction

Symptoms:
-progressive + abnormal movement (chorea)+ dementia+ dystonia + bradykinesia + rigidity+ behavioral change/agitation

Diagnosis:
- MRI shows atrophy of caudate nucleus + putamen

Treatment:
- antisense oligonucleotide therapy + supportive + survival 10-20 years

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64
Q

Phenytoin

A
  • drug used in treatment of generalized tonic-clonic seizure
  • slowly taper & discontinue in patient planning pregnancy or with low risk of recurrence
  • side effect: (fetal hydantoin syndrome; cardiac defect/abnormal facial feature of newborn)
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65
Q

Valproic acid

A
  • treatment of seizure
  • ## associated with congenital anomalies ( neural tube defect/ spina bifida & dysmorphic facial feature)
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66
Q

Evaluation of idiopathic intracranial HTN (IIH)

A

Sign:

  1. Headache + vision changes (optic disc edema)
  2. Obesity + women + childbearing age

Assessment:

  1. Suspected increase ICP:
    - visual acuity/field + funduscopy
  2. Papilledema:
    - evaluate for mass lesion:
    • CT/MRI
    • venography: cerebral venous sinus thrombosis
  3. Negative imaging:
    - lumbar puncture: opening pressure > 250 mm H2O+no sign of infection
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67
Q

Idiopathic intracranial HTN

A

SIGNS:
1. Positional Headache: (worse when laying flat = increase ICP; Improve with sitting = decrease ICP)

  1. Pulsatile tinnitus: (increase vascular pulsation)
  2. Blurry vision: increase pressure on optic nerve (due to increase ICP= papilloedema)
  3. Increase BMI + Women + pregnancy

Diagnosis:

  1. MRI/CT or Venography
  2. Lumbar puncture

Treatment:

  1. Weight loss
  2. Acetazolamide ( to decrease CSF production)
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68
Q

Pre-eclampsia

A

Pathology:

  1. Systolic > 140 or diastolic < 90
  2. In pregnant women
Signs:
1. Systolic > 140 or diastolic < 90 
2. AND proteinuria or end organ damage
Symptoms:
1. Headache + Blurry vision in pregnant women

Diagnosis:
1. 24 hr urine collection for total protein

Complication:
1. Seizure or stroke

Treatment:

  1. Magnesium sulfate
  2. Blood control ( labetalol or nifedipine) ( same as migraine in pregnant women)
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69
Q

Obstructive sleep apnea (OSA)

A

Signs:

  • morning headache
  • excessive daytime sleepiness
  • pregnancy + weight gain + obesity
  • no tinnitus + No papilledema

Diagnosis:
1. Polysomnography

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70
Q

Lateral medullary infarct (wallennerg syndrome)

A

Signs:

  1. Vertigo/nystagmus (vestibular nucleus)
  2. Ipsilateral face loss of pain/temp. (Trigeminal nucleus)+ contralateral body loss of pain/temp (spinothalamic tract)
  3. Bulbar weakness (lower cranial nerves)
  4. Ipsilateral Horner syndrome (descending sympathetic)
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71
Q

Antipsychotic medication effects (dopamine antagonism) in dopamine pathways

A

Pathway :

  1. Mesolimbic: efficacy
  2. Nigrostriatal: extrapyramidal symptoms (acute dystonia, akathisia, parkinsonism)
  3. Tuberoinfundibular: hyperprolactinemia (sexual dysfunction & gynecomastia in men;

Note:

  • dopamine inhibits prolactin release from anterior pituitary gland.
  • antipsychotic (risperidone) causes hyperprolactenimia by blocking dopamine activity in the tuberoinfundibular pathway. Clinical effects of hyperprolactinemia include, amenorrhea, glactorrhea, gynecomastia, & sexual dysfunction.
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72
Q

Seizure

A

Seizure Divided into:

  1. Epilepsy = have repeated seizure
    1. General (entire body) vs. partial (single part)
    2. Complex (LOC) vs. simple (no LOC)
    3. Atonic
    4. Myotonic
    5. Absence
  2. First time seizure (VITAMINS= RISK FACTORS)
    1. Vascular (stroke)
    2. Infection (meningitis, encephalitis)
    3. Trauma (TBI, Brain bleed)
    4. Autoimmune (Lupus)
    5. Metabolic (blood glucose, perfusion, shock, O2, Ca, Na)
    6. Ingestion/ withdrawal (alcohol, benzodiazepine)
    7. Neoplasm
    8. Psych
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73
Q

Seizure vs. syncope

A

Seizure:
1. LOC + Limb jerking + bowl/bladder incontinence + tongue biting + postictal state (pass out/confused)

Syncope:

  1. LOC + Limb jerking + bowl/bladder incontinence + tongue biting + NO postictal state
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74
Q

No drugs for epilepsy if,

A
  1. Normal MRI

2. Normal EEG

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75
Q

How to stop a seizure & manage it

A
  • Status: 1st time seizure that last > 5 min + failure to go to baseline for > 20 min

Steps to stop Status Epileptic:

  1. give benzodiazepine (BDZ) (raluzipam)

if it does not work,
2. IV- fos-phenytoin

if it does not work,
3. Incubated + midozalan + propofol

if it does not work,
4. Phenobarbital (drug-induced coma)

  • EEG (useful during seizure)
  • vitamins
  • give anti-epileptic drugs
    • valproate
    • lamotrigine
    • Levetiracetam (COPRA)
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76
Q

Type of seizure & drug given

A
  1. Atonic
    - No LOC + loss of tone (fall)/ football helmet
    - valproate
  2. Myoclonic
    - No LOC + Unnecessary tone (jerking)
    - valproate
  3. Absence
    - LOC in child + no loss of tone + ADHD + Boy
    - ethosuccimide
  4. Trigeminal neuralgia
    - carbamazepine
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77
Q

Non-convulsive status

A
  • full on generalized seizure

- can’t find a reason why they have seizure & they are not getting better !!

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78
Q

Vertigo (room spinning) investigation

A

Dizziness ?

  1. sensation of blacking out + pass out + LOC
    - presyncope vs. syncope
  2. Sensation of room spinning + unsteady on feet + no LOC
    - vertigo
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79
Q

Vertigo types

A

Peripheral vertigo

  • starts from hearing & balance
  • tinnitus + hearing loss + No brain stem lesion + No focal neurologic deficit
  • no imaging is needed

Central vertigo

  • No tinnitus + No hearing loss + brain stem lesion + FND (cerebellar signs)
  • common + require imaging (MRI)
  • POSTERIOR FOSSA lesion ( MS, Stroke, tumor, abscess, complex migraine, weird seizure)
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80
Q

Peripheral Vertigo types

A

BPPV:

  • PATH: otolith touch hair + brain think you moving
  • Sign: recurrent/reproducible vertigo + last for < 1 min+ get dizzy with sudden movement
  • DX: dix-hallpike
  • TRX epily maneuver (get stone out)

Vestibular neuritis = (no hearing loss) (similar to labyrenthitis = have hearing loss)

  • path: post viral
  • sign: 4 weeks after URI + vertigo for 1-10 min & +/- hearing loss & +/- N/V
  • DX: clinical
  • trx: steroid + meclizine

Menier’s disease

  • path: ?
  • triad: hearing loss+ tinnitus + vertigo between 30min-1hr.
  • dx: clinical
  • trx: salt restriction + thiazide diuretic + meclizine (anti-vertigo drug)
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81
Q

Severe hydrocephalus in a newborn

A

Caused by:
- vitamin K deficiency bleeding (VKDB)

Sign:

  1. first few weeks after birth:
    • easy bruising + mucosal/GI bleeding
  2. 2 weeks-6 months:
    • intracranial hemorrhage
    • obstructive hydrocephalus (bleeding block CSF flow= enlarged ventricle= bulging fontanelle & upward gaze movement)
    • increase ICP ( irritability, vomiting, bradycardia, HTN)

Note:

  • vitamin K activates coagulation factor 2,7,9,10
  • vitamin K deficiency increase risk of spontaneous bleeding
  • intramuscular vitamin K is administered at birth to prevent bleeding
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82
Q

Chiari II malformation

A

Signs:

  1. Lateral ventricular dilation (enlarged ventricle) (obstructive hydrocephalus)
  2. Obstruction of CSF flow through 4th ventricle
  3. Herniation of cerebellum through foramen magnum
  4. Myelo-meningocele
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83
Q

Polycystic kidney disease

A
  • associated with berry aneurysm

- a rupture that leads to subarachnoid hemorrhage

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84
Q

Cardiogenic emboli

A
  • cardioembolism (due to atrial fibrillation) lead to embolic stroke.
  • deficits are maximal at onset (due to rapid occlusion of vasculature) & localized to multiple vascular territories
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85
Q

Evaluation of bladder dysfunction in children

A

Bladder dysfunction in children

  1. Urinalysis
    • positive: treat underlying cause (UTI, diabetes)
    • negative:
      • night-time symptoms: manage nocturnal enuresis
      • daytime symptoms: red-flag for spinal cord anomaly ? (1. Neurologic deficits: low extremity weakness + hypotonia + hyporeflexia; 2. Cutaneous lumbosacral abnormality)
        - yes: do spinal MRI for spinal dysraphism (spina bifida occulta)
        - no: consider vesicoureteral reflux, constipation, behavioral etiologies
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86
Q

Spinal dysraphism (spina bifida occult)

A

Signs:

  1. Lumbosacral dermatologic finding (tuft of hair, dimple, hemangioma, lipoma)
  2. LMN signs: lower extremity weakness, hyporeflexia, sensory loss, hypotonia
  3. Urinary incontinence, recurrent UTI, constipation

Diagnosis:

  1. urinalysis
  2. MRI of the spine (shows spinal cord tethering)

Treatment:
1. Surgical detethering

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87
Q

Lumbosacral radiculopathy

A

+ pain + sensory loss

L2-L4:

  • reflux affected: patellar
  • sensory loss: anteromedial thigh + medial shin
  • weakness: hip flexion + hip adduction + knee extension (quadriceps)

L5
reflux affected: none
- sensory loss: lateral shin + dorsum of the foot
- weakness: foot dorsiflexion & inversion (tibialis anterior) + foot eversion (peroneus) + toes extension (extensor hallucis & digitorum)

S1
reflux affected: achilles
- sensory loss: posterior calf + sole + lateral foot
- weakness: hip extension (gluteus maximus) + knee flexion (hamstring) + foot planterflexion (gastrocnemius)

S2-S4
reflux affected: anocutanous
- sensory loss: perineum
- weakness: urinary or fecal incontinence + sexual dysfunction

Pathology:

  • nerve compression = due to disc herniation (disruption of annulus fibrosis leads to displacement of the nucleus pulposus & compress nerve root)
  • signs: acute onset + exacerbated by cough/sneezing
  • diagnosis of disc herniation: the crossed straight leg raise test (exacerbating of pain with passive lifting of the unaffected leg) (When flexion the extended leg on the unaffected side to around 60° the patient will report the same sharp shooting pain (radicular pain) that they usually experience on the other side. )
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88
Q

Glasgow Coma Scale (eye 4, verbal 5, motor 6)

A
  1. Eye Opening
    Response Spontaneously, 4
    To speech, 3
    To pain, 1
2. Best Verbal Response
Oriented, 5
Disoriented, 5
Inappropriate words, 4 
Incomprehensible sounds, 3 
No response, 1
3. Best Motor Response
Obeys commands, 6 
Localizes pain, 5 
Withdrawal from pain, 4 
Decorticate flexion, 3 
Decerebrate extension, 2 
No movement, 1
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89
Q

A person in coma require complex care and some of the needs might include:

A
  1. Postural management programme preventing deformities, contractures and pressure sores including muscle tone management through positioning, splinting, mobilising, sitting in alternative seating systems
  2. Bladder and bowel management
  3. Respiratory care including secretion management, eg suctioning, tracheostomy management
  4. Percutaneous endoscopic gastrostomy (PEG) Feeding
  5. Management of infections like urinary tract infection, chest infection
  6. Management or prevention of medical and neurological complications like seizures.
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90
Q

Coma

A
  1. An insult occurred that lead to person LOC

FINDING:

  • depressed cerebral function
  • brainstem reflexes present = breathing
  • heart in beating
  • if they are awake they can move & feel

Management:

  • naloxone to reverse opiate induced coma
  • thiamine & D50 for hypoglycemia
  • fluid to support BP
  • oxygen
  1. In order to determine the activity of cerebrum = get EEG
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91
Q

Persistence vegetative state

A
  • have sleep-wake cycle—> open eye, can’t communicate, they move,

FINDINGS:

  • no cerebral function (metabolic or anoxic) = no activity
  • brainstem reflux are intact = person going to breath
  • heart is beating
  • motor function intact
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92
Q

Locked-in syndrome

A
  1. Pontine stroke
  2. Patient is aware & have consciousness

Finding:

  1. Cerebral function present
  2. Brainstem reflex present
  3. Heart is beating
  4. They can feel, but they can’t move
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93
Q

Brain death

A

Findings:

  • no cerebral function = no brain activity
  • no brainstem reflux
  • heart is beating
  • no motor function

Diagnosis:

  • cerebral function = EEG
  • brainstem reflux = reflexes
  • heart = ECG
  • motor = physical exam

Note:

  • brain death = is absence of brainstem reflexes
    1. Corneal reflex
    2. Cold water calorics “ Cold=opposite, warm= same” —> quick beating movement of nystagmus —> shoot cold water, eye moves to water, nystagmus beats to other side. —> if no brainstem, shoot cold water, eye does not move vs. persistence vegetative state, shoot cold water, eye move, no beating nystagmus
    3. Doll’s eye: move head, eyes are fixed
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94
Q

Severe spondylolisthesis

A
  • vertebral body displacement
  • occurs gradually within weeks to months
  • elderly (degeneration) or athletes (overuse injury)
  • associated with 1. Back pain, followed by 2. Neurologic symptoms
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95
Q

Thickening of ligament flavum

A
  • causes: spinal stenosis + cord compression
  • signs: neurologic deficits
  1. Bilateral
  2. Develops over months to years
  3. Walking exacerbate symptoms of neurogenic claudication
96
Q

Multiple sclerosis

A
  • autoimmune disease of demyelinating of the CNS

SYMPTOMS:

  1. Sensory disturbance, motor weakness, bowl/bladder dysfunction
  2. Optic neuritis (painful eye movement), internuclear opthalmoplegia (impaired adduction on lateral gaze)
  3. Lhermitte sign: electrical sensation on limb or back
  4. Uhthoff phenomenon: symptoms worsen with increased body temperature

Risk factors:

  1. Female
  2. HLA DRB1
  3. Low vitamin D
  4. Epstein Barr infection
  5. Geographic location

Diagnosis:

  1. Episodic/progressive symptoms disseminated over time & space (relapse & remission)
  2. Hyperintense lesion on periventricular area on T2 MRI
  3. Oligoclonal IgG bands on CSF analysis (lumbar puncture)

Note:

  • non contrast MRI= evaluates disseminated in space (2 brain area involved)
  • contrast MRI= evaluate disseminated in time (active & chronic lesions)

Treatment:

  1. Flare: high dose IV steroid (methylprednisolone)
  2. Chronic
97
Q

Herpes simplex infection

A
  1. Associated with encephalitis + bell’s palsy
98
Q

Initial management of stroke

A

Acute stoke symptoms (hemiplegia + facial droop..)

  1. Assessment of ABCs
  2. Non-contrast CT of the head + laboratory studies
    1. Hemorrhagic stroke: “ prevent further bleeding”
      - BP control (goal systolic 140-160 mmHg)
      1. Nicardipine (iv)
      2. Labetalol (iv)
        - reverse anticoagulation
      3. Warfarin = vitamin K, 4 factor PCC
      4. Heparin/ LMWH = protamine sulfate
      5. Dabigatran = idarucizumab
      6. Apixaban, betrixaban, edoxaban, rivaroxaban
        = andexanet alfa, 4 factor PCC
        - maintain normal ICP (7-15 mmHg) “reduce risk
        of brain herniation”
      7. Elevate head of bed to 30 degree
      8. Keeping neck in neutral position
      9. Maintain a normal body temperature
      10. Prevent volume overload
      11. Sedation
        6.Osmotic: Hypertonic saline bolus /mannitol
      12. Hyperventilation
      13. Decompressive craniectomy
    2. Ischemic stroke:
      1. Permissive HTN
      2. Possible systemic thrombolytics +
        endovascular thrombectomy
99
Q

Fresh frozen plasma

A
  • is given to replete clotting factors in patient with:
    1. massive hemorrhage or
    2. Reverse the anti-coagulation effect of warfarin (in combination with vitamin K)
100
Q

Transfusion of packed red blood

A
  • improve oxygen delivery to tissue
  • occurs in:
    1. Massive hemorrhage
    2. Hemoglobin < 7 g/dL
101
Q

Median nerve injury

A
102
Q

Peripheral nerve compression

A

Common (peroneal) fibular neuropathy:

Risk factors:

  • immobilization (casts, bedrest)
  • prolonged leg crossing
  • protracted squating

Signs

  • foot drop = impaired ankle dorsiflexion & toes extension
  • impaired sensation (numbness/tingling) over lateral shin & dorsal foot
  • preserved planterflexion & reflexes

Diagnosis:

  • electromyography (EMG)
  • nerve conduction studies

Treatment:

  • reduce pressure on the nerve
  • ankle-foot orthosis splint
  • physical therapy
103
Q

Neurofibromatosis (NF) type 2

A

Genetics:

  • autosomal dominant
  • NF2 gene on chr. 22
  • merlin gene ( tumor suppressor)
  • variable expressivity (at age 20-30)

Symptoms:

  • bilateral vestibular schwannomas
  • meningioma
  • spinal tumor (shwannomas, ependymomas)
  • cataract
  • cutaneous tumors or skin plaques

Tumor surveillance:

  • audiogram —> initial to screening test to diagnose acoustic neuromas
  • opthalamologic evaluation
  • MRI of brain/spine

Signs: (of vestibular schwannomas)
- bilateral (sensorineural) hearing loss = cochlear nerve
-imbalance = vestibular nerve
- no vertigo = due to slow tumor growth = compensation
-

104
Q

Anterior spinal syndrome

A
  • thoracic aortic aneurysm repair can cause spinal cord ischemia, especially in the anterior cord

Sign:

  • loss of spinothalamic tract ( pain, temperature, crude touch)
  • loss of lateral corticospinal tract (UMN)
  • Loss of anterior horn ( LMN)
  • level of injury (just above umbilicus = T12)
  • related to surgery involving aortic aneurysm repair (thoracic aortic aneurysm repair)

Symptoms:

  • distal, bilateral loss of motor control (flaccid paralysis/LMN signs initially, followed by spasticity/hyperreflexia/UMN signs )
  • loss of pain, temperature & crude touch sensation in the lower extremities
  • urinary retention (damage to descending autonomic tract)
  • intact vibration, proprioception, & light touch ( dorsal column tract) (preserved posterior spinal artery —> vertebral/PICA—> normal perfusion to posterior spinal cord—> intact dorsal column tract)
105
Q

Central cord syndrome

A
  • develops due to hyper-flexion of neck (during incubation) + cervical spindylosis (degenerative changes in the spine)
  • greater motor impairment in the upper extremities compared to lower extremities

Signs:

  • loss of pain + temperature sensation of upper extremities
  • weakness in upper extremities > lower extremities
106
Q

Spinal cord compression

A

Sigs:
- lower extremities motor + sensory deficits

Caused by:
- epidural hematoma = puncture of the spinal dura

107
Q

Normal pressure hydrocephalus

A

Pathology:

  • decrease CSF absorption by arachnoid granulation
  • NPH is associated with meningitis + subarachnoid hemorrhage
  • NPH result in periventricular ischemia + increased venous resistance
  • Extra CSF in the ventricle = ventricles expand to prevent increase ICP = characteristics, ventriculomegaly out of proportion to sulcal enlargement

Signs:

  • gait change ( wide-based + frequent falls)
  • memory changes (forget familiar places/ can’t recall after 5 min)
  • urinary urgency
  • depressed affect (frontal lobe compression)
  • UMN signs (hyperreflexia/spasticity/ + Babniski/ increase tone) in the lower extremities

Diagnosis:
- Miller Fisher (lumbar tap) test = improved gait with spinal fluid removal

  • MRI = enlarged ventricle out of proportion to underlying brain atrophy

Treatment:
- ventriculoperitoneal shunting

108
Q

Parkinsons vs. NPH vs. Alzahiemer

A

Parkinsons:

  • bradykinesia + either tremor (pill-rolling) or rigidity (cogwheel)
  • later: postural instability (falls) + memory impairment
  • accumulation of alpha-synuclein within dopaminergic neurons

NPH:

  • gait change + memory change + urinary urgency
  • accumulation of CSF within ventricles + No ICP increase

Alzehimer’s:
- early memory impairment
Later: gait + urinary urgency
- accumulation of amyloid plaques

109
Q

Vitamin B 12 deficiency

A

Causes:

  • dementia
  • subacute combined degenerative (SCD) with ataxia & inconsistence

Signs:

  • bilateral lower extremity paresthesia
  • glossitis
  • macrocytic anemia
110
Q

Status epileptic management

A

Pathology:
- when seizure persists for more than 5 min or does not respond to 1st & 2nd line anti-convulsant —> patient is in status epilepticus

  • untreated SE is fetal or leave patient with severe neurologic problem

Management: (goal = get the seizure under control within 30 min)

  1. Assessment of ABCs (mask oxygen, pulse oximetry) (incubation is not necessary if oxygenation is good) (if not incubated put patient in the lateral position to prevent aspiration)
  2. Obtain finger-stick glucose, CBC, metabolic panel ( Mg, Ca, Na), LFT, ABG, level of anti-convulsant, toxicology screen
  3. Two large -bore IV + run maintenance normal saline
  4. If patient have no know seizure disorder or definitive etiology —> give: 100mg IV thiamine + 50mL 50% dextrose
    • note: don’t give dextrose, if finger-stick glucose is
      in normal range or is elevated
  5. Start first line anti-convulsant (Benzodiazepine)
    • 4 mg IV Lorazepam
    • OR 10 mg IM Midazolam (or diazepam)
    • repeat if no response within 30 sec or so
  6. If there is no response within 1 min to the 2nd dose of 1st line anti-convulsant —> go to 2nd line anti convulsant
    • 15-20 mg of Phenytoin equivalent
      (fosphenytoin) IV at a rate of 150 PE/min
    • OR 15-20 mg/kg phenytoin IV at rate of 50 mg/
      min
    • NOTE: Exceeding the dose lead to cardiac
      complication
    • A further dose of phenytoin (10 mg/kg IV rate 50
      mg/min) can be given if continued seizing
      despite administration of 1st dose
  7. Alternative 2nd-line anticonvulsant include:
    • Levetirazetam
    • valproate
    • phenobarbital
  8. If patient is not responsive to 1st & 2nd line medication or seizure is not seizing for more than 30 min, incubate patient, and administer general anesthesia
    • 0.2 mg IV load of Midazolam, then 0.2-0.6 mg/kg/
      hr. Infusion
    • 2 mg IV load of Propofol, then 2-5 mg/kg/hr
      infusion
  9. Patient responded to 1st or 2nd line should get = EEG
  10. Non contrast CT scan = after stopping seizure
  11. Pre- & post -contrast MRI
  12. If patient is still unresponsive & definitive etiology cant be determined = consider-non-convulsant status epileptics (NCSE)
    - Order 24 Hr EEG
Note:
Types of atypical status epilipticus:
1. Focal motor status
2. Absence status
3. Partial complex status
111
Q

Approach to COMA (early management of acute confusional state)

A

Early management:

  1. Assessment of ABCs
  2. Order laboratory tests (CBC, toxic, metabolic panel)
    - electrolyte disturbance, sepsis, vitamin deficiencies (wernickes encephalopathy), poisoning…
    1. Activated charcoal where appropriate
    2. 100mg IV thiamine + 50 mL Iv D50W
    3. BDZ overdose: 0.2 mg/min IV Flumazenil, 1mg
      max
    4. Opioid overdose: 0.4-2 mg IV naloxone (0.8 mg/
      KG/hr infusion)
  3. Neurological exam:
112
Q

Neuroanatomy of alertness, coma exam, & herniation syndromes

A
113
Q

Intracranial pressure crisis & cerebral edema

A
114
Q

Pathological differential diagnosis

A
115
Q

Outcome & brain death declaration

A
116
Q

Herniation

A
  1. Medial frontal lobe —> subfalcine
  2. Diencephalus —> central herniation into midbrain
    - shows: cheyne stokes: periods of hyperventilation + apnea alternating
    - small pupil + reactive = diencephalic pupil
    - brain stem reflex normal
    - paratonic = resist exam
    - symmetric
  3. Uncal herniation:
    - assymetric
    - dilation of ipsilateral pupil
    - ipsilateral eye down/out
    - contralateral motor effect
117
Q

Frontotemporal dementia

A
  • loss of frontotemporal neurons
  • degeneration of frontal/temporal lobes

Signs:

  1. Progressive dementia
  2. Changes in personality/behavior
  3. Aphasia
118
Q

Idiopathic intracranial HTN (caused by papilledema + increased ICP)

A

Signs:
1. obese/female
2. headache (worse with recumbent position, at night, after waking up, during straining for bowl motion, heavy lifting)
3.bilateral transient visual changes/loss
4.enlarged physiologic blind spot (Papilledema)
- due to bulging optic disc = due to CSF +
Increased ICP

Treatment:
- acetazolamide = reduce CSF production

119
Q

Amaurosis fugax

A
  • painless, rapid, & transient monocular vision loss
  • caused by retinal ischemia from atherosclerosis emboli
  • patient have vascular risk factors: smoking, hyperlipidemia
120
Q

Acute glaucoma

A

Signs:

  1. Corneal edema/cloudiness
  2. Fixed/middilated pupil
  3. Conjunctival redness
  4. Eye pain
121
Q

Uveitis

A

Anterior uveitis:

  1. Eye pain + redness +photophobia
  2. Signs: ciliary flush (perilimal injection) + hypopyon (liquid)

Posterior uveitis:
1. Painless + floaters + reduced visual acuity

122
Q

Aortic dissection

A

Type A:

  • signs: neck pain + headache + ischemic stroke
  • vertebral artery dissection
  • affect posterior spinal artery
  • lead to: loss of vibration/propioception/light touch below lesion + minimal weakness

Type B:

  • signs: bilateral paralysis+loss of pain/temp/crude touch/ urinary retention/ intact vibration
  • thoracic artery aneurysm
  • affect anterior spinal artery
  • anterior spinal cord ischemia
123
Q

Exertional heat stroke

A
  • exposed to hot environment while performing activities

Signs:

  1. Temperature > 40
  2. Altered mental status (confused)
  3. Tachycardia, tachypnea, flushing
  4. Complication:
    - rhabdomyolysis
    - DIC : epistaxis (nose bleed)
    - End organ dysfunction

Management:

  • patient stabilization (ABCs)
  • rapid cooling techniques (ice water immersion)
  • fluid resuscitation
  • treatment of End organ dysfunction
124
Q

Meningitis

A

Signs:

  • fever
  • altered mental status
  • DIC
  • Neck rigidity
125
Q

Salicylate toxicity

A

Signs:

  • fever
  • altered mental status
  • N/V
126
Q

Malignant hyperthermia

A

Signs:

  • anesthesia use (halothane, succinylcholine)
  • diffused rigidity
127
Q

Vertigo

https://www.youtube.com/watch?v=YMWZRwWIHZ4

A

Peripheral vertigo:

etiology:
1. BPPV (vertigo with head position changes, less than 1 min vertigo + diagnose with Dix-hall-pike maneuver + treated with epely maneuver)
2. Meniere disease (fluctuating hearing loss + episodic vertigo + tinnitus)
3. vestibular neuritis (continuous vertigo w/horizontal nystagmus after URI)
4. Labyrinthitis: continuous vertigo w/horizontal nystagmus after URI + hearing loss + treated with meclizine/motion sickness)

nystagmus:
1. Never purely vertical (horizontal or transverse)
2. Inhibition by fixation of gaze
3. Fatigable (<1min duration)
4. Latency period (2-40 sec)

Neurologic signs:

  1. Hearing loss + tinnitus
  2. Preserved walking
  3. No CNS

Central vertigo:

etiology:
stroke (cerebral), multiple sclerosis, migraine

Nystagmus:

  • any trajectory (vertical mostly)
  • not inhibited by gaze fixation
  • not fatigubale (> 1 min duration)
  • no latency period

Neurologic symptoms:

  • no hearing loss or tinnitus
  • severe postural instability (recurrent falls)
  • CNS signs: headache, diplopia, weakness/numbness of face or limbs, dysarthria

Diagnosis:
Noncontrast CT of head (evaluation of cerebellar stroke, hemorrhage)

128
Q

Chronic back pain in adolescence

A

Malignancy:

  1. Nocturnal pain
  2. Systemic symptoms (weight loss..)
  3. Neurologic symptoms (weakness/ hyporeflexia)
  4. New onset scoliosis

Tethered spinal cord (closed spinal dysraphism)

  1. Back/buttlock/leg pain worse with activity & improves with rest
  2. Neurologic symptoms: LMN signs (weakness/ hyporeflexia)
  3. New onset scoliosis
  4. Lumbosacral cutaneous abnormality (lipoma)
  5. Muscle atrophy + foot drop + bladder/bowel dysfunction
  6. MRI of spine is diagnostic

Chronic multifocal osteomyelitis:

  1. Nocturnal pain
  2. Systemic symptoms

Spondylosis & spondylolisthesis:

  1. After repetitive trauma (gymnastics)
  2. Pain with extension

Ankylosis spondolytis

  1. Morning stiffness
  2. Pain worse at rest
  3. Sacroiliac joint tenderness

Disc herniation:

  1. Due to trauma
  2. Lower back pain, worse with flexion, radiated to leg
129
Q

Classification of herpes zoster pain

A

Acute herpetic neuralgia:

  1. Persist < 30 days after skin rash onset
  2. Treat: analgesia, NSAIDS

Subacute herpetic neuralgia:

  1. Persist > 30 days, but resolves within 4 months of rash onset
  2. Treat: Analgesia, NSAIDS

Post-herpetic neuralgia:

  1. Persist > 4 months from rash onset
  2. Treat: Gabapentin, tricyclic antidepressant, pregabalin
130
Q

Evaluation of delirium

A

1) positive confusion assessment method (CAM)
- acute onset + fluctuation + inattention + disorganized thinking + altered consciousness

2) History ( pain, trauma, substance use, dehydration, urinary retention) + physical examination ( Vital signs, cardiopulmonary, neurologic, skin) + medication ( opioids, anticholinergic + BDZ) + data ( Complete metabolic panel, LFT, CBC, ECG, Urinalysis)

3) infectious:
- culture (urine, blood) + urinalysis
- chest xray: hypoxia, crackles
- lumbar puncture: meningismus

4) metabolic:
- ABC: hypoxia, acidosis
- Ammonia: liver failure
- TSH: suggestive symptoms (Hyper/Hypo)

5) neurologic:
- CT scan/MRI: focal deficits/ anticoagulation
- EEG: seizure history
- Toxicology screen: suggestive history

131
Q

Management of multiple sclerosis

A

1) depression:
- SSRI
- SNRI

2) fatigue:
- modafinil
- methylphenidata
- amantadine

3) neuropathic pain:
- gabapentin —> used for restless leg syndrome (urge to move leg, relief with movement)
- duloxetine —> depression/peripheral neuropathy

4) urinary urgency:
- oxybutynin
- tolterodine

5) spasticity:
- beclofen
- tizanidine

132
Q

Metformin vs vitamin B12 deficiency

A
  • long term (> 5 years) use of metformin can lead to vitamin B12 deficiency due to alternating in calcium hemostasis. —> lead to impaired absorption of Vitamin B12 at terminal ileum
  • signs:
    1) megaloblastic anemia (low MCV)
    2) lower extremity Paresthesias (myelinated peripheral nerves)
    3) impaired vibration/proprioception/light touch & sensory ataxia (dorsal column tract) ( gait impairment that worsen with eye closed —> positive romberg sign)
    4) neuropsychiatric changes (Irritability, crying spells)
    5) positive babniski sign (lateral cortical tracts)
133
Q

Parkinson disease vs. essential tremor

A

Parkinson disease:

  1. Rest tremor: (occurs at rest, improves with activity)
  2. Pill-rolling
  3. Affects hands or legs
  4. Often asymmetric
  5. Associated with rigidity + bradykinesia
  6. Drugs:
    - levadopa
    - dopamine agonist (pramipexole)
    - anticholinergic (trihexyphenidyl)

Essential tremor:

  1. Action tremor (occurs with activity)
  2. Hands, head, voice
  3. Usually bilateral
  4. Associated with: family history, improves with alcohol
    5) drugs:
    - propranolol
    - primidone
134
Q

Acute intermittent porphyria (AIP)

A

Pathology:

  1. Autosomal dominant
  2. Disorder of heme pathway
  3. Reduced activity of porphobilinogen deaminase

Triggers:

  • alcohol, tobacco
  • progesterone containing drugs
  • surgery, fasting

Signs:

  1. Abdominal pain
  2. Weakness of proximal upper extremity + areflexia
  3. Autonomic dysfunction (hypertension, tachycardia, diaphoresis, tremor)
  4. Neuropsychiatric symptoms ( hallucination, anxiety, psychosis, restlessness)
  5. Red-tinged urine that oxidize with light/air exposure

Diagnosis:

  1. Elevated serum & urine: PBG, ALA, porphyrins
  2. +/- hyponatremia, elevated transaminases
  3. Positive urobilinogen

Management:
1. Glucose & hemin (heme analogue)

135
Q

Lead poisoning

A

Signs:

  1. Abdominal pain
  2. Neuropsychatric symptoms
  3. Anemia
  4. Joint pain
  5. Fatigue
136
Q

Friedreich ataxia

A

Pathology:

  • autosomal recessive
  • GAA repeat
  • abnormal frataxin protein (expressed in brain/heart/pancreas)

Lead to:

  • neurologic dysfunction (brain) (dysarthria, nystagmus, loss of DTR, Progressive gait & limb ataxia in adolescent)
  • cardiomyopathy (heat) (age of death 30-40)
  • diabetes (pancreas)

Signs:

  1. Kypho-scoliosis
  2. Hypertrophic cardiomyopathy
  3. High arch (pes cavus + hammer toes)

Symptoms:

  1. Dorsal column: loss of vibratory/proprioception/light touch
  2. Dorsal root ganglion: decrease DTR
  3. Lateral corticospinal: spastic weakness
  4. Spinocerebellar: ataxia

Diagnosis:
1) genetic testing

Management:

1) physical therapy
2) support

137
Q

Nuerofibromatosis type 1

A

Pathology:

  • autosomal dominant

Signs:

1) rash:
- cafe au lait macules (chest/ arm)
- freckles at axillary & groin
- neurofibromas (thigh)
2) scoliosis (spinal cord vertebrae)
3) pseudoarthrosis (long bones, tibia)
4) optic glioma (eyes)
5) Lisch nodules (eyes) (iris hemartoma)

Symptoms:

1) headache (worse in the morning, due to increase ICP while laying supine at night)
2) decrease visual acuity
3) seizure + intellectual disability

Increase risk for:

  • intracranial mass —> assess with MRI of brain/eye
  • astrocytoma
  • brainstem glioma
138
Q

Lumbar puncture

A
  1. Rule out: meningitis or encephalitis
  2. Carry risk for brain herniation
  3. Should be considered only if brain imaging excludes space-occupying mass, which should be suspected in patient with morning headache
139
Q

Causes of peripheral neuropathy

A

Signs:

  1. Burning pain
  2. Paresthesia
  3. Ataxia
  4. Stock-glove pattern
  5. Loss of DTR + light touch + vibration senses
140
Q

Parkinson disease symptoms

A

Motor deficits:

  1. anticholinergic drugs (trihexyphenydil)
  2. Amantadine
  3. Dopamine agonist (pramipexole)
  4. MAO-inhibitor
  5. COMT inhibitor
  6. Levadopa

Mood/anxiety:

  • SSRI
  • SNRI

Psychosis:

  • anti-psychotic agents ( pimavanserin, quetiapin)
  • cholinsterase inhibitor (donepezil)

Sleeping disturbance:

  • melatonin
  • trazodone
  • quetiapin
  • mirtazapine

Cognitive impairement:

  • acetylcholinesterase inhibitor (donepezil)
  • NMD receptor antagonist ()
141
Q

Headache types

A
  1. Tension-like headache:
    - worsen by stress
    - bilateral & band-like pain
    - no visual changes
  2. Cluster headache:
    - unilateral & orbital or temporal pain
    - associated with ptosis, miosis, & lacrimation
    - no blurry vision
    - resolution of symptoms (<3 hrs)
142
Q

Memory cognition impairement

A
  1. Frontotemporal dementia:
    - Early personality/ behavior changes
    - late memory deficit
    - manifest around age 60 (earlier than AD)
  2. Dementia with lewy body:
    - dementia + Parkinsonism ( together)
    - visual hallucination & REM-sleep behavior disorder
  3. Normal pressure hydrocephalus (NPH):
    - early gait change (broad base/shuffling) + memory change + urinary urgency
  4. Vascular dementia:
    - stepwise decline is classic
    - early deficits in executive function > memory deficit
    - cerebral vascular disease ( atherosclerosis, arteriosclerosis, cerebral amyloid angiopathy)
    - Ex: rapid development of memory loss, executive dysfunction, & behavioral changes (apathy, paranoia, social withdrawn) 4 months after a stroke
    - treatment: control HTN/diabetes + anti platelet + cholinesterase inhibitors
  5. Alzheimer disease:
    - early anterograde memory loss (immediate recall affected, distant memory preserved), late behavioral/personality changes
    - visuospatial deficit (lost in own neighborhood)
    - language difficulties (difficulty finding words)
    - cognitive impairment with progressive decline
    - Late findings:
  6. Hallucination, wandering
  7. Difficulty performing learned motor task
  8. Urinary urgency
  9. Personality/ behavioral change
143
Q

Migraine

A
144
Q

Muscular dystrophies

A

Duchenne muscular dystrophy (DMD):

  1. X-linked recessive
  2. Deletion of Dystrophin gene
  3. Age 2-3
  4. Signs: proximal muscle weakness (Gowers sign) + calf pseudohypertrophy
  5. Associated with: cardiomyopathy + arrhythmia + scoliosis
  6. Adolescent in wheelchair + death by age 20-30 from respiratory or heart failure

Becker muscular dystrophy (BMD):

  1. X-linked recessive
  2. Deletion of Dystrophin gene
  3. Age 5-15
  4. Signs: milder proximal muscle weakness compared to DMD
  5. Associated symptoms: cardiomyopathy + arrhythmias
  6. Death by age of 40-50 due to heart failure

Classic myotonic: (muscle pain, wasting, delayed relaxation)

  1. Autosomal dominant
  2. Trinucleotide in DMPK gene (CTG repeat)
  3. Age 12-30
  4. Signs: Facial & distal muscle atrophy + Grip (hand) myotonia
  5. Associated with: cardiomyopathy, arrhythmia, dysphagia, cataract, testicular atrophy
  6. Death from 45-55 due to respiratory or heart failure
145
Q

Lesion in basal ganglia

A
  1. Shuffling gait as seen in Parkinson disease
146
Q

Cerebral lesion

A

Type of cerebellar ataxia:

  1. Lesion of the vermis = truncal ataxia
  2. Lesion of the cerebral hemisphere =limb ataxia

Type of ataxia:
1. Staggering & swaying from side to side

147
Q

Pyramidal tract or corticospinal tract (CST) lesion

A
  • spastic ataxia
  • the gait appears stiff or rigid with circumduction ( the spastic leg leg is abducted & advanced while in extension & internal rotation) & planter flexion of the affected limb.
148
Q

Damage to the cortico-cortical white matter fiber of the frontal lobe

A
  • gait apraxia ( Bruns ataxia)
  • seen in normal pressure hydrocephalus (NPH)
  • Strength, coordination, & sensory function are intact, but difficulty initiation of forward movement of the feet, when they are in contact with the ground (magnetic gait)
149
Q

Loss of proprioception due to sensory neuropathy

A
  • slap gait
  • gait instability + wide-based gait
  • patient may stomp their feet against the floor (slap gait) to help them know where their lower limbs are relative to the ground
150
Q

Common peroneal neuropathy lesion caused by L5 radiculopathy/neuropathy

A
  • high steppage gait due to foot drop

- patient flex hip & knee to raise the foot & avoid dragging the toe with each step

151
Q

L5 radiculopathy vs neuropathy

A

L5 radiculopathy:

  1. back pain
  2. Weakness in eversion + dorsiflexion

Peroneal neuropathy:

  1. Compression of nerve at head of fibula
  2. Due to crossing/squatting
  3. Paresthesia + sensory loss over dorsum of foot
  4. Normal foot inversion + planter flexion (tibial nerve)

Diagnosis:

  1. EMG
  2. Nerve conduction studies
152
Q

Acute labyrinthitis & Meniere disease

A
  • vestibular gait
  • unsteady, falling to one side
  • normal sensation, reflexes & motor strength
  • Nausea
  • vertigo
153
Q

Traumatic brain injury

A

High- risk factors: require CT to rule out intracranial hemorrhage

  1. Altered mental status
  2. LOC
  3. Trauma
  4. Headache + vomiting
  5. Basilar skull fracture ( raccoon+ battle signs/ CSF & blood from nose)

Resolved high-risk: require 4-6 hrs observation
- no altered mental status + no signs of basilar fracture + resolved high-risk symptom (vomiting)

Low-risk: discharge home

154
Q

Torticollis

A
  • focal dystonia of (sternocleidomastoid) muscle
  • sustained muscle contraction resulting in twisting, repetitive movement, or abnormal posture
  • discontinue of causative agent= improvement
155
Q

Chorea

A
  • brief, irregular, unintentional muscle contraction

- movement tend to flow from one to another but are not repetitive or rhythmic

156
Q

Akathisia

A
  • sensation of restlessness that causes the patient to move frequently
157
Q

Athetosis

A
  • refers to slow, writhing movement that affects the hand & feet
  • occurs in Huntington disease ( + chorea)
158
Q

Hamiballismus

A
  • unilateral, violent arm flinging
  • caused by damage to the contralateral sub-thalamic nucleus
  • disruptive & self-limited
159
Q

Myoclonic

A
  • involuntary jerking movement
  • rhythmic or patterned ( opposite to chorea)
  • initiated by contraction or relaxation
160
Q

Shaken baby syndrome

A

Caused by:
- repetitive shaking of the baby (acceleration-deceleration) forces lead to subdural bleeding (hematoma) due to shearing of the bridge veins & coup-contecoup injury

Signs:

  • seizure (tonic-clonic)
  • increasing head circumference
  • bulging/tense anterior frontanelle
  • papilledema (blurred optic disc margin)
  • retinal hemorrhage
161
Q

Intraventricular hemorrhage

A
  • shows in premature babies, in the first few days
  • lead to hydrocephalus ( obstruction of CSF flow)
  • shows seizure + bulging fontanelle
162
Q

Retinitis pigmentosa

A

Etiology:

  1. Genetic mutation causing loss of photoreceptors
  2. Progressive retinal degeneration
  3. Symptoms onset from age 10 through adulthood

Feature:

  1. Night blindness
  2. Progressive visual field loss ( mid-periphery defect/ high density of rod)
  3. Decrease visual acuity (late finding)

Fundoscopic finding:
1. Retinal vessels attenuation + optic disc pallor + abnormal retinal pigmentation

Prognosis:
- legally blind by age of 40

Treatment:
1. Measure to slow disease progression = omega-3 fatty acid

163
Q

Vitamin A deficiency

A

Signs:

  1. Excessive dryness of cornea + conjunctiva
  2. Night blindness
164
Q

Retinal detachment

A

Signs:

  1. Acute vision loss
  2. Floaters
  3. Flashes of light

Fonduscopic finding:
1. Retinal tear &/or elevation

165
Q

Open- angle glaucoma

A

Signs:

  1. Painless peripheral vision loss ( in late adulthood)
  2. Otic disc cupping
166
Q

Initial management of ischemic stroke

A

Ischemic stroke:

  1. Symptoms + non/contrast CT of head (consistence)

Time since symptoms onset:

1) <4.5 hr: give thrombolysis (tPA)
2) > 4.5 - 24 hrs: not eligible for Thrombolysis

  • obtain CT angio of head/neck
    • ) large vessel occlusion: mechanical thrombectomy
    • *) absent of large vessel occlusion: antiplatelet therapy: aspirin/clopidogrel + investigate for embolic source

3) > 24 hrs: not eligible for thrombolysis
- standard post-ischemic stroke management (anti-platelet therapy: aspirin/clopidogrel + investigate for embolic source)

NOTE:
- When ischemic stoke causes massive cerebral edema & hemorrhagic transformation = malignant hemispheric infarction (MHI)

167
Q

Thrombolytics administered

A

-

168
Q

Stroke due to cardioembolic event (atrial fibrillation)

A

initiate:
- Anti-platelet therapy

To rule out AF:

  • ECG
  • telemetry

Long-term stroke prevention:

  • oral anticholinergic:
    1) rivaroxaban
    2) apixaban
    3) warfarin
169
Q

Restless leg syndrome

A

Signs:

  • urge to move leg
  • Discomfort improves with movement (dysesthesia)
  • Discomfort increase at rest/night

Associated with:

  1. Pregnancy
  2. Iron deficiency
  3. Diabetes
  4. Uremia
  5. PD & MS
  6. DRUGS: antidepressant, antipsychotics, antiemetics

Treatment:

  • iron supplement (if ferritin < 75 ng/mL)
  • mild symptoms/ intermittent: carbidopa-levadopa
  • frequent symptoms: gebapentin or pregabalin (calcium channel blockers)
  • avoid (dopamine aginists: pramipexole or ropinirole)
170
Q

Compressive neuropathy (sciatica & meralgia paresthetica)

A
  • common in pregnancy

Signs:

  • numbness
  • motor weakness
  • not immediately relieved with movement
  • pain is burning & shock-like
171
Q

Tuberous sclerosis complex (TSC)

A

Scenario:
- child with refractory epilepsy + development delay + subependymal nodule + hypopigmented macules

Pathology:

  • autosomal dominant
  • mutation in TSC 1 or TSC2 gene (inherited or de novo)
  • lead to benign tumors (hemartomas) of brain, skin & other organs

Feature:

  1. Dermatology:
    - ash-leaf spots
    - angiofibromas of malar region (facial)
    - shagreen patches
  2. Neurologic:
    - CNS lesions ( subependymal tumors/nodules)
    - epilepsy (infantile spasm, tonic-clonic)
    - intellectual disability / development delay (in refractory epilepsy)
    - autisms or behavioral disorder (hyperactivity)
  3. Cardiovascular:
    - rhabdomyomas
  4. Renal:
    - angiomyolipomas (benign tumor of kidney: anemia, fever, pain or HTN)

Surveillance:

  • regular eye & skin examination
  • serial MRI of brain & kidney
  • serial ECG & baseline Echo
  • baseline EEG
  • neuropsychatric screening
172
Q

Huntington disease

A
173
Q

Kluver-Bucy syndrome

A

Cuased by:

  • bilateral temporal lobe lesion
  • especially in, hippocampus & amygdala

Signs:

  1. Excessive touching + hyper-sexuality
  2. Bulimia
  3. Placidity
  4. Visual agnosia
  5. Amnesia
174
Q

Ventral brainstem injury

A
  • lead to “locked-in syndrome”
  • signs: qaudraplagia + inability to speak + inability to swallow + able to blink & move eyes vertically + conscious preserved
175
Q

Central cord syndrome

A
  • sings:
    1. Decrease sensation & motor of arms + Sparing of legs + bladder dysfunction
    2. Neck pain + stiffness
    3. Caused by Hyperextension (falling, whiplash)
    4. Seen in elderly with cervical spondylotic myopathy
176
Q

Anterior cord syndrome

A

Signs:

  1. Loss of pain/temperature (1-2 levels below injury level)
  2. Bilateral hemiparesis (at injury level)
  3. Intact vibratory/proprioception/light touch
177
Q

Major Depressive Disorder (MDD)

A

Signs:

  1. Increasing forgetfulness
  2. Low energy + motivation
  3. Sleep disturbance
  4. Psychomotor retardation (slow speech, thinking, movement)
178
Q

MDD vs AD:

A

MDD vs. AD:
- in AD (dementia + apraxia + agnosia + aphasia)

  1. aphasia: loss of the ability to understand and express speech.
  2. agnosia: inability to recognize people, objects, sounds, shapes, or smells.
  3. apraxia: inability to have purposeful body movements.
  • in AD, patients are less concern about their memory loss + brought by family member for evaluation
179
Q

Syringomyelia

A

Pathology:

  • disruption of CSF drainage from the central canal
  • formation of fluid-filled cavity (syrinx) that compress surrounding tissue
  • seen with:
    1. Arnold -chiari type 1 malformation (extension of the cerebellar tonsils into foramen magnum)
    2. Meningitis
    3. Inflammatory disorder
    4. Tumor
    5. Tumors

Signs:

  • symptoms can present after months to years after incident
  • involves the cervical or thoracic spine
  • upper extremity loss of pain/temperature (cape-like)
  • upper extremity intact vibratory/proprioception/light touch
  • if syrinx is enlarged = lead to flaccid paralysis/ weakness + central pain + urgency + lower extremity

Diagnosis:
- MRI shows intra-medullary cavity filled with CSF

Management:
1. Surgical intervention (shunt placement)

180
Q

Differential flaccid paralysis

A

Infant botulism

  • ingestion of C.botulinum spores (from environment/ construction work/ honey)
  • sign: descending flaccid paralysis
  • treat: human-derived botulism immune globulin + respiratory support + nasogastric tube feeding
  • signs: bilateral bulbar palsies ( ptosis, sluggish pupillary response to light, poor suck & gag reflexes) + followed by descending flaccid paralysis (hypotonia) + constipation & drooling (autonomic dysfunction)

Food-brone botulism

  • ingestion of preformed C.botulinum toxin (canned food)
  • descending flaccid paralysis + N/V + Abdominal pain + diarrhea
  • treat: equine-derived botulism antitoxin

Guillain-Barre syndrome

  • autoimmune demyelination of PNS
  • ascending flaccid paralysis + to the rest of the body
  • treat: pooled human-immune globulin
181
Q

Internuclear opthalamoplegia

A
  • seen in MS
  • caused by conjugate horizontal gaze
  • the ipsilateral eye (to the damage of MLF) can’t be adducted & the contralateral eye to the MLF damage can be abducted with horizontal nystagmus
  • preserved convergence & reactivity to light

Note:
- unilateral MLF = seen with lacunar stroke in the pontine artery distribution

  • bilateral MLF = seen with multiple sclerosis
182
Q

Edinger westphal nucleus (EWN)

A
  • preganglionic parasympathetic

- damage to EWN= ipsilateral fixed & dilated pupil that is un-reactive to light or accommodation

183
Q

Myasthenia gravis disorder

A
  • ptosis, diplopia, dysphagia
184
Q

Lateral ganiculate nucleus (LGN)

A
  • Located in thalamus
  • rely information to ipsilateral primary visual cortex
  • damage= contralateral homonymous hemianopsia
185
Q

Medial lemniscus

A
  • damage to ML = contralateral loss of vibration, proprioception, and light touch
186
Q

Nerve palsy

A

Oclumotor (3) nerve palsy:
- mydriasis (dilated pupil), ptosis, down/out

Trochlear (4) nerve palsy:
- vertical diplopia + worsen when affected eye looks down/towards the nose (ex: walking downstairs, reading)

Abducens (6) nerve palsy:
- inability to abduct the eye

187
Q

Optic chiasm

A
  • damage of OC= bitemporal hemianopsia
  • occurs with anterior communicating artery aneurysm or tumor with the sella turcica ( craniopharyngioma or pituitary adenoma)
188
Q

Optic nerve

A
  • a lesion of ON = optic neuritis

- result in monocular vision loss

189
Q

Receptive aphasia (wernicke’s area)

A
  • well articulated, non-sensical speech & lack of language comprehension
  • word salad type of speech
  • seen with wernicke aphasia (located in the caudal superior temporal gyrus of the dominant temporal lobe)
  • caused by = infarcts at the inferior division of the MCA
190
Q

Expressive aphasia (broca’s area)

A
  • speech is halted & non-fluid
  • caused by= infarct at the superior division of MCA
  • located at the superior region of frontal lobe = broca’s area
  • usually seen with contralateral hemiparesis (due to involvement of primary motor cortex)
191
Q

Brainstem strokes

A
  • produce cranial nerve dysfunction
  • lead to dysarthria (motor speech disorder leading to difficulty producing sounds)
  • does not lead to aphasia (disorder of language processing)
192
Q

Hemorrhagic stroke in children

A

Etiology:

  1. AVM ( isolated or associated with hereditary hemorrhagic telangiectasia/HHT)
  2. Aneurysm
  3. Sickle cell disease or hemophilia

Signs:

  1. Headache + vomiting
  2. Seizure
  3. Focal neurologic deficits (contralateral hemiparesis)
  4. Altered mental status (somnolence)
  5. HTN —> due to increased ICP

Imaging:
1. Head CT: intraparenchymal, intraventricular or subarachnoid hyperdense fluid collection with irregular margin

Management:

  1. Supportive (anti-epileptics)
  2. Reduction of ICP (elevated head of bed, surgical decompression)
193
Q

Medulloblastoma

A
  • cerebellar mass presents with increased ICP

- signs: morning headache + vomiting + ataxia + poor coordination

194
Q

Fragile X syndrome

A

Pathology:

  • (CGG) repeats expansion of FMR1
  • gene methylation silences FMR gene
  • X-linked dominant

Feature:

  • developmental delay + intellectual disabilities
  • ADHD + autism
  • anxiety
  • self-harming behavior (hand biting)

Findings:

  • long faces with prominent forehead + chin
  • large, protruding ears
  • macroorchidism (age > 8) (large testes)
  • macrocephaly + hypotonia
  • joint hypermobility (finger, wrists)

Diagnosis:
- FMR1 DNA analysis (PCR or southern blot)

Management:
- normal life expectancy

195
Q

Marfan syndrome

A

Signs:

  • joint hyper-mobility
  • increase risk of aortic root disease
196
Q

RET syndrome

A
  • progressive brain deterioration
  • microcephaly
  • development regression
  • most common in girls (MECP2 mutation )
  • mutation is fetal in boys
197
Q

Concussion (after Traumatic brain injury)

A

Feature:

  • transient neurologic disturbance ( dizziness, disorientation, amnesia ) following a TBI
  • no structural intracranial injury
  • signs: headache + vomiting + dizziness + slurred speech + incoordination + LOC

Management:

  • rest for > 24 hours
  • gradual return to activity:
    1) aerobic exercise —> non-contact sport —> contact sport
    2) limited screen time + shortened school day + frequent breaks
198
Q

Spinal cord compression (myelopathy) ( signs: LE weakness + gait/bladder/bowel dysfunction + Lhermitte sign )

A

Signs:

  • unsteady gait
  • electric shock sensation in the spine
  • upper limb weakness + atrophy (LMN lesion)
  • lower extremity tone/reflexes increased ( UMN lesion)
  • bowel/bladder incontinence

Note:
- myelopathy —> elderly —> cervical spondylosis (spinal canal narrowing due to formation of osteophytes in the lateral vertebral body or ossification of the posterior longitudinal ligament/ ligamentum flavum)

  • history of fibromyalgia + shoulder/neck pain
199
Q

Distal symmetrical polyneuropathy

A

Triggers:

  1. Diabetes
  2. Long standing HIV infection
  3. Uremia
  4. Medication: flouroquinolones & metronidazole ( 2 months of use)
  5. Chemotherapy: cisplatin
  6. Toxicity: alcohol, heavy metal
  7. Others: digoxin, amiodarone, dapsone

Etiology:
- damage to distal sensory peripheral nerve axons

Features:

  • symptoms begins in toes/feet: progress proximally over time —> in stock-glove distribution (2-3 wks)
  • distal numbness/tingling/ pins & needle sensation
  • decrease in pain, temperature, vibration sensation
  • decrease ankle & babniski reflexes

Treatment:

  • pain management:
    1) gabapentinoid
    2) Tricyclic antidepressant
    3) duloxetine
    4) capsaicin cream
200
Q

Creutzfeldt-Jakob disease (CJD)

A

Pathology:
- fetal disease caused by abnormally folded protein (prion)

Features:

  1. Rapidly progressive dementia
  2. Myoclonus ( provoked by startle)
  3. Cerebellar signs (ex: ataxia)
  4. Upper motor neuron signs ( ex: hyperreflexia)
  5. Extra-pyramidal signs (ex: hypokinesia)
  6. Mood/sleep disturbance

Findings:

  • MRI shows:
    1. Widespread atrophy of cerebral or cerebellum
    2. Enhancement of putamen or caudate head (hockey stick sign)
    3. Enhancement of cortical ( cortical ribboning)
  • CSF: normal routine analysis (non-inflammatory), positive 14-3-3 protein titers, positive RT-QuIC test
  • EEG: Tri-phasic, synchronous discharges
  • Neuropath: spongiform degeneration without inflammation

Management:
1. Fetal in < 12 months

201
Q

Von- Hippel- Lindau disease

A

Pathology:

  • autosomal dominant
  • mutation in VHL gene in chromosome 3

Feature:

  1. Cerebellar/ CNS & retinal hemangioblastoma
    - decrease visual acuity due to edema or distortion of retina
    - bleeding from tumor leads to retinal detachment + glaucoma + vision loss
  2. Renal cell carcinoma ( clear cell type)
  3. Pheochromocytoma
  4. Light -brown facial patches (cafe au lait)

Management:

  1. Eye/retinal examination
  2. MRI of brain + spine + abdomen
  3. Plasma or urine metanephrines
  4. Tumor resection

Note:
- Bilateral retinal hemangioma + family history of adrenal tumor = VHL disease

202
Q

Signs of facet joint osteophytes

A

Signs:

  • one/side neck & shoulder pain
  • numbness + decrease pinprick sensation in forearm
  • worse with neck movement
  • refractory to pain killer
  • limited neck rotation + limited lateral bending
203
Q

Differential diagnosis of neck pain

A
  1. Strain
    - neck injury
    - pain/stiffness with neck movement
  2. Facet osteoarthritis
    - older patient
    - pain/stiffness worse with movement
    - relieved with rest
  3. Radiculopathy (cervical disc herniation or cervical spondylosis) in elderly
    - pain radiates to shoulder/arm
    - dermatomal senory/motor/reflex finding
    - positive spurling test
  4. Spondylitic myelopathy
    - lower extremity weakness + gait change + bowel/bladder dysfunction
    - Lhermitte sign
  5. Spondyloarthropathy
    - young men
    - HLA- B27
    - relieved with exercise
    - prolonged morning stiffness

Spinal metastasis:

  • constant pain
  • worse at night
  • not responsive to position changes

Vertebral osteomyelitis:

  • focal tenderness
  • fever & night sweat
  • intravenous drug use, recent infection, immune compromised
204
Q

Idiopathic

A
205
Q

Idiopathic intracranial HTN

A

Risk factors:
- obesity, pregnancy, medication ( retinoid/vitamin A, tetracycline, growth hormones)

Signs:
- headache, n/v, visual changes, neck pain, back pain

Findings:

  • elevated opening pressure (LP) (> 250 mm H2O)
  • papilledema/enlarged blind spot
  • MRI to rule out tumor
  • MRI venography to rule out venous thrombosis

Management:

  • weight loss
  • carbonic anhydrase inhibitor ( topiramate, acetazolamide)
  • shunting or optic nerve sheath fenestration
206
Q

malignant hemispheric infarction (MHI)

A
  • When ischemic stroke causes cerebral edema & hemorrhagic transformation
  • signs:
    1. Rapid neurologic deterioration (obtundation, eye deviation) 48 hours after ischemic stroke
    2. Cerebral edema: lead to brain herniation due to increased ICP
    3. Hemorrhagic transformation: blood go from injured blood vessels into brain parenchyma

Diagnosis:
- repeated non-contrast CT of head

Management:
- decompression hemicraniectomy

207
Q

Wernicke encephalitis

A

Associated conditions:

  1. Chronic alcoholism (most common)
  2. Malnutrition (Anorexia nervosa)
  3. Hyperemesis gravidarum

Pathology:
- thiamine deficiency (vitamin B12)

Feature:

  • encephalopathy (confusion)
  • oculomotor dysfunction/opthalamoplegia (horizontal nystagmus & bilateral abducens palsy)
  • postural/gait ataxia

Treatment:
- vitamin B1 (thiamine) followed by Iv glucose

208
Q

Idiopathic transverse myelitis

A

Pathology:

  • immune mediated destruction of spinal cord
  • often post-infection

Feature:

  1. Bilateral weakness: initially flaccid (LMN), followed by spastic (UMN)
  2. Bilateral sensory dysfunction
  3. Distinct sensory level
  4. Bowel/bladder dysfunction

Diagnosis:

  • MRI of spine: no compressive lesion, increased T2 signal (hyper-intensity) in the cervical spinal cord (spinal cord edema !!)
  • Lumbar puncture: increase WBC, increase IgG index (evident of inflammation)

Treatment:

  • high-dose IV glucocorticoids
  • plasmapheresis
209
Q

Cerebral amyloid angiopathy

A
  • second leading cause of intracerebral hemorrhage, after HTN in adults
  • lobar in location
  • signs:
    1. Patient > 75
    2. Abnormal beta-pleated amyloid protein deposition In cerebral blood vessel —> lead to arteriopathy & vessel fragility
Findings:
- MRI shows
1. Initial: multiple small foci of prior hemorrhage 
(microbleed)
- non-contrast CT shows:
1. Lobar Hemorrhage (hyper-dense)
210
Q

C6 radiculopathy due to C5/C6 subluxation

A
  • weakness in wrist extension + numbness in forearm & thumb
211
Q

C7 radiculopathy due to C6/C7 subluxation

A
  • weakness in triceps extension & wrist flexion + numbness in index & middle finger
212
Q

Brain tumor symptoms based on location (CNS tumors)

A

Cerebral cortex: (astrocytoma,

  1. Headache + seizure + focal neurologic deficits ( hemiparesis /speech problem)
  2. Increase ICP signs (morning headache, vomiting, papilledema)

Brainstem: (brainstem glioma, craniopharangioma, optic glioma, pinealoma)

  1. Cranial nerve deficits
  2. Ataxia + poor coordination

Posterior fossa: (cerebellar astrocytoma + medulloblastoma, infratentorial ependyndoma)

  1. Increase ICP signs (morning headache, vomiting, papilledema)
  2. Ataxia + poor coordination
213
Q

Giant cell arteritis (aka: temporal arteritis, horton disease)

A

Symptoms:

  1. Systemic: fever, weight loss, fatigue, malaise
  2. Headache
  3. Jaw claudication
  4. Polymylagia rheumatica (muscle pain/stiffness of shoulder + hip)
  5. Visual disturbance: amaurosis fugax + anterior ischemic optic neuropathy (vision loss)
  6. Age > 50

Diagnosis:

  1. Normochromic anemia
  2. Elevated ESR & CRP
  3. Temporal artery biopsy

Treatment:
1. Polymyalgia rheumatica only: low-dose oral glucocorticoids ( prednisone 10-20 mg daily)

  1. Giant-cell arteritis: intermediate-high dose of oral glucocorticoid (prednisone 40-60 mg daily)
  2. Giant cell arteritis with vision loss: Pulse high-dose IV glucocorticoid (methylprednisolone, 1000mg DAILY) for 3 days, followed with intermediate-high dose of oral glucocorticoids.

Note:
** to prevent blindness = treatment with systemic glucocorticoid should not be delayed while awaiting for temporal artery biopsy

214
Q

Evaluation of new onset of headache

A
  • if patient have type of a headache (migraine, or cluster headache …)
  • have a new onset of headache that is different in characteristic + increase ICP signs (morning headache + vomit + papilledema)
  • immediately evaluate with MRI of brain
215
Q

Oclumotor nerve palsy

A

Diplopia + ptosis (motor fibers/ paralysis of levator palpebrea superioris)+ mydriasis (parasympathetic fibers/sphincter papillae muscle) + impaired adduction/impaired lateral gaze (opthalamoplegia)

Caused by:

  1. Aneurysm, tumor ( mass effect) = non-pupil sparing CN 3
  2. Microvascular ischemia (diabetes, HTN, hyperlipidemia, advanced age) = pupil-sparing CN3

Diagnosis:
1. MRI or CT angio

216
Q

Lacunar stroke

A

Etiology:

  1. Small penetrating artery occlusion due to HTN arteriolar sclerosis
  2. Combination of microatheroma formation + lipohyalinosis that ultimately leads to thrombotic small-vessel occlusion

Affected areas:

  1. Basal ganglia
  2. Subcortical white matter ( internal capsule, corona radiata)
  3. Pons

Risk factors:
1. HTN

Signs:

  1. Absence of:
    - cortical signs ( aphasia, agnosia, neglect, apraxia, hemianopia)
    - seizure
    - mental status changes
    - CN deficits
  2. Common syndromes:
    - pure motor hemiparesis (common)
    - pure sensory stroke
    - ataxic hemiparesis
    - dysarthria- clumsy hand syndrome

Diagnosis:
- usually not seen on non-contrast CT scan after incident

217
Q

Carotid artery dissection (intimal tear)

A
  • trauma
  • Lead to ischemic stroke due to thromboembolism or hypo-perfusion

Signs:

  1. Neck pain
  2. Partial Hornor syndrome ( ptosis + miosis)
218
Q

Cerebral venous sinus thrombosis

A
  • occurs in patients with hyper-coagulable state ( pregnancy, malignancy)

Signs:

  • headache
  • seizure
  • altered mental status
  • increased ICP
  • Focal/diffused neurogenic defects
  • CT shows: cerebral edema, venous infarction, areas of hemorrhage
219
Q

Carotid artery occlusion

A

-large -artery occlusion causes more severe symptoms than small-artery occlusion (lacunar stroke)

Signs:

  • contralateral homonymous hemianopsia
  • hemiparesis
  • hemisensory loss
  • seizure
  • altered mental status
  • cortical signs
220
Q

Spinal epidural abscess

A

Epidemiology:

  • staph aureus
  • HIV, Diabetes, alcohol, elderly
  • distant infection, spinal procedure, injection drugs

Signs:

  • triads: fever + back pain + neurologic sign ( sensory/motor deficits, bowl/bladder dysfunction, paralysis)
  • increase ESR

Diagnosis:

  • MRI of spine with contrast
  • blood & aspirate culture

Treatment:

  1. broad-spectrum antibiotics (vancomycin + ceftriaxone)
  2. Urgent aspiration/ surgical decompression
221
Q

Encephalitis vs meningitis

A

Encephalitis:

  • inflammation of the brain
  • signs:
    1. Acute onset of focal neurologic findings ( altered mental status, focal CN deficits, ataxia, hyperreflexia, focal seizure)
    2. Fever
    3. Confusion + strange Behavioral (hypomenia, hyperphagia, hypersexuality, amnesia)
    4. CSF:
  • lymphocytic pleocytosis ( increase erythrocyte, increase protein)
    5. Temporal lobe lesion seen with HSV ENCEPHALITIS

Meningitis:
-inflammation of the meninges that surrounds the brain & spinal cord

222
Q

Migraine

A

Note: estrogen- containing OCP is contraindicated in patients with migraine with aura due to increase risk of ischemic stroke

Signs:

  • unilateral throbbing headache
  • associated with photophobia, phonophobia, N/V
  • lasts 4-72 hours
  • symptoms of aura:
    1. Visual ( wavy lines, visual loss)
    2. Sensory ( paresthesia, numbness)
    3. Auditory ( sounds, hearing loss)
    4. Motor dysfunction ( tremor, weakness)

Increase risk factor:

  • migraines are associated with ischemic stroke
  • the risk increases with the use of Estrogen-containing OCP—> should be discontinued
223
Q

Abnormal gait pattern

A

Parkinsonian:

  1. Parkinson disease, dementia with lewy body, vascular dementia, multiple system atrophy
  2. Narrow-based + shuffling gait ( reduced stride length

Weakness with spasticity

  1. Myelopathy
  2. UMN lesion (spinal cord injury, cerebral palsy)
  3. Narrow-based + scissoring + toes scrape the floor

Myopathic

  1. Myopathies , myasthenia gravis
  2. Waddling, abnormal pelvic tilt (limb gridle weakness)

Magnetic

  1. Normal pressure hydrocephalus
  2. Difficulty initiating steps ( move slowly + wide base + several step to turn around)

Vestibular:

  1. Benign positional paroxysmal vertigo, meniere disease
  2. Stumbling or veering to one side ( usually the affected side)

Cerebellar ataxia:

  1. Stroke, drug toxicity, alcohol, hereditary
  2. Wide-based + stumbling, lurching + maybe one side of bilateral depending on etiology

Sensory ataxia:

  1. Sensory neuropathy, B12 deficiency
  2. Wide-based + high-stepping/ stamping + worse in the dark
224
Q

Bilirubin-induced neurologic dysfunction in infancy

A

Etiology:

  • unconjugated bilirubin crosses BBB
  • deposits in basal ganglia & brainstem nuclei (neuronal damage, atrophy, necrosis)

Signs;

  1. Hyperkinetic movement (chorea, dystonia)
  2. Hearing loss (sensorineural)
  3. Gaze abnormality
225
Q

Multiple system atrophy

A

Signs:

  1. Early postural instability (falls)
  2. Early autonomic dysfunction ( orthostatic, urinary urgency)
  3. Ataxic gait
226
Q

Chronic lithium toxicity

A
  • caused by decrease lithium clearance due to decrease renal perfusion (dehydration)

Signs:

  1. Neurologic symptoms (lethargy/confusion/ataxia/tremor)
  2. Cardiac abnormality ( QT prolongation, bradycardia)
227
Q

Antipsychotic Extrapyramidal effect & pharmacology

A
  • associated with 1st generation antipsychotics (haloperidol & fluphenazine)

Acute dystonia:

  • sustained contraction of the neck/tongue/mouth/eye muscles
  • treatment: benztropine, diphenhydramine

Akathesia:

  • subjective restlessness (inability to sit still)
  • treatment: beta-blocker (propranolol), benzodiazepine (lorazepam), benztropine

Parkinsonism:

  • gradual onset tremor + bradykinesia + rigidity
  • treatment: benztropine, amantadine

Tarda dykinesia:

  • gradual onset after prolonged therapy (> 6 months)—> dykinesia of mouth/face/trunk/extremity
  • treatment: valbenazine, deutetrabenazine
228
Q

Carpal tunnel syndrome common in patients on hemodialysis due to ESRD

A
  • compression of median nerve leads to carpal tunnel syndrome
  • CTS common in patients on hemodialysis
  • signs:
    1. Pain/tingling/numbness in the lateral hand
229
Q

Parkinson disease anticholinergic (trihexyphenidyl) can cause acute close-angle glaucoma

A
  • patient with PD
  • take anticholenergic drug (trihexyphenidyl) for resting tremor
  • predispose to acute angle-closure glaucoma

Signs:

  • acute headache, nausea, blurry vision, dilated pupil, red eye, see halos, eye pain
  • can develop vision loss within 2-5 hours

Note:
Other drugs associated with ACG: antiemetics, decongestant, anticholinergics

230
Q

Mytonic dystrophy

A

Etiology:

  • CTG repeat expansion in DMPK gene
  • autosomal dominant

Signs:

231
Q

Cerebellar stroke

A
  • sudden onset, persistence vertigo + neurologic signs (headache + dysmetria + ataxia)
  • thrombolysis + IV alteplase = (administer to patient with ischemic stroke who do not have aphasia, weakness)
232
Q

Post concussive syndrome

A

Different signs occurs hours to days after TRAUMATIC BRAIN INJURY:

  • headache, dizziness, fatigue, confusion, amnesia, difficulty concentrating, or multitasking, sleep disturbance, anxiety , mood change, vertigo
  • resolve by it is own
233
Q

Neoroblastoma

A

Signs:

  • lump in abdomen and chest
  • bruised eye
  • back pain
  • horner syndrome (ptosis, miosis)
  • cervical paravertebral sympathetic chain
234
Q

Vestibular schwannoma (acoustic neuroma)

A
  • benign tumor of CN 8

Signs:

  • sensorineuronal hearing loss + imbalance (CN 8 dys)
  • facial numbness/ paralysis (CN 5/ 7 dys)
  • unilateral deficit (bilateral seen with NF type 2)
235
Q

Sickle cell disease in pediatric

A
  • lead to stroke
  • treat with transfusion exchange
  • diagnose with Hemoglobin electrophoresis
236
Q

000plk

A
237
Q

Giant cell arteritis vs temporomandibular joint disorder

A
  • GCA typically in patient > 50 (headache, jaw pain when chewing)
  • TMJD: headache, muscle spasm, jaw fatigue, facial pain (+ tenderness of muscle of mastication, pain/crepitus/audible clicks from the joint)

Nuerology Shelf Exam UWORLD (1 decks)

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