Neurology Flashcards

Question

Mnenomic for GCS

Answer 1

My Extra Value Meal costs $4.56

Answer 2

It is an autoimmune disease, where the body creates antibodies against the NMDA receptors in the brain. These antibodies disrupt normal brain signaling and cause brain swelling, or encephalitis.

Answer 3

Recurrent or continuous subclinical seizures resulting in a fluctuating or diminished level of consciousness. Electrographic seizures seen on EEG with little or no obvious motor manifestations of seizure. Think of this when a person has ongoing delirium with no identifiable cause. EEG when suspicion.

Answer 4

o Initially, pulse oximetry, fingerstick plasma glucose measurements, and cardiac monitoring are done. o Blood tests should include a comprehensive metabolic panel (including at least serum electrolytes, blood urea nitrogen [BUN], creatinine, and calcium levels), complete blood count (CBC) with differential and platelets, liver tests, and ammonia level. o Arterial blood gases (ABGs) are measured, and if carbon monoxide toxicity is suspected, carboxyhemoglobin level is measured. o Blood and urine should be obtained for culture and routine toxicology screening; serum ethanol level is also measured. o ECG + CXR + EEG (seizures) o If the cause is not immediately apparent, noncontrast head CT should be done o If coma is unexplained after MRI or CT and other tests, lumbar puncture (spinal tap) is done to check opening pressure and to exclude infection, subarachnoid hemorrhage, and other abnormalities

Answer 5

▪ Cerebral Damage: fast phase absent, deviation to same side if cold water ▪ Brainstem Damage: fast + slow phase absent

Answer 6

Treat with CSF drain (30-40 cc’s) and place a VP shunt

Answer 7

Coma refers to a state of unresponsiveness without arousal caused by interruption of neuronal networks that regulate consciousness.

Answer 8

AST, ALT, ALP, bilirubin, INR, PTT, NH4

Answer 9

General: neck flexion, breath odor, look for head trauma Neuro: LOC, GCS, oculocephalic reflex, CN (pupils, corneal reflex, gag reflex), reflexes (Babinski’s and Hoffman’s), tone

Answer 10

Non-Convulsive Status Epilepticus Aphasia Lewy-Body Dementia Encephalitis

Answer 11

- environment: quiet, well-lit, near window for cues regarding time of day - optimize hearing and vision - room near nursing station for closer observation; constant care if patient jumping out of bed, pulling out lines - family member present for reassurance and re-orientation - frequent orientation: calendar, clock, reminders - avoid frequent changes of assigned nursing staff

Answer 12

GAMETIMES: o G - Hyperosmolar + Hypo/hyperglycemia o A - Anoxia + Acidosis + Alcohol + Aneurysm o M - Meds + Drugs (alcohol, anesthetics, opioid, sedatives, SSRI, cocaine) o E – Electrolytes (hypercalcemia, hyper/hyponatremia) + Environment (Hypo/hyperthermia, hypoxia) o T - Tumor + Toxin (CO) + Trauma (Subarachnoid Hemorrhage, Subdural Hematoma) o I – Infections (encephalitis, malaria, meningitis) o M - Metabolic + Adrenal + Renal + Hepatic o E – Epilepsy (focal) o S - Stroke + Psychiatric

Answer 13

1. Conductive Hearing Loss - Conduction of sound to the cochlea is impaired. 2. Sensorineural Hearing Loss - Defect in the conversion of sound into neural signals or in the transmission of those signals to the cortex 3. Mixed Hearing Loss - Combination of CHL and SNHL

Answer 14

Cerumen accumulation, Noise, Aging (Presbycusis), Infections (particularly among children and young adults)

Answer 15

Conductive: Genetic, Developmental (eg, ossicular fixation), Idiopathic (unknown) malformation, Drug-induced malformation (eg, with thalidomide) Sensory: Genetic, Idiopathic (unknown) malformation, Congenital infection (eg, rubella, cytomegalovirus infection, toxoplasmosis, syphilis), Rh incompatibility, Anoxia, Maternal ingestion of ototoxic drugs (eg, for tuberculosis or severe infection), Drug-induced malformation (eg, with thalidomide) Neural: Anoxia, Idiopathic (unknown) malformation, Genetic, Congenital infection (eg, rubella, cytomegalovirus infection, toxoplasmosis, syphilis), Neurofibromatosis (type 2), Hyperbilirubinemia

Answer 16

External ear (conductive loss): Obstruction (eg, caused by cerumen, a foreign body, otitis externa, or, rarely, tumor) Middle ear (conductive loss): otitis media, ear trauma, otosclerosis, tumors Inner (sensory loss): Genetic disorders (eg, connexin 26 mutation – most common), noise exposure, presbycusis, ototoxic drugs (eg, aspirin, aminoglycosides), infections (eg, meningitis, purulent labyrinthitis), Meniere syndrome, barotrauma CNS (neural loss): Tumors of the cerebellopontine angle (eg, acoustic neuroma, meningioma - tinnitus), Demyelinating disease (eg, multiple sclerosis)

Answer 17

Abnormal bone growth inside the ear, FHx, age of onset 20-30s

Answer 18

White forelock of hair or different colored eyes

Answer 19

Associated with thyroid gland disorders, SLC26A4 gene, enlarged vestibular aqueducts

Answer 20

Low birthweight/prematurity, perinatal anoxia (low APGARs), kernicterus:bilirubin \>25mg/dL, craniofacial abnormality, family history of deafness in childhood, 1st trimester illness: TORCH infections, neonatal sepsis, ototoxic drugs, perinatal infection, including post-natal meningitis, consanguinity

Answer 21

All infants

Answer 22

Should note how long hearing loss has been perceived, how it began (eg, gradual, acute), whether it is unilateral or bilateral, and whether sound is distorted (eg, music is off—dull or lifeless) or there is difficulty with speech discrimination. Any acute event. other otologic symptoms (eg, ear pain, tinnitus, ear discharge), vestibular symptoms (eg, disorientation in the dark, vertigo), and other neurologic symptoms (eg, headache, weakness or asymmetry of the face, an abnormal sense of taste, fullness of the ear).

Answer 23

External ear + TM inspection, Neuro exam (particular attention needs to be paid to the 2nd through 7th cranial nerves). Weber + Rhine testing

Answer 24

The 5th or 7th cranial nerve or both are often affected by tumors that involve the 8th nerve, so loss of facial sensation and weak jaw clench (5th) and hemifacial weakness and taste abnormalities (7th) point to a lesion in that area

Answer 25

512 Hz tuning fork is held on vertex of head and patient states whether it is heard centrally (Weber negative) or is lateralized to one side (Weber right, Weber left)

Answer 26

Weber Test lateralization = ipsilateral conductive hearing loss or contralateral sensorineural loss

Answer 27

Right-sided SNHL, normal left ear

Answer 28

Normal or bilateral SNHL

Answer 29

512 Hz tuning fork is struck and held firmly on mastoid process to test bone conduction (BC); the tuning fork is then placed beside the pinna to test air conduction (AC), If AC \> BC = positive Rinne (normal)

Answer 30

- BC in normal range - AC outside of normal range - Gap between AC and BC thresholds \>10dB (an air-bone gap)

Answer 31

- Both air and bone conduction thresholds below normal - Gap between AC and BC \<10 dB (no air-bone gap)

Answer 32

- Both air and bone conduction thresholds below normal - Gap between AC and BC threshold s\>10dB (an air-bone gap)

Answer 33

SNHL associated with aging (starting in 5th and 6th decades)

Answer 34

- Progressive, bilateral hearing loss initially at high frequencies, then middle frequencies - Loss of discrimination of speech, especially with background noise present – patients describe people as mumbling - Recruitment phenomenon: inability to tolerate loud sounds - Tinnitus

Answer 35

- Stop ototoxic meds - Fluid from middle ear effusion can be drained by myringotomy and prevented from reaccumulating with the insertion of a tympanostomy tube. - Damage to the tympanic membrane or ossicles or otosclerosis may require reconstructive surgery. Brain tumors causing hearing loss may in some cases be removed or radiated and hearing preserved. - Many causes of hearing loss have no cure, and treatment involves compensating for the hearing loss with hearing aids and, for severe to profound loss, a cochlear implant

Answer 36

Prevention of hearing loss consists mainly of limiting duration and intensity of noise exposure. People required to expose themselves to loud noise must wear ear protectors (eg, plastic plugs in the ear canals or glycerin-filled muffs over the ears).

Answer 37

No identifiable cause

Answer 38

Underlying etiology present

Answer 39

* Onset (episodic vs chronic) * Associated sx (visual changes, change in mental status, nausea/vomiting, fever, meningismus, photophobia, phonophobia) * Precipitating/alleviating factors (triggering factors, analgesics), medications (especially nitrates, calcium channel blockers, NSAIDs, anticoagulants), PMHx, FHx * Lifestyle factors (caffeine, exercise – regular exercise is the best prophylaxis, sleep)

Answer 40

* Vitals: is there fever? Is there severe HTN (sBP \>180 or dBP \>120)? * Fundoscopy * Focal Neuro Exam * Is there papilledema? Consider carotid artery dissection

Answer 41

SNOOPED * Systemic Signs/Symptoms: papilledema, neck stiffness, immunocompromised, personality change * Neurologic symptoms (focal neuro deficits) * Old age (\>50) * Onset is sudden or worst ever or thunderclap * Pattern is progressive or new * Evoked by valsalva, exercise, certain positions (lying down), or post-trauma * Daily

Answer 42

If red flags or risk of herniation (i.e. intracranial pathology)

Answer 43

If immunocompromised, if \>60 with suspect temporal arteritis

Answer 44

* If suspect infection (WBCs), bleeding (RBCs), or malignancy (abnormal cells)

Answer 45

POUND: Pulsating/throbbing, 4-72 hOurs, Unilateral, Nausea/vomit, Disabling

Answer 46

2/3: nausea/vomiting, photophobia, or worse w/ exercise

Answer 47

Cyclical vomiting, episodic stomach pain.

Answer 48

1. Aura 2. Without aura

Answer 49

5-60 mins (if longer than 60 mins – get CT scan) - visual disturbance (fortification spectra - zigzags; scintillating scotomata - spots), unilateral paresthesia and numbness, dysphasic speech – NOT MOTOR WEAKNESS

Answer 50

Cortical spreading depression (CSD) is a propagating wave of depolarization of neurons and glial cells that spreads across cerebral cortex. The spreading of this wave is hypothesized to activate the trigeminal nerve afferents, causing inflammatory changes in pain-sensitive structures that create migraine headache

Answer 51

Lifestyle (caffeine – drink same amount everyday, sleep – change in hours of sleep triggers, diet, triggers, exercise), headache diary + stress management

Answer 52

Hierarchy: Ibuprofen, naproxen, Tylenol → triptans → naproxen sodium + triptan, narcotic analgesic are not recommended.

Answer 53

Use at headache onset and hit it hard, not daily otherwise you can make their migraine transform to daily.

Answer 54

Indicated if headache \>3 days/month and acute meds not effective OR \>8 days/month OR Disability despite acute meds

Answer 55

1st line: propanolol, metoprolol (avoid in asthma), amitryptiline (consider if depression, anxiety, insomnia)

Answer 56

2nd line: topiramate (1st line if overweight), candesartan (avoid in pregnancy)

Answer 57

Other: divalproex sodium (avoid in pregnancy), pizotifen (monitor for somnolence and weight gain), botox (chronic headache only)

Answer 58

Squeezing sensation (tight-band), bilateral, fronto-occipital or generalized, no N/V, no photophobia, no migrainous Sx, not worse with activity

Answer 59

\>15 days/month for \>6 months

Answer 60

Rest and relaxation Abortive Tx with OTC meds (ibuprofen, ASA, acetaminophen, limit 2-3x/ week) Prophylactic Tx if severe (1st line – amitriptyline, 2nd – venlafaxine (Effexor))

Answer 61

Severe, unilateral headaches (retro-orbital) associated with autonomic Sx (tearing, nasal congestion, rhinorrhea, conjunctivitis, horner’s syndrome, agitation), once begin often recur daily for several weeks (cluster) @ same time everyday (classically awakens @ night), \<3h

Answer 62

Abortive: triptans, oxygen 100% Preventative: verapamil 120-900mg – get ECG, lithium carbonate, valproate, topiramate, melatonin

Answer 63

If more than two attacks per day, consider transitional therapy while verapamil is built up (e.g., prednisone 60 mg for five days, then reduced by 10 mg every two days until discontinued, or occipital nerve blockage with steroids)

Answer 64

Consistent headache worse in AM, whooshing in ears, papilledema + focal neuro deficits

Answer 65

Typically in young overweight women

Answer 66

CT venogram (rule out venous sinus thrombosis)

Answer 67

Steroids/repeat LP until optic nerve fenestration completed. Diamox – acetazolamide (reduces CSF produced), topiramate (helps with weight loss), weight loss, urgent referral to ophtho and neuro.

Answer 68

Acute onset, reaches max intensity within minutes, worse with exertion

Answer 69

Causes: SAH and arterial dissection

Answer 70

Fever/chills, nuchal rigidity, photophobia, N/V, confusion/altered LOC, rash, immunocompromised

Answer 71

50yo localized headache (temporal), jaw claud, scalp tenderness tender temporal artery, PMR association, visual symptoms.

Answer 72

Temporal artery biopsy and CRP.

Answer 73

Immediate high-dose moderate corticosteroids (prednisone)! Can lead to permanent blindness.

Answer 74

* Ergots, triptans, combination analgesics or codeine/other opioids ≥ 10 days a month OR * Acetaminophen or NSAIDs ≥ 15 days a month

Answer 75

* Suspected CNS infection (with the exception of brain abscess or a parameningeal process). * Suspected SAH in a patient with a negative CT scan

Answer 76

* Possible raised intracranial pressure (ICP) with risk for cerebral herniation * Thrombocytopenia or other bleeding diathesis, including ongoing anticoagulant therapy * Suspected spinal epidural abscess

Answer 77

Sudden onset of neurological deficits of a vascular etiology with infarction of CNS tissue

Answer 78

* Sudden onset of focal neurological deficits lasting \< 24hrof a vascular etiology without infarction (i.e. no imaging evidence of stroke). May present with amaurosis fugax (transient monocular painless vision loss) * Tissue based definition: Rapidly resolving neurologic symptoms, typically lasting \<1 hour, with no evidence of infarction on MRI (DWI – Diffusion Weighted Imaging)

Answer 79

* Ischemic (85%) * Hemorrhagic (15%)

Answer 80

* Arterial thrombosis: thrombus formation in artery (local/in situ) * Large vessel: stenosis or occlusion of the internal carotid artery, vertebral, or intracranial arteries * Small vessel/lacunar * Cardioembolic: blockage of cerebral arterial blood flow due to particles originating from a cardiac source * Atrial fibrillation (most common), rheumatic valve disease, prosthetic heart valves, recent MI, fibrous and infectious endocarditis * Systemic hypoperfusion (global cerebral ischemia): Inadequate blood flow to brain, usually secondary to cardiac pump failure (e.g. cardiac arrest, arrhythmia, or MI)

Answer 81

* Intracerebral (HTN, amyloid angiopathy, other) * Hypertensive (most common): rupture of small microaneurysms (Charcot-Bouchard aneurysms) causing intraparenchymal hemorrhage * Other: trauma, amyloid angiopathy (associated with lobar hemorrhage), vascular malformations, vasculitis, drug use (cocaine or amphetamines) * Subarachnoid

Answer 82

Contralateral leg paresis, sensory loss, cognitive deficits (e.g. apathy, confusion, and poor judgment). Right hemi – neglect. Left hemi – aphasia. Sensory loss + incontinence

Answer 83

Primarily affects watershed areas (between the major cerebral arterial territories)

Answer 84

Chronic HTN and DM cause vessel wall thickening and decreased luminal diameter

Answer 85

1. contralateral weakness and sensory loss of face and arm 2. cortical sensory loss 3. may have contralateral homonymous hemianopia or quadrantanopia 4. if dominant (usually left) hemisphere: aphasia 5. if non-dominant (usually right) hemisphere: neglect 6. eye deviation towards the side of the lesion (away from the weak side)

Answer 86

1. contralateral hemianopia or quadrantanopia 2. midbrain findings: CN III and IV palsy/pupillary changes, hemiparesis 3. thalamic findings: sensory loss, amnesia, decreased LOC 4. if bilateral: cortical blindness or prosopagnosia 5. hemiballismus

Answer 87

Proximal (usually thrombosis): impaired EOM, vertical nystagmus, reactive miosis, hemi- or quadriplegia, dysarthria, locked-in syndrome, coma

Answer 88

Distal (usually embolic, i.e. top of the basilar syndrome): somnolence, memory and behaviour abnormalities, oculomotor deficit

Answer 89

Ipsilateral ataxia, ipsilateral Horner's, ipsilateral facial sensory loss, contralateral limb impairment of pain and temperature sensation, nystagmus, vertigo, nausea/vomiting, dysphagia, dysarthria, hiccups

Answer 90

Contralateral hemiparesis (facial sparing), contralateral impaired proprioception and vibration sensation, ipsilateral tongue weakness

Answer 91

Contralateral weakness without cortical deficits since the stroke is now subcortical. Face/arm/leg equally involved.

Answer 92

Lipohyalinosis

Answer 93

* ABCs, full vital sign monitoring, capillary glucose, urgent CODE STROKE if \<4.5h from symptom onset (for possible thrombolysis) * LOC (knows age, month; obeys commands), dysarthria, dysnomia (cannot name objects) * Gaze preference, visual fields, facial palsy * Arm drift, leg weakness, ataxia * Sensation to pinprick, extinction/neglect

Answer 94

Seizure/post-ictal, hypoglycemia, migraine, conversion disorder

Answer 95

* Non-contrast CT head (STAT): to rule out hemorrhage and assess extent of infarct * ECG: to rule out atrial fibrillation (cardioembolic cause), Holter monitor * CTA, carotid dopplers, ECG, transthoracic echo * CBC, electrolytes, creatinine, partial thromboplastin time/international normalized ratio (aim for \<1.7), blood glucose (\>2.7 rules out hypoglycemia), lipid profile, platelets (\>100 for treatment)

Answer 96

Early ischemic changes: loss of cortical grey-white matter differentiation, hyperdense vessels, sulcal effacement (i.e. mass effect decreases visualization of sulci). Later ischemia changes: hypodense of parenchyma

Answer 97

Hyperdense. Diffusion weighted MRI is WAY BETTER than CT. The white areas on MRI showing the stroke are demonstrations of acute ischemia, which causes loss of oxygen and therefore ATPase shuts down, restricted diffusion of water out of ischemic tissue (cytotoxic edema) – this shows up as bright on the scan and can last for 4wks!

Answer 98

1. Thrombolysis 2. Anti-Platelet Therapy 3. Acute Anti-Coagulant Therapy 4. Intra-Arterial Mechanical Thrombectomy

Answer 99

* Hx: improving Sx, minor Sx, seizure at stroke onset, recent major surgery or trauma (within 14 d), recent GI or urinary hemorrhage (within 21 d), recent LP or arterial puncture at noncompressible site, PMHx of ICH, Sx of SAH/ pericarditis/MI, pregnancy * P/E: sBP \>185, dBP \>110, aggressive treatment to decrease BP, uncontrolled serum glucose \<2.7, thrombocytopenia * Labs: hemorrhage or mass on CT, high INR or aPT \>1.7

Answer 100

* Loading dose of antiplatelets at presentation of TIA or stroke if rtPA not received * Loading dose of ASA: recommended dose 160 mg chewed * If patient intolerant to ASA, use another antiplatelet agent (e.g. clopidogrel 300 mg)

Answer 101

* recommend IV heparin (or ensuring international normalized ratio between 2-3 if already anticoagulated on warfarin) * may delay initiation of oral anticoagulation depending on size of infarct and presence of petechial/frank hemorrhage

Answer 102

CT angio needed to document LVO and: \<6 hrs – patients must have small to moderate core on non-contrast. \>6 hrs with CTP selection. Up to 24hrs!!

Answer 103

* Avoid hyperglycemia which can increase the infarct size * Lower temperature if febrile (febrile stroke: think septic emboli from endocarditis) * Prevent complications * NPO if dysphagia (to be reassessed by SLP) * DVT prophylaxis if bed-bound * initiate rehabilitation early

Answer 104

* Do NOT lower the blood pressure unless the HTN Is severe * Antihypertensive therapy is withheld for 48-72h (permissive hypertension) after thromboembolic stroke unless sBP \>220 mmHg or dBP \>120 mmHg, or in the setting of acute MI, renal failure, aortic dissection (IV labetalol first-line if needed) * Acutely elevated BP is necessary to maintain brain perfusion to the ischemic penumbra * Most patients with an acute cerebral infarct are initially hypertensive but their BP will improve within 1-2 d

Answer 105

\*1. Hemibody Weakness – TIA until proven otherwise \*2. Speech Disturbance: for a defined period of time (definite dysarthria, muteness or marked word finding difficulty, paraphasic speech) 3. Monocular or Hemifield Visual Loss: not blurring of entire visual field 4. Double Vision, Crossed Numbness or Weakness, Slurred Speech, Ataxia of Gait. Localizes to the brainstem. 5. Hemibody Numbness 6. Vertigo: only if present with brainstem sx

Answer 106

Positional and recurrent numbness of one limb or tingling of all 4 extremities. Scintillating or flashing visual disturbances. Symptoms of durations \< 30 seconds. Seizure of convulsions at onset. Isolated syncope. Postural dizziness alone.

Answer 107

Well-defined onset time, definite focal neurological symptoms, presence of neurological signs on examination, being able to lateralize signs to the left or right side of the brain, being able to determine a clinical stroke subclassification

Answer 108

Age (\>60), BP (\>140/90), \*Clinical Features (unilateral weakness w/ or w/o speech disturbance get 2 points or speech deficit w/o weakness), Duration (\>10min \< 59 min get 1 point, \>60 min get 2 points), Diabetes

Answer 109

TIA symptoms in the last 48 hours with high risk sx (speech disturbance or weakness of the face, arm or leg) or lower risk symptoms (hemianopsia, monocular visual loss, brainstem syndrome)

Answer 110

TIA symptoms in the last 48 hours to 2 weeks with high risk sx (speech disturbance or weakness of the face, arm or leg)

Answer 111

TIA symptoms in the last 48 hours to 2 weeks with lower risk symptoms (hemianopsia, monocular visual loss, brainstem syndrome)

Answer 112

TIA symptoms more than 2 weeks ago regardless of symptoms

Answer 113

Emergent assessment (brain and vascular imaging – CTA neck vessels or carotid doppler) should be done within hours. Immediate EKG and antiplatelet therapy.

Answer 114

24 hours to get a clinical assessment done and then vascular imaging and brain imaging. EKG and immediate antiplatelet therapy.

Answer 115

Investigation by a stroke expert within two weeks of first contact. Brain imaging and vascular imaging within 2 weeks. EKG and immediate antiplatelet therapy.

Answer 116

Referral to clinic with neurological expertise to be seen within 1 month

Answer 117

ASA + clopidogrel given for 30 days post high risk TIA reduces recurrent stroke risk. Usually, beyond 30 days we stop clopidogrel and keep them on ASA long term

Answer 118

* Initial choice: ASA * If cerebrovascular symptoms while on ASA or if unable to tolerate ASA: Aggrenox, clopidogrel

Answer 119

* Carotid endarterectomy clearly benefits those with symptomatic severe stenosis (70-99%), and is less beneficial for those with symptomatic moderate stenosis (50-69%) * See significant reduction in stroke prevention if done w/n 6 months of symptoms.

Answer 120

* Primary and secondary prevention with anticoagulation * All patients with atrial fibrillation without contraindication be anticoagulated * Anticoagulation therapy * warfarin (titrate to international normalized ratio 2-3) * dabigatran (110 or 150 mg PO bid), apixaban (2.5 or 5 mg PO bid) or rivaroxaban (15 or 20 mg PO daily) may be alternatives to warfarin, but should be used cautiously

Answer 121

* Primary prevention * Targets: BP \<140/90 (sBP \<120 for high risk without diabetes or \<130/80 for diabetics or renal disease) * ACEI: ramipril 10mg PO OD is effective in patients at high risk for cardiovascular disease * Secondary prevention * ACEI and thiazide diuretics are recommended in patients with previous stroke/TIA

Answer 122

* Primary prevention: statins in patients with CAD or at high risk for cardiovascular events, even with normal cholesterol * Secondary prevention: high dose atorvastatin but lower doses may be adequate if patient cannot tolerate high dose

Answer 123

* Primary prevention: smoking increases risk of stroke in a dose-dependent manner * Secondary prevention: after smoking cessation, the risk of stroke decreases to baseline within 2-5yr

Answer 124

1. Cerebellar ataxia * Tumors * Vascular * Hereditary * Multiple sclerosis * Drugs * Alcohol 2. Sensory ataxia * Vestibular * Proprioceptive * Visual 3. Other disorders of locomotion * Other central nervous system (e.g., cerebral) * Parkinson's disease

Answer 125

* Full neurological exam * Coordination exam * finger-to-nose, heel-to-shin, knee taps, rapid alternating movements * Stance and gait * Posture, stride length, width between feet, height of step, stability of pelvis, symmetry, arm swing, difficulty turning, tremor, elaborate/inconsistent movements, standing from sitting * Romberg test * pull test or push and release test for postural instability * gait: antalgic, hemiplegic, ataxic, apraxic, Parkinsonian gait, steppage gait, broad-based * tandem gait (heel-to-toe test)

Answer 126

Sudden onset, cerebellar ataxia, paresis (hemi-, para- or quadri-), bowel/bladder incontinence

Answer 127

Painful limb, decreased stance phase; quick/short steps

Answer 128

Distal weakness esp. dorsiflexors, difficulty clearing toes in swing phase→ audible double slap

Answer 129

As a result of spasticity – UMN lesions - knee extension + plantar flexion, difficulty clearing toes in swing phase→ circumduction gait due to the weakness of distal muscles and extensor hypertonia in LL

Answer 130

Proximal muscle weakness of hip abductors, pelvis excessively drops on unaffected limb during swing, to clear toes→ excessively laterally bend trunk on affected side

Answer 131

When the trendelenburg gait is bilateral in conditions such as Muscular dystrophies or Myopathies then they demonstrate bilateral excessive lateral trunk bending

Answer 132

Unsteady on feet→ is wide based (patients overshoot or undershoot with each step.)

Answer 133

Slow start, trunk bent forward, legs and arms stiff with short shuffling steps (aka festinating)

Answer 134

Increased muscle tone in hip adductors. Leg crosses the midline in swing phase and effects balance and stability. Base is very narrow. To compensate the trunk movements.

Answer 135

Muscle disease, weakness is generally proximal and symmetrical (patients have problems reaching for high objects, getting up from chairs and going up stairs). Tredelenberg gait

Answer 136

Muscular dystrophy, autoimmune disorders, myasthenia gravis (neuromuscular junction)

Answer 137

Nerve disease, distal weakness and sensory change in polyneuropathy – ‘glove and stocking’. Gait pattern is related to both weakness and sensory change. High steppage gait

Answer 138

Diabetic neuropathy, hereditary neuropathies (Charcot-Marie-Tooth Disease), Guillian Barre syndrome, peroneal n. injury, L5 radiculopathies

Answer 139

PD, neuroleptic-induced

Answer 140

Huntington’s disease

Answer 141

Variable steps, poor balance. movement disorder that causes involuntary, unpredictable body movements.

Answer 142

Involuntary posturing of the feet brought on by actions or intentions.

Answer 143

Cerebral palsy, birth injury, prenatal injury.

Answer 144

Patients have involvement on both sides with spasticity in lower extremities worse than upper extremities. The patient walks with an abnormally narrow base, dragging both legs and scraping the toes. This gait is seen in bilateral periventricular lesions, such as those seen in cerebral palsy. There is also characteristic extreme tightness of hip adductors which can cause legs to cross the midline referred to as a scissors gait.

Answer 145

Cerebral palsy

Answer 146

This is clinically commonly seen in stroke patients. Also seen in any brain (e.g. brain injury, brain abscess, brain tumor, multiple sclerosis etc) lesion that results in upper motor neuron lesions.

Answer 147

Patients can show dysmetria and intention tremor (only present during movement) on upper and lower limb testing (finger nose/heel shin). Ataxic gait

Answer 148

Alcoholic cerebellar degeneration (midline), stroke, tumor, demyelination

Answer 149

Most common ‘gait disorder’, adaptation to impairment, slower, short stride length, wide base – like walking on ice. Non-specific. May be associated with aging.

Answer 150

Early onset non-progressive ataxias associated with various syndromes as well as developmental abnormalities (e.g. Arnold-Chiari malformation, Dandy-Walker cysts)

Answer 151

* Neurodegeneration: e.g. multiple system atrophy * Systemic: alcohol, celiac sprue, hypothyroidism, Wilson's, thiamine deficiency, vitamin E deficiency * Toxins: carbon monoxide, heavy metals, lithium, anticonvulsants, solvents * Vascular: infarct, bleed, basilar migraine * Autoimmune: MS, Miller-Fischer (GBS)

Answer 152

Friedrich's ataxia, ataxia with oculomotor apraxia, ataxia telangiectasia, vitamin E deficiency

Answer 153

Signs: gait and limb ataxia, weakness, areflexia, extensor plantar reflex, impaired proprioception and vibration, dysarthria

Answer 154

Most commonly spinocerebellar ataxias (SCAs); 30+ types, most commonly due to CAG repeats

Answer 155

Ataxia and dysarthria, chorea, polyneuropathy, pyramidal and/or extrapyramidal features, dementia

Answer 156

Syncope is a sudden transient self-limited loss of consciousness from global cerebral hypoperfusion with spontaneous recovery

Answer 157

Preceding aura , tonic-clonic movements, tongue biting, urinary incontinence during episode, postictal state (confusion over 5 minutes after event vs return of consciousness with syncope)

Answer 158

Weakness, numbness, visual changes, CN symptoms (diplopia, dysphasia, dysarthria) other focal neuro deficits, CVA risk factors (HTN, CAD, DM, DLD, Afib, obesity)

Answer 159

Sudden onset, severe headache (thunderclap), nausea/vomiting, photophobia, CN palsy (III, IV)

Answer 160

SYNCOPE * Neurally-mediated (reflex) * Situational * Vasovagal (most common) * Carotid sinus hypersensitivity * Neurogenic - vestibular stroke, seizures, autonomic insufficiency * Cardiogenic * Arrhythmia (brady, SVT, VT, AFib) * Valvular - aortic stenosis, mitral stenosis, pulmonary stenosis, tricuspid stenosis * Vascular - pulmonary hypertension, pulmonary embolism * Pericardial - tamponade * Myocardial - myocardial infarction, hypertrophic cardiomyopathy * Orthostatic – 30-20-10 (HR increases 30, SBP 20, DBP10) * Hypovolemia - acute blood loss, anemia, nausea/vomiting/diarrhea, inadequate intake. Presyncope/ syncope upon sitting/standing quickly * ANS dysfunction - focal neurological deficits (MS , MSA, PD, DM). Presyncope/ syncope upon sitting/standing quickly * Drugs - anti hypertensive (diuretics, nitrate, ACEi), rate slowing drugs (B blockers, digoxin, amiodarone), QTc-prolonging drugs (phenothiazine, quinidine, psych meds) * Psychogenic * Eating - hypoglycemic

Answer 161

Sudden-onset syncope during exertion (HOCM) or when supine – may have CP, dyspnea, palpitations. FHx of sudden death, PMHx - HTN

Answer 162

Usually sudden and unprovoked (may have preceding palpitations). FHx of sudden death, PMHx - cardiac risk factors

Answer 163

Triggers: head rotation/pressure on carotid sinus (shaving, tight collar)

Answer 164

Triggers: walking, defecation, micturition, prolong standing, after exercise, history similar events

Answer 165

Triggers: Fear, heat, pain, stress, coughing, sneezing, GI stimulation/micturition (straining on BM or urine)

Answer 166

* Labs: CBCd, lytes, Cr/urea, glucose, Mg, Ca, TSH * Imaging: * CT head – if clinically indicated (focal neuro deficits) * Echocardiogram – if clinically indicated (valvular heart disease evaluation) * CXR – if chest discomfort, SOB, crackles in lungs * Special Tests: * ECG * Carotid massage * EEG – seizures * 24h Holter * Cardiac enzymes – rule out ACS * Exercise stress test – if concerned about exercise-induced arrhythmia * Tilt table test – confirms vasovagal syncope

Answer 167

Admit patient if they have 5 of any of the CHESS risk factors: CHF, Hematocrit\<30, ECG Abnormalities, SBP\<90mmHg, Shortness of Breath

Answer 168

Lie down if pre syncope, adequate fluids and salt intake, SSRI (paroxetine 20 mg PO daily), vasoconstrictor (midodrine 2.5 10 mg PO TID), permanent cardiac pacing if recurrent , compression stockings

Answer 169

Gradual staged movements with postural changes, exercises, increase salt/fluid intake, elastic stockings, and minimize antihypertensive medication use. Medications include fludrocortisone 0.05 0.1 mg PO daily, midodrine, pseudoephedrine, ephedrine, DDAVP, and potentially pyridostigmine

Answer 170

* CNS * Cortex/Thalamus,Brainstem * Spinal cord * PNS * Mononeuropathy * Radiculopathy * Polyneuropathy * Drugs: Chemotherapy, heavy metals, metronidazole * Psychogenic disorders – panic attacks, conversion disorder * Dermatological - herpes zoster, angioedema

Answer 171

Ischemic stroke, MS, inflammation, mass lesion (tumor, mets, abscess)

Answer 172

Trauma, compression (disc, tumor, mets, abscess), MS, infection (HIV, syphilis), cord infarction, vit B12 deficiency

Answer 173

Trigeminal neuralgia, carpal tunnel syndrome (median nerve), Saturday Night Palsy (radial nerve), ulnar nerve palsy, meralgia paresthetica, peroneal nere palsy

Answer 174

Disc herniation, osteophytes, infections, epidural abscess, arachnoiditis, herpes zoster

Answer 175

* DM, uremia, vasculitis, amyloidosis, hypothyroidism, Sjogren’s syndrome * Syphilis, HIV, lyme disease, EBV * EtOH, vit B12 deficiency, crticial illness polyneuropathy, GBS, paraneoplastic, hereditary neuropathies

Answer 176

Parasthesia/dysesthesia = tingling, pins and needles, prickling, burning, stabbing

Answer 177

* Hypoesthesia – numbness, loss of sensation, analgesia, ataxia * Difficult to detect on exam (numbness, diminution/absence of feeling)

Answer 178

Vibration, proprioception, pain, and temperature

Answer 179

* Quality, Timing, Migration, Progression * Distribution - focal, multifocal, unilateral or bilateral * Precipitating factors: recent trauma or injury, infection or illness, or occupational/environmental exposure, time of day, positional differences, anxiety provoking incidents that precede abnormal sensations * Associated symptoms: headache, weakness, rashes * PMHx: DM, stroke risk factors, Hx of autoimmune diseases, recent infections, malignancy * FHx - ask about childhood history of clumsiness or poor athletic performance, suggestive of hereditary cause

Answer 180

* Inspection: gait, atrophy, fasciculations, signs of trauma/injury or vasculitic rashes (purpura, livedo reticularis) * Motor exam (strength and tone) – unilateral weakness (stroke) * DTR * Sensory exam: Differentiate between spinothalamic tract deficits (using light touch, sharp versus dull discrimination and temperature discrimination as necessary) and dorsal column deficits (using vibration and tests of proprioception) * Special tests: Compression test, Tinel’s test and Phalen’s maneuver. Straight leg raise and cross straight leg tests are indicated if sciatica is suspected

Answer 181

* Labs * CBCd, glucose, HbA1c * Vit B12, TSH, urea, Cr * Rheumatolgic (ANA, ANCA, RF, anti-SSA, anti-SSB, CRP) * Infectious screen (HIV, HepBsAg, HBV/HCV serology) * Toxin screen (heavy metals) * Malignancy screen (SPEP, UPEP) * Radiology/Imaging * Central lesions: MRI, MRA, CT, CTA * Spinal cord lesions: MRI of brainstem down to spinal sensory level * LMN lesions: EMG, nerve conduction studies, muscle/nerve bx * Lumbar puncture is myelitis is suspected

Answer 182

Focal lesion of individual nerve trunk, numbness/weakness in nerve territory, caused by compression/trauma/inflammation

Answer 183

Carpal tunnel syndrome is most common, caused by median nerve compression between carpal bones, flexor tendons, and carpal ligament

Answer 184

Cubital tunnel syndrome is second most common, caused by ulnar nerve entrapment at elbow

Answer 185

Diffuse process that is dependent on length (often appears in longer nerves first) Stocking glove distribution. Symmetrical numbness, paresthesia. Associated with LMN findings – loss of reflex, weakness if motor nerve affected

Answer 186

* Neoplastic - carcinoma, lymphoma, MGUS – IgA, IgG, IgM * Infectious - sepsis, HIV, Lyme * Metabolic - DM, uremia in chronic kidney disease * Vitamin deficiency – malabsorption * Drugs - cisplatin, taxanes, vincristine, isoniazid, nucleoside analogue * Inflammatory (disorders causing primary vasculitis - RA, Sjogren syndrome, polyarteritis nodosa)

Answer 187

Guillain-Barre + neoplastic + drugs (taxanes) + chronic inflammatory demyelinating polyneuropathy, hereditary neuropathies

Answer 188

Subtype of polyneuropathy with isolated involvement of small nerve fibers (pain and temp). Pain, burning, aching in the feet particularly at night. Preserved vibration/proprioception – dissociated sense loss

Answer 189

If lesion is at carpal tunnel – LOAF muscles affected; if lesion at or above the elbow – may be lateral forearm wasting and the index finger held in extension (Benediction Sign)

Answer 190

Low lesion (below the wrist) characterized by marked hand clawing (because of unopposed flexor digitorum profundus flexion of DIPs) – high lesions have subtle clawing, termed ulnar paradox

Answer 191

Compression of the radial nerve in the arm causing wrist drop with numbness on the back of the hand

Answer 192

Lesion of trunk of an individual nerve root compression (herniated disc, abscess, tumors). See change in sensation, weakness, atrophy in territory of individual nerve root distal to lesion site (dermatomal, myotomal territory – unilateral) + back/neck and limb pain +/- reflex loss.

Answer 193

Degeneration of cell bodies in dorsal root ganglion. Affects all sensation – numbness, dysesthesia. Sensory ataxia, absent reflexes. Profound sensory loss is a hallmark of neuron sensory loss. NO MOTOR WEAKNESS. Very disabling than other neuropathies

Answer 194

* Often paraneoplastic – most common small cell lung cancer * Chemotherapeutic drugs, autoimmune

Answer 195

Complete lesion of the spinal cord resulting in a sensory level (loss of sensations below the level of the lesion), paraplegia (paralysis of both legs) or quadriplegia with higher cervical lesions. Urinary incontinence/retention. LMN findings at the level of lesion and UMN findings below level of lesion

Answer 196

Lesions involve the crossing spinothalamic fibers usually in cervical cord. Loss of pain and temperature across the upper back, shoulders “Cape Distribution Sensory loss”, sacral sparing. With increasing size – weakness of hand muscles due to motor neurons in the anterior horn

Answer 197

Diminished proprioception and vibration, on the side ipsilateral to the lesion. Decreased pinprick and temperature sensation on the contralateral side, weakness on ipsilateral side.

Answer 198

* Ipsilateral CN weakness (facial droop, ptosis, dysarthria, dysphagia), contralateral body weakness: cerebellar ataxia, Horner’s syndrome. * Sensory deficit in the ipsilateral face and contralateral body. Crossed signs – face (ipsilateral), limbs (contralateral).

Answer 199

Loss of pain and temp in the ipsilateral face, contralateral body (spinothalamic), ipsilateral ataxia (cerebellar fibers), ipsilateral Horner’s Syndrome (sympathetics), dysphagia, hoarseness (IX, X), vomiting, vertigo (VII)

Answer 200

Normal reflexes/tone; contralateral hemianesthesia, thalamic pain syndrome, contralateral homonymous hemianopsia, complex movement disorders, eye movements, can mimic cortical

Answer 201

Central post-stroke pain in the recovery phase of strokes. Sensory exam normal but severe pain + allodynia in contralateral hemibody

Answer 202

* Hyperreflexia, spasticity (velocity dependent), “A words” (aphasia: loss of acquired language, alexia: inability to read, agraphia: trouble writing, agnosia: inability to interpret sensations + recognize things, anosognosia: denial of neurological deficit, esp. paralysis, acalculia: inability to do simple math, astereognosis: cannot ID object by touch, agraphesthesia: cannot ID figures when written on skin); seizures, altered cognition, personality changes, hemiparesis (face \> arm \> leg MCA territory, leg \> arm \> face ACA), visual field defects (occipital lobe), neglect (esp. R parietal), memory loss

Answer 203

Carpal tunnel syndrome is treated conservatively with night splints and glucocorticoid injections, oral glucocorticoids, surgical decompression

Answer 204

Carbamazepine, gabapentin, lamotrigine, microvascular decompression, rhizotomy

Answer 205

Glycemic control; pain control; foot care; gabapentin, pregabalin, amitryptyline

Answer 206

Radiculopathies are managed pharmacologically with NSAIDs, analgesics, and muscle relaxants, or with physiotherapy and injections. Open discectomy/microdiscectomy

Answer 207

Peripheral neuropathies are treated symptomatically with analgesia and anticonvulsants such as gabapentin and pregabalin. Tricyclic antidepressants and SNRIs may also be used. Transcutaneous electronic nerve stimulation (TENS) has been shown to be effective in some people.

Answer 208

* Fine paresthesias in toes and fingertips \> weakness in lower/upper extremities (often ascending) \> potential autonomic dysfunction (5%), cranial nerves, and respiratory involvementAscending glove and stocking distribution (sensory AND motor) * Ascending motor \> sensory symptoms (but still get BOTH) * Areflexia: key feature (but preserved early on) + low/mid back pain * Check for predominant proximal and distal weakness and numbness suggesting polyradiculoneuropathy such as Guillain-Barre syndrome

Answer 209

Campylobacter, CMV, URTIs, possibly flu shots

Answer 210

EMG (demyelinating neuropathy) + lumbar puncture (cytoalbuminergic dissociation = normal CSF WBC but increased CSF protein) + PFT (decreased FVC, MIP, MEP)

Answer 211

Plasmapheresis first then IVIG 0.5-1g/kg IV daily + ICU admission with respiratory support if FVC \<20ml/kg or max inspiratory pressure \<30 or max expiratory pressure \<40 or rapid progression (\<7 days) or autonomic involvement

Answer 212

Autoimmune condition that targets acetylcholine receptors

Answer 213

Dysphagia, diplopia, proximal muscle wasting, fatigable weakness

Answer 214

Acetyl choline receptor antibodies

Answer 215

IVIG, dexamethasone, severe plasmaphoresis

Answer 216

Defined as the abnormal synchronized firing of electrical impulses in the brain causing a change in motor activity and/or behavior. Essentially, excitation of cortical neurons overcomes normal inhibition.

Answer 217

* Startle (Moro) reflex * GERD (Sandifer syndrome) * Breath-holding spells * Syncope * Psychogenic non-epileptic attacks (e.g. pseudoseizures) * Movement/tic disorders * Sleep disorders * Migraine variants * Myoclonic jerks secondary to brain injury - Lack of inhibition

Answer 218

Syncope - Shaking after a LOC. Difference is that syncope has some dizziness and the LOC happens before the shaking. Seizure - movement starts before LOC. In syncope you don’t have a prolonged post-ictal confusion

Answer 219

* Generalized * Focal/Partial

Answer 220

* Both hemispheres simultaneously, no aura, seizure may start with a scream then evolve into tonic (stiffening) and then clonic (twitching or jerking) activity. Lasts for 60-90s. Heavy breathing at the end, foaming at mouth, tongue biting and bladder incontinence. Loss of consciousness * May have prodrome of unease or irritability hours to days before

Answer 221

* Clonic: whole body repetitive rhythmic jerking movements * Tonic: whole body muscle rigidity in flexion or extension

Answer 222

Partial onset seizure with secondary generalization

Answer 223

Hyperventilation

Answer 224

Generalized seizure that starts at 5-10yo but usually disappears as you get older. Starts abruptly without aura. Child may exhibit rapid eye blinking/fluttering – spacing out. 5-10s. Can be activated by hyperventilation! No aura or post-ictal fatigue/confusion.

Answer 225

* Myoclonic jerks – no preceding aura. Early morning myoclonus, Brief and bilateral but not always symmetric, Consciousness is preserved so patient aware of jerking movement * Triad of myoclonic jerk, generalized tonic-clonic seizure, absences

Answer 226

Is a brief loss of muscle tone, lasting only a few seconds that may cause injury for instance if the patient falls, or the head drops unexpectedly. If the patient falls, this is commonly referred to as a "drop attack".

Answer 227

* Arising from an area of a brain then spreads * Patient may appear to be awake but with impairment of awareness (not LOC)

Answer 228

* Can arise from any area of the brain (focal or lateral), no LOC/intact awareness

Answer 229

* Aura: Happens right before a seizure starts (seconds) – déjà vu, jamais vu, vision problems, dizziness, odd smells, sounds, headache, nausea * Prodrome (hours to days before): changes in mood, sleep changes, anxiety, problems with focus * Preceding Events: sitting still vs. going to the bathroom - urination/defecation ~ vasovagal syncope * Preceding Symptoms: sweating + light-headedness + nausea - ~ syncope * Trigger: head injury + strenuous physical exercise (arrhythmia) + preceding illness (febrile seizure)

Answer 230

* Motor Symptoms: repetitive non-purposeful movements + staring + lip-smacking + fainting + stiffening of extremities + rhythmic shaking of extremities + unilateral or bilateral * Autonomic/Sensory Symptoms: nausea + feeling odd/peculiar + bowel/bladder control loss + numbness + tingling + odd smells or sounds + any change in skin color (cyanosis ~ seizure) + drooling + vomiting + tongue biting (~seizure) * Responsiveness: during spell + eyes open/closed + eyes attending to environment vs. fixed * Most seizure activity cannot be interrupted with verbal/physical stimulation * Duration: \<30 seconds more likely syncope, \>30 seconds more likely seizure

Answer 231

Awareness during ictal phase + eye fluttering + bystanders can modulate symptoms intensity + pelvic thrusting + slow onset + non-rhythmic + closed eyes + no post-ictal confusion + situation dependent

Answer 232

Post-event amnesia/confusion/lethargy/tiredness + headaches + muscle aches + transient focal weakness of one side (Todd’s Paresis) + nausea + vomiting + how long until back to baseline

Answer 233

Absence or frontal lobe seizures

Answer 234

* Vitals (sinus rhythm? HR? Orthostatic hypotension? Mental status?) * Always Examine for: meningismus + signs of systemic illness + toxidromes + tongue biting * Neurological Exam * Cardiac Exam

Answer 235

* Blood Work * CBC, lytes, Na, Ca, glucose, troponin, urea * CK, TSH, INR, AST, ALT, bilirubin, ALP, b-HCG, tox screen * Prolactin * Imaging * Emergency non-contrast CT if significantly aLOC + new focal deficit + no return to baseline * MRI * Special Tests: * EEG as outpatient to get license back - Don’t need to repeat this if history of seizures; sleep deprived EEG best * Do drug level if seizure and S/E * LP (if suspect meningitis/encephalitis)

Answer 236

\<1yo + significant acute cognitive/motor impairment + unexplained abnormal neuro exam + focal onset seizure without generalization + abnormal focal EEG

Answer 237

After 2nd seizure or if high risk for recurrent seizures then treat after first

Answer 238

* Under 2: Phenobarbital, Levitiracetam (Keppra) * Over 2: Carbamazepine, Lamotrigine

Answer 239

* Under 2: Phenobarbital, Levitiracetam * Over 2: Valproate, Topiramate, Carbamazepine

Answer 240

Ethosuximide (Zarontin)

Answer 241

Valproic Acid (Depakene/Epival) or Phenobarbital

Answer 242

Risk of second seizure is 30-50%, then after second seizure risk of next one is \>80%

Answer 243

Take precautions with fire, water, heights, and roads + no driving until cleared by physician (no driving after 3 months, and must be seizure free for 6 months)

Answer 244

Benign paroxysmal disorder occurring in healthy children usually provoked by emotional upset or minor injury - unintentional involuntary reflex

Answer 245

* 6m – 6y; breath holding progressing to cyanosis or syncope * There is an antecedent event, heart rate decreases and the child becomes pale, and there is NO post-ictal period.

Answer 246

Two types are cyanotic (see facial cyanosis), and pallid (child becomes very pale) - soon thereafter infant regains consciousness and breathes normally.

Answer 247

Investigations/Management: ECG for first spell to rule out prolonged QT - reassurance, sometimes blowing air forcefully on face will terminate episode early

Answer 248

Benign childhood epilepsy with centrotemporal spikes (often first thing in the morning, awakes them from sleep); self-limited/benign (between 5-10 years) – often see single nocturnal seizure with clonic movement of the mouth accompanied by gurgling sounds (seizures usually resolve by 16 years old) and may be associated with migraine Start in face (drooling) and then move into arm and then leg. As it moves to legs, consciousness is not preserved

Answer 249

Usually eyes are closed/rolled upwards + kids/babies are sleeping + notice motor ‘twitch’

Answer 250

Precipitated by fever + normal development + no focal neurologic deficits after the seizure + 1 in 24hrs + generalized + \<15m + \<5y old = simple febrile seizure. If not, then “complex” and higher risk of recurrence

Answer 251

No diagnostic testing needed - if still seizing can give IV lorazepam or midazolam + reassurance that they are harmless and do not cause brain damage; if 5+ febrile seizures = you have epilepsy - note that antipyretics don’t prevent febrile seizures

Answer 252

\>5 minutes of continuous seizure activity or 2+ seizures without returning to baseline mental status - usually generalized tonic-clonic

Answer 253

ABG + CBCd + lytes + extended lytes (Mg/Ca/Po) + Cr + glucose + toxic screen + antiepileptic drug level

Answer 254

* ABCs + O2 + establish IV + monitors * If Hypoglycemia: thiamine 100mg IV + 50% dextrose 50ml IV * First Line: lorazepam 2mg q1-3min IV push or rectal diazepam if no IV access * Second Line: phenytoin 20mg/kg IV, no faster than 50mg/min – start continuous monitor * Note: give benzos and phenytoin in DIFFERENT IVs – they precipitate

Answer 255

Alcohol and illicit drug use/withdrawal, brain injury/abnormality (tumour, trauma, vascular), CNS infection, fever (children), metabolic (hypoglycemia, electrolyte abnormalities, liver/renal failure), medications - bupropion, theophylline, isoniazide, imipenem, high dose penicillin, meperidine, or be a genetic or inherited cause

Answer 256

Any of 1/3 criteria - =\> 2 unprovoked seizures \>24h apart; 1 unprovoked seizure with probable seizure -\>60% over next 10 years (e.g. a structural lesion associated with high risk of recurrence)

Answer 257

Genetic, structural (e.g. prior stroke, tumour, meningo/encephalitis, perinatal insult, vascular malformation, malformation of cortical development, neurodegenerative disorders), or unknown

Answer 258

Sandifer syndrome

Answer 259

Post-ictal phase: flaccid limbs, extensor plantar reflexes, headache, confusion, aching muscles, sore tongue, amnesia, elevated serum CK lasting hours; may have focal paralysis (Todd’s paralysis)

Answer 260

Seizures are precipitated by sleep deprivation, alcohol ingestion, flickering light and awakening from sleep

Answer 261

* Clonic * Tonic * Tonic-clonic (grand mal) * Absence * Myoclonic * Atonic

Answer 262

Dystonic posturing, clonic movements, forceful turning of eyes and/or head, focal muscle rigidity/jerking ¬± Jacksonian march (spreading to adjacent muscle groups)

Answer 263

Unusual sensations affecting vision, hearing, smell, taste, or touch

Answer 264

Epigastric discomfort, pallor, sweating, flushing, piloerection, pupillary dilatation

Answer 265

Automatisms

Answer 266

Focal seizures that spread and involve the 2 hemispheres. Looks like tonic-clonic seizure

Answer 267

* Benzodiazepines: lorazepam 1mg IV/SL PRN up to a total dose of 0.1mg/kg or diazepam 10mg PO q6h and 5mg PO q2h PRN * Antiepileptic: fosphenytoin 20mg/kg IV or phenytoin 300mg IV over 10 min or phenobarbital, carbamazepine, valproate * If EtOH Withdrawal: add thiamine 100mg IV/PO daily + multivitamin 1 tab IV/PO daily + folate

Answer 268

Most common, must be generalized, \<15 minutes and must not recur within a 24 hr period. Must have a normal developmental history, no evidence of CNS infection and usually a FHx of febrile seizures

Answer 269

Either focal, prolonged or recurs within 24 hour. Indicates more serious underlying etiology such as encephalitis, meningitis, or an underlying seizure disorder.

Answer 270

Motionless stare at onset, aura (fear/anxiety, deja-vu, odd smell, rising epigastric sensation), automatisms (lip-smacking, or gestures). Duration is 30-120seconds. May have secondary generalization

Answer 271

Abrupt onset, prominent motor and complex gestural automatisms , forced vocalization, secondary generalization is COMMON as is nocturnal concurrence. Rapid recovery. Can be misdiagnosed as pseudoseizures.

Answer 272

Simple partial onset, decreased vison, forced blinking, ocular flutter. Contralateral head/eye deviation, secondary generalization.

Answer 273

* Generalized * Myopathy (proximal \> distal weakness) * endocrine: hypothyroidism, hyperthyroidism, Cushing’s syndrome * rheumatologic: polymyositis, vasculitis * infectious: HIV, influenza * other: collagen vascular disorders, steroids, statins, alcohol, electrolyte disorders * NMJ (MG, botulism, LEMS, organophosphate poisoning) * Cachexia * Localized * UMN (vasculitis, abscess, brain tumour, vitamin B12 deficiency, MS, stroke) * radicular pain (i.e. nerve root) * anterior horn cell (spinal muscular atrophy, ALS, polio, paraneoplastic) * peripheral neuropathy (peroneal muscle atrophy, GBS, leprosy, amyloid, myeloma, DM, lead toxicity)

Answer 274

* Chronic illness (cardiac, pulmonary, anemia, infection, malignancy) * Depression, deconditioning

Answer 275

* 0 - no contraction * 1 - flicker of contraction – no movement * 2 - active movement with gravity eliminated * 3 - active movement against gravity * 4 - active movement against gravity and resistance (4-, 4, 4+) * 5 - normal power

Answer 276

Normal bulk, spastic/hypertonicity, weakness, upper extremity flexors stronger, lower extremity extensors stronger→ pyramidal position, hyperreflexia, babinski +, contralateral pronator drift, positive forearm rolling + finger tapping tests

Answer 277

^resistance to passive displacement across a joint, velocity dependent

Answer 278

Atrophy, fasciculations, flaccid/normal tone, hypo/areflexia, plantar responses down-going, +/-sensory distortion

Answer 279

Note change in sensation, weakness, atrophy in territories of several individual nerves distal to lesion site +/- reflex loss +/- autonomic changes

Answer 280

Suggests systemic process. Usually due to vasculitis. Lesions of trunks of several individual nerves (not at usual sites of entrapment/compression) – ischemia secondary to vasculitis

Answer 281

Proximal, symmetric muscle weakness (hips/shoulders→ trendelenburg gait) +/-tenderness, atrophy, hyporeflexia, increased CK→ necrosis; more likely chronic/insidious, no sensory Sx, pain related to disability, respiratory + swallowing usually later/absent

Answer 282

Chronic/progressive, autosomal dominant (50% inheritance risk, trinucleotide repeat, DMPK gene, variability with age of onset

Answer 283

Appearance: ptosis, bifacial weakness, frontal baldness (including women), triangular face giving a drooping/dull appearance

Answer 284

* Distribution of weakness: distal weaker than proximal (in contrast to other myopathies), steppage gait * Myotonia: delayed relaxation of muscles after exertion (elicit by tapping on thenar muscles with hammer) * Cardiac: 90% have conduction defects (1o heart block; atrial arrhythmias) * Respiratory: hypoventilation 2o to muscle weakness * Ocular: subcapsular cataracts, retinal degeneration, decreased intraocular pressure * Other: DM, infertility, testicular atrophy * EMG: subclinical myotonia - long runs with declining frequency and amplitude

Answer 285

* Management of myotonia: phenytoin * Genetic counselling, monitor cardiac, respiratory, swallowing function; supportive therapies

Answer 286

Fluctuating weakness (fatiguability), usually oculobulbar first (diplopia, lid droop – ptosis, dysarthria, dysphagia, drooling), or generalized to muscles of respiration. No pain, no change in sensation, and no alteration in stretch reflexes or presence of increased CK or atrophy.

Answer 287

Autoimmune condition due to anti-Ach or anti-muscle specific kinase antibodies, resulting in early saturation at the NMJ and inadequate muscle activation with increasing nerve stimulation

Answer 288

* Fatigable, symmetric, or asymmetric weakness without reflex changes, sensory changes, or coordination abnormalities * Ocular (diplopia/ptosis), bulbar (dysarthria/dysphagia), and/or proximal limb weakness * Symptoms may be exacerbated by infection, pregnancy, menses, and various drugs * Respiratory muscle weakness may lead to respiratory failure

Answer 289

Clinical Dx, ice pack test (ocular signs reverse with cooling), edrophonium test (tensilon test): reduces muscle weakness by blocking acetylcholinesterase enzymes prolonging ACh in synaptic cleft, anti-ACh R antibodies

Answer 290

* First line treatment: anticholinesterase drugs (pyridostigmine) * Corticosteroids (e.g.prednisone): If acetylcholinesterase inhibitors not effective * Immunosuppression (e.g. azathioprine, cyclophosphamide, mycophenolate): can be used as steroid-sparing therapy * Short-term immunomodulation (e.g. IVIG and plasma pheresis): for crisis * Thymectomy: option in non-thymomatous MG; 85% remission rate

Answer 291

Hallmark symptom is intermittent pain and numbness in feet/legs while walking from narrowing of the spinal cord “neurogenic claudication”

Answer 292

Disease affecting motor neurons diffusely (infectious – poliomyelitis / degenerative – ALS). Weakness if bilateral but often asymmetric; bulbar – dysarthria, dysphagia (but NOT oculomotor), atrophy, and fasciculations. No change in sensation.

Answer 293

Progressive neurodegenerative disease that causes UMN and LMN symptoms and is ultimately fatal

Answer 294

* Limb motor symptoms: segmental and asymmetrical UMN and LMN symptoms * Bulbar findings: dysarthria (flaccid or spastic), dysphagia, tongue atrophy and fasciculations, facial weakness and atrophy * Pseudobulbar affect, frontotemporal dementia (up to 10%) * Sparing of sensation, ocular muscles, bowel, bladder, sphincters * Swallowing failure = aspiration. Respiratory muscle weakness = ventilator failure.

Answer 295

* Riluzole * Symptomatic relief * Spasticity/cramping: baclofen, tizanidine, regular exercise, and physical therapy * Sialorrhea: TCA (i.e. amitriptyline), sublingual atropine drops, parotid/submandibular Botox (rare) * Pseudobulbar affect: dextromethorphan/quinidine, TCA, SSRI * Non-pharmacologic: high caloric diet, ventilatory support (especially BiPAP), early nutritional support (i.e. percutaneous endoscopic gastrostomy tube), rehabilitation (PT, OT, SLP), psychosocial support

Answer 296

Vit B12 deficiency, syphilis, specific stroke/MS lesion, vit E. deficiency

Answer 297

* Fibromyalgia/Chronic fatigue syndrome * Polymyalgia rheumatica (PMR) * Mental health disorders (e.g., depression, somatic symptom disorders)

Answer 298

Age of onset typically \>50yr, F:M=2:1

Answer 299

* Constitutional symptoms prominent (fever, weight loss, malaise) * Pain and stiffness of symmetrical proximal muscles (neck, shoulder and hip girdles, thighs) * Shoulder symptoms reflect proximal bursitis (eg, subdeltoid, subacromial) and less often bicipital tenosynovitis or joint synovitis. * Gel phenomenon (stiffness after prolonged inactivity) - Stiffness in the morning is typical and lasts \> 60 minutes.

Answer 300

Physical exam reveals tender muscles, but no true weakness or atrophy

Answer 301

Often shows anemia of chronic disease, elevated platelets, elevated ESR and CRP, and normal CK; up to 5% of PMR reported with normal inflammatory markers

Answer 302

Start with low prednisone dose of 15- 20mg PO OD, reconsider diagnosis if no response within several days Taper slowly over 1yr period with closely monitoring Relapses should be diagnosed and treated on clinical basis; do not treat a rise in ESR as a relapse Monitor for steroid side effects, glucocorticoid-induced osteoporosis prevention, and follow for symptoms of GCA

Answer 303

* Abrupt monocular loss of vision, pain over the temporal artery, jaw claudication, scalp tenderness, * Constitutional symptoms, and PMHx of polymyalgia rheumatica * Ischemic optic atrophy * 50% lose vision in contralateral eye if untreated

Answer 304

More common in women \>60yr

Answer 305

* Temporal artery biopsy + increased ESR and CRP (ESR can be normal, but likely 80-100 in first hour) * If biopsy is negative, biopsy contralateral side

Answer 306

* High dose corticosteroids (12mos) [no other immunosuppressants allow steroid sparing!], calcium/vit D/bisphosphonates, low dose ASA to prevent strokes * If diagnosis of GCA is suspected clinically: start STAT treatment + perform temporal artery biopsy to confirm diagnosis within 2 wk of initial presentation

Answer 307

Chronic (\>3mo), widespread (axial, left-and right-sided, upper and lower segment), non-articular pain with characteristic tender points

Answer 308

A patient satisfies diagnostic criteria for fibromyalgia if the following 3 conditions are met: 1. Widespread Pain Index (WPI) ≥7 and Symptom Severity (SS) scale score ≥5 or WPI 3–6 and SS scale score ≥9 2. Symptoms have been present at a similar level for at least 3 mo 3. The patient does not have a disorder that would otherwise explain the pain

Answer 309

* F:M=3:1 * Primarily ages 25-45yr, some adolescents * Overlaps with chronic fatigue syndrome and myofascial pain syndrome * Strong association with psychiatric illness

Answer 310

* Wide spread aching, stiffness * Easy fatigability * Sleep disturbance: non-restorative sleep, difficulty falling asleep, and frequent wakening * Symptoms aggravated by physical activity, poor sleep, emotional stress * Patient feels that joints are diffusely swollen although joint examination is normal * Neurologic symptoms of hyperalgesia, paresthesias, allodynia * Associated with irritable bowel or bladder syndrome, migraines, tensionH/As, restless leg syndrome, obesity, depression, and anxiety

Answer 311

Should reveal only tenderness with palpation of soft tissues, with no specificity for trigger/tender points

Answer 312

* Blood work: includes TSH and ESR; all typically normal unless unrelated, underlying illness present * Serology: do not order ANA or RF unless there is clinical suspicion for a connective tissue disease * Laboratory sleep assessment

Answer 313

* Education * Exercise program (walking, aquatic exercises), physical therapy (good posture, stretching, muscle strengthening, massage) * Stress reduction, CBT * No evidence for alternative medicine such as biofeedback, meditation, acupuncture

Answer 314

* low dose tricyclic antidepressant (e.g. amitriptyline) * for sleep restoration * select those with lower anticholinergic side effects * SNRI: duloxetine, milnacipran * anticonvulsant: pregabalin, gabapentin * analgesics may be beneficial for pain that interferes with sleep (NSAIDs, not narcotics)

Answer 315

Nociceptive pain is due to chemical, thermal, and/or mechanical damage activating nociceptors and inflammatory response.

Answer 316

Neuropathic pain is caused by damage to or disorders of the central or peripheral nervous system that results in pain perception without actual tissue injury.

Answer 317

Unprovoked burning, shooting, or lancinating pain

Answer 318

Spontaneous abnormal non-painful sensation (e.g. tingling)

Answer 319

Evoked pain with inappropriate quality or excessive quantity

Answer 320

A dysesthetic response to a non-noxious stimulus

Answer 321

An exaggerated pain response to anoxious stimulus

Answer 322

Nausea, drowsiness (No driving at all on initiation or adjustment of opioids because of drowsiness), pruritis, constipation (Everyone gets constipated and this never goes away - Need to also give them a laxative), addiction, pseudo-addiction, withdrawal, tolerance, opioid induced neurotoxicity

Answer 323

* Step 1: non-opioids (acetaminophen or NSAID) * Step 2: weak opioids - codeine and tramadol. These both also have ceiling effects at certain daily doses. Tramadol can be serotonergic so be mindful of serotonin syndrome * Step 3: strong opioids - oxycodone, methadone, hydromorphone, morphine. Sometimes you can go directly to step 3 if you're sure that's the appropriate first thing to try

Answer 324

Vivid dream, hallucinations, myoclonus, confusion, hyperalgesia

Answer 325

Goal is to catch it early, screen everyone for these symptoms. Hydration, decrease/rotate opioid, educate patient/caregivers, usually takes 3-4 days for metabolites to clear

Answer 326

10% of total daily dose

Answer 327

* Add up BTA + ATC = total 24 hr dose; divide by 6 for new q4h dosing schedule * Increase ATC dosing by 20-30% (to nearest tablets that are possible)

Answer 328

Corticosteroids (Usually use dexamethasone for the corticosteroid), bisphosphonates

Answer 329

Antidepressants (Duloxetine), corticosteroids, anticonvulsants (gabapentin much cheaper than pregabalin, carbamazepine)

Answer 330

* Physical (PT, acupuncture, chiropractic manipulation, massage) * Psychoeducational (CBT, family therapy, education, psychotherapy)

Answer 331

* Hyperalgesia, allodynia * Subjectively described as burning, heat/cold, pricking, electric shock, perception of swelling, numbness * Can be spontaneous or stimulus evoked, distribution may not fall along classical neuro-anatomical lines * Associated issues: sleep difficulty, anxiety/stress/mood alteration

Answer 332

* Sympathetic: CRPS * Non-sympathetic: damage to peripheral nerves * Systemic disease: DM, thyroid disease, renal disease, rheumatoid arthritis, multiple sclerosis * Nutritional/toxicity: alcoholism, pernicious anemia, chemotherapy * Infectious: post-herpetic, HIV * Trauma/compression: nerve entrapment, trigeminal neuralgia, post-surgical, nerve injury, cervical/lumbar radiculopathy, plexopathy * Central: abnormal CNS activity * Phantom limb, post spinal cord injury, post stroke, MS

Answer 333

Stepwise approach (Canadian Pain Society, 2014) * 1st line: gabapentinoids, TCA, SNRI * 2nd line: tramadol, opioid analgesics * 3rd line: cannabinoids * 4th line: topical lidocaine (second line for postherpetic neuralgia), methadone, lamotrigine, lacosamide, tapentadol, botulinum toxin

Answer 334

* Neuropsychiatry: CBT, psychotherapy * Rehabilitation: physiotherapy

Answer 335

Dorsal column neurostimulator, DBS (thalamus)

Answer 336

* Recurrent episodes of sudden onset, severe, excruciating, unilateral, paroxysmal, shooting "electric" pain in trigeminal root territory (V3\>V2\>\>V1) * May have normal sensory exam * Pain lasts seconds/minutes over days/weeks; may remit for weeks/months

Answer 337

Triggers: touching face, eating, talking, cold wind, shaving, applying make-up

Answer 338

* Classic TN: compression of CN V by tortuous blood vessel (usually superior cerebellar artery) * 2o TN: cerebellopontine angle tumour (5%), MS (5%) * Idiopathic TN

Answer 339

* Clinical diagnosis * Investigate for secondary causes, which are more likely if bilateral TN or associated sensory loss * MRI to rule out structural lesion, MS, or vascular lesion

Answer 340

* First line: carbamazepine or oxcarbazepine * Second line: baclofen or lamotrigine * For medically-refractory classic TN, consider microvascular decompression * Neurosurgical options for medically-refractory TN: trigeminal ganglion percutaneous technique, gamma knife radiosurgery, invasive percutaneous denervation (radiofrequency/glycerol), percutaneous balloon microcompression, microvascular decompression * Narcotics not generally recommended

Answer 341

* Pain persisting in the region of a cutaneous outbreak of herpe szoster * Constant deep ache or burning, intermittent spontaneous lancinating/jabbing pain, allodynia * Distribution: thoracic, trigeminal, cervical, lumbar, sacral * Associated symptoms: impaired sleep, decreased appetite, decreased libido

Answer 342

* Varicella zoster vaccine (Varivax) in childhood reduces incidence of varicella zoster * Herpes zoster vaccine (Zostavax or Shingrix) reduces incidences of shingles, PHN, and other herpetic sequelae * Zostavax is a live vaccine, recommended for patients \>60 yr old * Shingrix is a recombinant vaccine, recommended for patients \>50 yr old (more efficacious than Zostavax)

Answer 343

Destruction of the sensory ganglion neurons (e.g. dorsal root, trigeminal, or geniculate ganglia) secondary to reactivation of herpes zoster infection

Answer 344

* Medical: TCA (e.g. amitriptyline), anti-convulsants (e.g. pregabalin, gabapentin), analgesia (e.g. opiates, lidocaine patch), intrathecal methylprednisolone, topical capsaicin * Early treatment of acute herpes zoster with antivirals (longer-acting famciclovir and valacyclovir more effective) * Treatment of herpes zoster with corticosteroids DOES NOT decrease PHN * Surgical: spinal tractotomy, dorsal root entry zone lesion, DBS of thalamus

Answer 345

Deltoid (axillary n), Biceps (musculocutaneous n), Brachioradialis (radial n)

Answer 346

C7 but also has some C8 and occasionally C6

Answer 347

L2, L3, L4

Answer 348

L5, S1, S2

Answer 349

L4, L5, S1

Answer 350

* Ischemia * Nerve entrapment – nerve compressed by nearby anatomic structures, often secondary to localized, repetitive mechanical trauma with additional vascular injury to nerve * Direct trauma (e.g. transection, laceration, contusion) * Iatrogenic

Answer 351

* Demyelination: damage to myelin sheath. * Wallerian degeneration: axonal damage leading to degeneration of the nerve distal to injury but not proximally, makes sense because if you damage upstream, downstream is messed.

Answer 352

* Electrophysiological studies: EMG/nerve conduction study (assess nerve integrity and monitoring recovery after 2-3 wk post-injury) * Labs: blood work (e.g. CBC, TSH, Vitamin B12), CSF * Imaging: C-spine, chest/bone x-rays, myelogram, CT, magnetic resonance neurography, angiogram if vascular damage is suspected

Answer 353

Clinical exam: muscle bulk and tone, power, sensation, reflexes, localization via Tinel's sign (paresthesias elicited by tapping along the course of a nerve)

Answer 354

* Neurapraxia (class I) * Axonotmesis (class II) * Neurotmesis (class III)

Answer 355

Axon structurally intact but fails to function. Though we have some demyelination there is no axonal damage and so we do not have Wallerian degeneration (part of axon separates distal to cell body). Demyelination – reduced conduction velocity. Neuropraxia affects thick myelinated fibres usually and has a very good prognosis for recovery (3-4months).

Answer 356

Axon and myelin sheath disrupted but endoneurium and supporting structures intact → Wallerian degeneration of axon segment distal to injury. Axons can regenerate at 1-2mm/day. Better prognosis with distal injuries (less growth needed and nerve can survive).

Answer 357

Nerve completely transected

Answer 358

Conservative: Prevent repeated stress/injury, physiotherapy, NSAIDs, local anesthesia/steroid injection

Answer 359

Surgical: Nerve decompression ± transposition for progressive deficits, muscle weakness/atrophy, failure of medical management

Answer 360

Follow-up clinically for recovery; exploration if no recovery in 3 mo

Answer 361

If no evidence of recovery, resect damaged segment. Prompt physical therapy and rehabilitation to increase muscle function, maintain joint ROM, maximize return of useful function. Recovery usually incomplete

Answer 362

* Surgical repair of nerve sheath unless known to be intact (suture nerve sheaths directly if ends approximate or nerve graft [usually sural nerve]). * Clean laceration: early exploration and repair. * Contamination or associated injuries: tag initially with nonabsorbable suture, reapproach within 10 d

Answer 363

1. Hyperkinetic 2. Bradykinetic 3. Tremor

Answer 364

* Tics * Primary (sporadic and inherited) * Tourette syndrome * Huntington disease * Secondary * Infections (e.g., encephalitis, Creutzfeldt-Jakob) * Drugs (e.g., stimulants, levodopa) * Dystonia * Primary (sporadic and inherited) * Dystonia plus syndromes (e.g., medication) * Stereotypies (typically with mental retardation or autism) * Chorea/Athetosis/Ballism * Essential tremor * Myoclonus

Answer 365

* Parkinson disease * Wilson disease * Huntington disease

Answer 366

Subjective generalized restlessness relieved by voluntary stereotypic movements (e.g. squirming)

Answer 367

Brief, unpredictable, irregular movements, flowing from one body part to another; can appear purposeful in milder forms

Answer 368

Large-amplitude, involuntary, flinging movements that are most commonly unilateral (hemiballism) due to vascular lesion at STN

Answer 369

Abnormal muscle contractions cause involuntary writhing movements, usually distally

Answer 370

* Rhythmicity * Speed * Duration (continuous vs episodic) * Location (distal or proximal) * Suppressibility

Answer 371

Rhythmic oscillation of a body part around a joint with alternating contraction in agonist/antag muscle groups

Answer 372

Can be described as rhythmic, can be fast or slow, is continuous, distal (often involves the hand) and not suppressible

Answer 373

* Resting tremor * Action tremor * Postural tremor * Intention tremor * Task-specific tremor * Kinetic tremor

Answer 374

* Characteristics: 3-7 Hz pillrolling * Worse with – rest while concentrating * Associated Sx: TRAP

Answer 375

PD, Parkinsonism, Wilson’s disease, mercury poisoning

Answer 376

Occurs when a person maintains a position against gravity, such as holding the arms outstretched.

Answer 377

Carbidopa-levodopa, surgery, DBS

Answer 378

* Characteristics: 6-12 Hz fine tremor * Worse with – sustained posture (outstretched arms) * Associated Sx: Autosomal dominant FHx

Answer 379

Physiologic, essential, hyperthyroidism, hyperglycemia, heavy metal poisoning, CO poisoning, drug toxicity, sedative/alcohol withdrawal

Answer 380

Propranolol, primidone, topiramate

Answer 381

Produced with purposeful movement toward a target, such as lifting a finger to touch the nose. Typically the tremor will become worse as an individual gets closer to their target.

Answer 382

* Characteristics: \<5 Hz coarse tremor * Worse with – finger to nose * Associated Sx: Cerebellar findings

Answer 383

Cerebellar disorders, Wilson’s disease, MS, anticonvulsants, alcohol, sedatives

Answer 384

Only appears when performing highly-skilled, goal-oriented tasks such as handwriting or speaking.

Answer 385

Associated with any voluntary movement, such as moving the wrists up and down or closing and opening the eyes.

Answer 386

* No additional symptoms predating tremor * Made better with alcohol * Present for years * Affects ability to do fine tasks, present all the time to some degree * Fast, small amplitude. * Present bilateral hands * Worse with posture, finger to nose, writing

Answer 387

Cerebellar tremor is typically a slow, high-amplitude (easily visible) tremor of the extremities (e.g., arm, leg) that occurs at the end of a purposeful movement

Answer 388

Can appear as any form of tremor. The tremor increases in times of stress and decreases or disappears when distracted. Many individuals with psychogenic tremor have an underlying psychiatric disorder such as depression or post-traumatic stress disorder (PTSD).

Answer 389

Physiologic tremor occurs in all healthy individuals. It is rarely visible to the eye and typically involves a fine shaking of both of the hands and also the fingers. It is not considered a disease but is a normal human phenomenon

Answer 390

* Sporadic: Combination of oxidative stress to dopaminergic neurons, environmental toxins (e.g. pesticides), accelerated aging, genetics * Familial (10%): autosomal dominant alpha-synuclein or LRRK2 mutations, autosomal recessive parkin, PINK1, or DJ-1mutation (juvenile onset) * MPTP(neurotoxin)

Answer 391

* Anosmia – loss of smell * REM Sleep Behaviour Disorder * New tremor * Noticed by others at first cause mostly present with rest * Doesn’t impair functioning as much (only present at rest) * Slow, present at rest * Unilateral * Disappears with movements

Answer 392

* Risk: family history, male, head injury, rural living, exposure to certain neurotoxins * Protective: coffee drinking, smoking, NSAID use, estrogen replacement in post-menopausal women

Answer 393

* Loss of dopaminergic neurons in pars compacta of substantia nigra – decreased dopamine in striatum - 1. disinhibition of the indirect pathway, and 2. decreased activation of the direct pathway - increased inhibition of cortical motor areas * alpha-synucleinopathy: alpha-synuclein accumulates in Lewy bodies and causes neurotoxicity in substantia nigra

Answer 394

TRAP: Tremor (resting), Rigidity, Akinesia/bradykinesia, Postural instability

Answer 395

* Bradykinesia with either rest tremor or rigidity * 2 or more supportive criteria (clear and dramatic beneficial response to dopaminergic therapy, levodopa- induced dyskinesia, rest tremor of a limb, olfactory loss/cardiac sympathetic denervation on MIBG scintigraphy) * Asymmetry of signs – starts unilateral, stays asymmetric although bilateral disease, absence of autonomic dysfunction/dementia/EOM abnormalities (early) with absence of other causes like drugs that block dopamine.

Answer 396

1. Negative motor * Bradykinesia: slow, small amplitude movements, fatigue from rapid alternating movements, difficulty initiating movement, starts unilateral 2. Positive motor * resting tremor: typically 4-6 Hz "pill-rolling" tremor, especially in hands * rigidity: lead-pipe rigidity with cogwheeling due to superimposed tremor 3. Asymmetric onset of tremor, rigidity, bradykinesia 4. Progressive course * related findings: masked facies, hypophonia, aprosody (monotonous speech), dysarthria, micrographia, shuffling gait with decreased arm swing * freezing of gait: occurs with walking triggered by initiating stride or barriers/destinations, lasting seconds * postural instability: late finding presenting as falls * cognition: bradyphrenia (slow to think/respond), dementia (late finding) * behavioural: decreased spontaneous speech, depression, sleep disturbances, anxiety * autonomic: constipation, urinary retention, sexual dysfunction, orthostatic hypotension, clinostatic hypertension

Answer 397

REPLACE DOPAMINE: levodopa/carbidopa (Sinemet) or levodopa/benserazide (Prolopa)

Answer 398

Dopamine agonists, MAOI, anticholinergics (especially if prominent tremors), catechol-O-methyltransferase inhibitors

Answer 399

* thalamotomy * pallidotomy * deep brain stimulation (thalamic, pallidal, subthalamic) - this helps the motor symptoms

Answer 400

* SSRIs first line * TCAs (beware fall risk, cognitive impairment, and worsening symptoms of Parkinson's disease)

Answer 401

Amantadine

Answer 402

Tauopathy with limited vertical gaze (down gaze more specific) that can be overcome by the oculocephalic reflex, early falls, wide-based unsteady gait, axial rigidity; and akinesia, dysarthia, and dysphagia

Answer 403

Tauopathy with varied presentations but classically presenting with unilateral parkinsonism, dystonia/myoclonus, and apraxia ± "alien limbs" phenomenon; ¬± progressive non-fluent aphasia

Answer 404

Synucleinopathy presenting as either cerebellar predominant (MSA-C, previously olivopontocerebellar atrophy) or parkinsonism predominant (MSA-P, previously nigrostriatal degeneration); both are associated with early autonomic dysfunction (blood pressure, breathing, bladder function and muscle control)

Answer 405

Multi-infarct presentation with gait instability and lower body parkinsonism; less likely associated with tremor

Answer 406

Atrophy of putamen/cerebellum and putaminal hypointensity with rim hyperintensity on T2W – with HOT CROSS BUN SIGN in basis pontis

Answer 407

Sudden, brief, shock-like movement that is nonsuppressible. Faster than tic, usually distal limbs.

Answer 408

* Focal – single body part * Segmental – 2+ contiguous body regions (body parts close together) * Multifocal – 2+ regions, not contiguous * Generalized - we can kind of experience this when falling asleep and having a jerk that wakes you up

Answer 409

Characterized by recurrent episodes of frequent limb movements while sleeping. It mostly happens in the lower parts of the body like the toes, ankles, knees and hips.

Answer 410

Commoner causes of myoclonus that may be seen in primary care include: medication side effects, toxin or chemical ingestion, metabolic encephalopathy, hypoxic-ischemic encephalopathy, myoclonus in the elderly, in the setting of an epilepsy syndrome

Answer 411

Co-contraction of agonist and antagonist muscles causing sustained twisting movements which can be tonic (dystonic postures) or phasic (dystonic movements)

Answer 412

familial, sporadic (torticollis, blepharospasm, writer'scramp)

Answer 413

Dystonia-plus syndromes results from nondegenerative, neurochemical disorders associated with other neurological conditions; dopa-responsive dystonia, myoclonus-dystonia

Answer 414

Thalamotomy, stroke, CNS tumour, demyelination, PNS injury, drugs/toxins (L-dopa, neuroleptics, anticonvulsants, Mn, CO, cyanide, methanol)

Answer 415

Heredodegenerative dystonia generally results from neurodegenerative disorders in which other neurological symptoms are present and in which heredity plays a role. Parkinsonian disorders, Wilson's disease, Huntington

Answer 416

* Sustained or intermittent twitching movements caused by co-contraction of agonist and antagonist muscles * Symptoms exacerbated by fatigue, stress, and emotions; relieved by sleep or specific tactile/ proprioceptive stimuli ('geste antagoniste', e.g. place hand on face for cervical dystonia) * More likely to be progressive and generalized if younger onset or leg dystonia

Answer 417

Local medical: botulinum toxin

Answer 418

Systemic medical: anticholinergics (benztropine), muscle relaxants (baclofen), or benzodiazepines, dopamine depletors (tetrabenazine); dopamine for dopa-responsive dystonia

Answer 419

Surgical: surgical denervation of affected muscle, stereotactic thalamotomy (unilateral dystonia), posteroventral pallidotomy, or DBS

Answer 420

Genetics: autosomal dominant CAG repeats (with anticipation) in huntingtin (HTT) gene on chromosome 4, which leads to accumulation of defective protein in neurons

Answer 421

Global cerebral atrophy, especially affecting the striatum, leading to increased activity of the direct pathway, and decreased activity of the indirect pathway

Answer 422

* Typical progression: insidious onset with clumsiness, fidgetiness, and irritability, progressing over 15yr to major NCD, psychosis, and chorea * major NCD: progressive memory impairment and loss of intellectual capacity * chorea: begins as movement of eyebrows and forehead, shrugging of shoulders, and parakinesia (pseudo-purposeful movement to mask involuntary limb jerking) * Progresses to dance-like or ballism, and in late stage is replaced by dystonia and rigidity * Mood changes: irritability, depression, anhedonia, impulsivity, bouts of violence

Answer 423

Parkinsonism, dystonia, rigidity, seizures

Answer 424

* MRI * enlarged ventricles, atrophy of cerebral cortex, and caudate nucleus * Genetic testing * expansion of the cytosine-adenine-guanine (CAG) trinucleotide repeats in the HTT gene * CAG repeats (\>28) on chromosome 4p16.3 that encodes the protein huntingtin

Answer 425

* No disease-altering treatment * Psychiatric symptoms: antidepressants and antipsychotics * Chorea: neuroleptics and benzodiazepines * Dystonia: botulinum toxin

Answer 426

Suppressible, distractible, suggestible, variable, worsen with stress or excitement, premonitory urge - describe something that predicts something bad will happen

Answer 427

A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization

Answer 428

* tics may wax and wane in frequency but have persisted for an extended period of time * onset is \<18 yr * disturbance is not attributable to the physiological effects of a substance or another medical condition

Answer 429

Multiple motor and \>1 vocal tics that have persisted for \>1yr since onset

Answer 430

* Genetic factors: \>55% concordance rate in monozygotic twins. * Prenatal/perinatal factors: Older paternal age, obstetrical complications, maternal smoking, and low birth weight. * Psychological factors: Symptom exacerbations with stressful life events.

Answer 431

* Psychoeducation. * Behavioral interventions-habit reversal therapy. * Medications-utilize only if tics become severely impairing or also treating comorbidities. Due to the fluctuating course of the disorder, it can be difficult to determine medication efficacy. * Alpha-2 agonists: guanfacine (first choice), clonidine (more sedating).

Answer 432

Single or multiple motor or vocal tics (but not both motor and vocal) that have persisted for \>1 yr since onset

Answer 433

Single or multiple motor and/or vocal tics present for \<1yr since first tic onset

Answer 434

Encephalitis, CJD, Sydenham's chorea, head trauma, drugs, mental retardation syndromes

Answer 435

* Simple tics: short duration (msec) * Complex tics: longer (seconds), more purposeful and often include a combination of simple tics

Answer 436

* simple: blinking, head jerking, shoulder shrugging, extension of the extremities * dystonic: bruxism (grinding teeth), abdominal tension, sustained mouth opening * complex: copropraxia (obscene gestures), echopraxia (imitate gestures), throwing, touching

Answer 437

* simple: blowing, coughing, grunting, throat clearing * complex: coprolalia (shout obscenities), echolalia (repeat others' phrases), palilalia (repeat own phrases)

Answer 438

Dopamine blockers, dopamine depletors (tetrabenazine), clonidine, clonazepam, or DBS

Answer 439

Patients with impairment in comprehension and/or use of the form, content, or function of language

Answer 440

Patients with impaired articulation, fluency and voice

Answer 441

* Delayed and developmental language impairment (e.g., deafness, autism spectrum disorder, neglect, abuse) * Degenerative, vascular, or other central nervous system disorders (e.g., stroke) * Head injury

Answer 442

* Articulation disorder (e.g., dysarthria) * Fluency (e.g, stuttering, Parkinson disease) * Speech apparatus lesions (e.g., cleft palate, head and neck neoplasm)

Answer 443

An acquired disturbance of language characterized by errors in language production, writing, comprehension, or reading

Answer 444

Language production (expressive)

Answer 445

Comprehension of language (receptive)