Neurology

Question 1

Mild Cognitive Impairment

Answer 1

Mild cognitive impairment (MCI) is an intermediate state between normal cognition and dementia.

• 10-15%/y conversion to dementia;  with smaller temporal lobe volume (MRI), apoE4 allele
• No evidence that therapy delays progression or how to diagnose it

Question 2

Dementia

Diagnosis?

Answer 2

The American Academy of Neurology has published guidelines for tests that should be performed when making a diagnosis of dementia.

• NC CT or MRI
• NO genetic testing (tau mutations, beta-amyloid, APOE E4 allele)
• CSF ONLY when suspecing CJD - testing for the 14-3-3 protein
• B/W: B12, thyroid
• Depression screen
• Screening tests: MMSE, clock drawing, mini-cog, MOCA (canadian)

Question 3

Primary Dementia Syndromes

and they are?

Answer 3

Think APPLE

• AD (60%): slow, insidious, little findings other than disorientation, visuospatial probs, depression
• Parkinson/plus: see Parkinson’s
• Pick’s (FTD): pick cells, apathetic, disinhibited, more prominent cog. deficit at presentation
• Lewy body disease: neuronal inclusions, less early mem probs, v hallucinations, parkinsonian
• Endothelial (Vascular): AKA Binswanger’s - no specific treatment other than stroke prevention
• ?Huntington’s

Question 4

Severity of Dementia (MMSE)

Question 5

Dementia

Secondary Causes?

Answer 5

Important b/c potentially reversible

• Mimics: 3 D’s (depression, delirium, developement d/o)
• Drugs: EtOH, antihistamine/diarrheal/depressant/epileptic
• Infections/Infarct/inflammation: HIV, syphilis, whipple’s, CJD, vasculitis, MS
• Metabolic: B12, thyroid, vit E, cortisol, electrolytes, liver dz
• Structural: bleed, tumors, CSF (NPH)

Question 6

Huntington’s Disease

Answer 6

Defn: Sx: Findings: Mgmt: Note:

Question 7

Normal Pressure Hydrocephalus / NPH

What is it?

Answer 7

Hydrocephalus is either communicating or not communicating type.

DIG: Dementia, Incontinence, Gate disturbance.

Imaging: large ventricles with shallow cerebral sulci

Treatment: repeat or large volume lumbar punctures, VP shunts, SA drains.

Selection for shunt surgery requires positive response to supplementary tests such as large volume LP, extended CSF divergent by a lumbar spinal catheter, or CSF outflow resistance tests

Question 8

Frontal Lobe Dementia

Answer 8

Defn: Sx: Findings: Mgmt: Note:

Question 9

Treatment of Dementia (pharmacologic)

Answer 9

We suggest a treatment trial with a cholinesterase inhibitor for patients with mild to moderate dementia (MMSE 10-26). The choice between donepezil, rivastigmine, and galantamine

In patients with moderate to advanced dementia (MMSE memantine (10 mg twice daily) to a cholinesterase inhibitor, or using memantine alone in patients who do not tolerate or benefit from a cholinesterase inhibitor

In patients with severe dementia (MMSE memantine even in severe dementia, given the possibility that memantine may be disease-modifying.

Question 10

Alzheimer’s Dementia

Diagnosis & prognosis

Answer 10

The form of cerebral amyloidosis from an accumulation of amyloid beta – protein. Incurable.

In AD, memory impairment, specifically loss of memory of recent events, is an essential feature of AD and is usually its first manifestation. Language function and visuospatial skills tend to be affected relatively early, while deficits in executive function and behavioral symptoms often manifest later in the disease course.

Diagnosis:

• Prominent Memory Impairment: newly acquired information (early) and remote events (late)
• And one or more of:
• Aphasia: language disturbance
• Apraxia: inability to carry out skilled motor activities despite intact motor function
• Agnosia: failure to recognize/identify entities despite intact sensory function
• Executive dysfunction: planning, organizing, sequencing, abstracting
• Psychiatric manifestations (BPSD) - Major depression (5-8%), Psychosis (20%)
• Motor manifestations (late) – Parkinsonism (30% late in the course)
• Gradation of Severity:
• Mild MMSE 20-26 - mild functional dependence (ie. finances)
• Moderate MMSE 10-20 - increased dependency (drive, shop, hygiene) and memory impairment
• Severe MMSE <10 - state of total dependence and need for constant supervision
• Treatment:
• Disease modifying
  
  • Cholinesterase inhibitors: donepezil (Aricept), rivastigmine (Exelon), galantamine (Reminyl)
    
    • Mild-moderate AD - shown to improve cognitive function and some behavioural Sx
  • Vitamin E (1000 IU bid) and Selegine (5 mg bid)
    
    • Moderate AD - Delays progression by 230 and 215 days respectively
  • NMDA Receptor Blockers (Memantine 20 mg daily)
    
    • Moderate-Severe AD - shown to improve global function and cognition
  • Other: Ginko Biloba (mild effect but many drug interactions), tacrine (hepatotoxic)
• Behavioural management
  
  • Low dose neuroleptic (Seroquel); trazodone for sleep disturbance
• Symptom relief and support (family/caregiver relief)
• Prognosis:
  
  • Progressive decline leading to death (average survival is 10 years)

Question 11

Alzheimer’s Dementia

Treatment

Answer 11

There are no treatments either to stop the progression or reverse the disease.

Mild to moderate (MMSE 10-26): any centrally acting anticholinesterase

• donepezil, rivastigmine, and galantamine
• NSAIDs, ginko, ERT and vitamin E have some benefit

Moderate to advanced dementia (MMSE<10):

• N-methyl-D-aspartate (NMDA) receptor antagonist memantine (10 mg twice daily) to a cholinesterase inhibitor, or using memantine alone in patients who do not tolerate or benefit from a cholinesterase inhibitor

A 28-week randomized trial in 252 patients with Mini Mental Status Examination (MMSE) scores of 3 to 14 (mean approximately 8) at study entry found that memantine significantly reduced deterioration on multiple scales of clinical efficacy

Question 12

Diffuse Lewy Body Disease

Answer 12

Lewy body dementia = Dementia + Parkinsonism + visual hallucinations + dysautonomia + sleep disorders + neuroleptic sensitivity.

Clinical features:

Central Feature (essential for Dx)

Dementia (progressive cognitive decline)

Core Features (2 :probable DLB, 1: possible DLB)*

Fluctuating cognition

Recurrent well-formed, detailed visual hallucinations

Spontaneous features of parkinsonism

Suggestive Features (1+core feature: may Dx probable DLB, ≥1: may Dx possible DLB)*

REM sleep disorder

Severe neuroleptic sensitivity

Low dopamine transporter uptake in basal ganglia on SPECT or PET

Question 13

Lewy Body Dementia (treatment)

Answer 13

Dementia with Lewy bodies (DLB) is increasingly recognized clinically as the second most common type of degenerative dementia after Alzheimer disease (AD). In addition to dementia, distinctive clinical features include: visual hallucinations, parkinsonism, cognitive fluctuations, dysautonomia, sleep disorders, and neuroleptic sensitivity. s with all of the degenerative dementias, cognitive decline in Dementia with Lewy bodies (DLB) progresses inexorably to death. Psychotic symptoms, particularly visual hallucinations, tend to persist in patients with DLB [1]. Parkinsonism also worsens over time, especially in patients for whom this is an early feature [2]. In one retrospective series, individuals with impairment of visuospatial skill on baseline neuropsychological testing had a faster rate of clinical decline compared with those who did not [3].

While some have reported that the rates of cognitive decline are similar for Alzheimer disease (AD) and DLB [4-7], others report a more stable course for DLB [8], and others have found a faster rate of cognitive decline in DLB

Treatment of Dementia with Lewy bodies (DLB) is symptomatic, targeted toward specific disease manifestations, and based upon somewhat limited evidence. There are no treatments with evidence of disease-modifying effects.

General strategies — Because medications may be poorly tolerated in DLB, nonpharmacologic, behavioral strategies aimed at modifying stressors in the environment should be employed whenever possible. (See “Treatment of behavioral symptoms related to dementia”, section on ‘Nonpharmacologic management’.) Physical therapy and mobility aids may help in the management of parkinsonism.(See “Nonpharmacologic management of Parkinson disease”.)

Patient and caregiver education regarding risks, benefits, and limitations of treatments is important. In many cases, treatment choices represent a trade-off between parkinsonism and psychosis, and the relative preferences of the patient and caregiver will be decisive.

Cognition and neuropsychiatric disturbances

Cholinesterase inhibitors — Cholinesterase inhibitors may represent a first line pharmacologic treatment in DLB. While cholinesterase inhibitors were initially developed for Alzheimer disease (AD), the evidence suggests that cholinergic deficits are even greater in DLB. Multiple anecdotal reports [13,14], open-label studies [15-17], and one randomized, controlled trial [18] suggest that cholinesterase inhibitors are efficacious in DLB, with reported benefit not only in cognition, but also for fluctuations, psychotic symptoms, and parkinsonian symptoms. In some instances, the response has been dramatic, but temporary. The effect size has been reported to be larger than that in patients with AD.

Memantine — Memantine has reported efficacy in moderate to severe Alzheimer disease and vascular dementia. A randomized controlled trial of 72 patients with either DLB or Parkinson disease dementia found that patients treated with memantine for 24 weeks performed better on the primary outcome assessment measure, the clinical global impression of change, but not on most of the other secondary outcome measures [23]. In a subsequent 24-week trial that compared memantine to placebo in 199 patients with either DLB or Parkinson disease dementia, the subset of 75 patients with DLB (but not PDD or the group overall) demonstrated improved outcome on the same primary outcome measure, but again not on most secondary outcome measures [24]. In both of these studies, memantine was well-tolerated. However, some other studies have noted that worsening of delusions and hallucinations with memantine may be particularly problematic in patients with DLB [25,26].

REM sleep disorder — REM sleep disorder often responds to low doses of clonazepam (0.25 to 1.5 mg) or melatonin (3 to 12 mg) given at bedtime [27-29]. Melatonin may be preferred in the setting of cognitive impairment. Quetiapine (12.5 mg) may also be successful, but is probably a third choice in this population.

Neuroleptics — The potential for severe neuroleptic sensitivity reactions, including exacerbation of parkinsonism, confusion, or autonomic dysfunction, limits the usefulness of neuroleptic medications in DLB.

ther psychotropic medications — There have been no systematic studies of the use of antidepressants, anxiolytics, benzodiazepines, or anticonvulsants in the treatment of the behavioral and psychiatric symptoms in DLB [4]. Selective serotonin reuptake inhibitors are commonly used in the treatment of depression. Benzodiazepines are generally AVOIDED (except for REM sleep disorder), especially for long term use, because of the potential for worsening confusion, gait disorder, and paradoxical agitation. Tricyclic agents are AVOIDED because of their anticholinergic properties. Electroconvulsive therapy has been successfully employed in depressed patients with DLB [35]. (See “Treatment of behavioral symptoms related to dementia”.)

Antiparkinson medications — Treatment of parkinsonian symptoms in DLB is similar to that for Parkinson disease (PD), if somewhat less successful [36-38]. (See“Pharmacologic treatment of Parkinson disease”, section on ‘Symptomatic therapy’.)

There is concern that these medications may exacerbate psychotic symptoms in DLB, but our clinical experience and small case series suggest that by using a conservative approach - small doses and slow upward titration - these agents are generally effective and well tolerated [21,39]. Worsening of psychotic symptoms and REM sleep disorder may require addition of a small dose of an atypical neuroleptic [39,40].

Levodopa seems to be more effective than dopamine agonists and produces fewer side effects [41]. A suggested initial dose is one-half tablet of carbidopa-levodopa (Sinemet) 25/100 mg three times daily, titrated upward over several weeks as tolerated and according to the clinical response. Anticholinergic agents are generally avoided in DLB because they may worsen cognitive function.

Orthostatic hypotension — Medical therapy can improve symptoms of orthostatic hypotension in most DLB patients [42,43]. We have used fludrocortisone, midodrine, and a combination of the two successfully in several of our patients. Anticholinergic agents for the treatment of urinary incontinence should probably be avoided in DLB patients who have orthostatic hypotension.

Question 14

Ischemic Cerebrovascular Disease

Ddx?

Answer 14

Anatomic

• Non thrombotic:
  
  • Watershed: hypotension/shock - bilateral weakness
  • Vasospasm: cocaine, migraine - focal neuro signs
  • Vasculopathy: FMD, dissxn, vaculitis - various
• Arterial:
  
  • Embolic: cardiac (AF, DCM, IE), coaulopathy (APLS, SCD)
  • Atherosclerosis/thrombosis: large vs small/lacunar
• Venous:
  
  • Sagittal sinus thrombosis

Question 15

Cold Caloric Testing

Answer 15

In cases of cervical fractures or indeterminate OCR testing, the VOR should be tested with cold calorics. After visual inspection to make sure that the tympanic membrane is intact, 10 cc of ice water is infused into the external auditory canal, and the eye movements are observed over the next 30 to 60 seconds. In a conscious person, the eyes will slowly move conjugately toward the side of the ice water infusion (due to ipsilateral inhibition of the vestibular complex), followed by a fast, compensatory phase away from the irrigated ear (driven by the contralateral frontal eye field). In the setting of coma due to bihemispheric disease, the fast “corrective phase” is absent (due to frontal lobe dysfunction), and the eyes remain deviated toward the side of the cold water infusion. In the setting of brainstem injury, there are no eye movements.

Question 16

Midbrain CVA

Answer 16

WEBERS SYNDROME:

Midbrain CVA involving the 3rd nerve nucleus and cerebral peduncle – causes ophthalomoplegia & ptosis on ipsilateral side & paralysis of contralateral limb.

It is caused as a result of occlusion of the paramedian branches of the posterior cerebral artery or of basilar bifurcation perforating arteries.

Horner’s?

Question 17

Pontine Bleed

Cranial nerves VI and VII are most commonly affected but III, IV, IX and X may also be involved.

Pin point pupil!

Answer 17

Depends on Size, location of bleed..

• A large central hemorrhage: progressive decrease in the level of consciousness rapidly leading to coma. Bilateral bulbar muscle weakness, “pinpoint” pupils, hyperthermia, and hyperventilation (or abn breathing pattern – 70%) are common associated findings. (seen most often in patients with uncontrolled hypertension).
• Contralateral hyperhidrosis in the subacute or late phase after pontine hemorrhage may be seen (thought to be secondary to disruption of contralateral inhibitory sweating pathway).
• Up to one third of patients may develop a severe headache before the onset of focal signs Vomiting may be seen in 20% of patients, and seizures (mostly flexor spasms and not true convulsions) have been reported in 30% of patients.
• Ophthalmological examination may show small pupils, absence of horizontal eye movements, and ocular bobbing.
• With large hemorrhages, bulbar musculature is invariably affected. This may increase the risk of aspiration. Silent aspiration may be difficult to recognize in the comatose patient.
• Massive pontine hemorrhage is invariably fatal; 80% of patients die within 48 hours (Silverstein 1982). Most hemorrhages occurred in the midpons at the junction of basis pontis and tegmentum. Hemorrhages frequently spread to the midbrain but rarely into the medulla. They frequently rupture into the fourth ventricle.
• Smaller hemorrhages in the tegmentum or basal regions may present with focal signs and no alteration in consciousness. Usually unsuspected until after the CT or MR scans.
• Hemorrhages restricted to the basal region present with hemiplegic deficits or ataxia-hemiparesis. Clinically, such syndromes are indistinguishable from lacunar infarction in the same region.
• With hemorrhages restricted to the lateral tegmentum, sensory deficits, ataxia, and oculomotor abnormalities (one-and-one-half syndrome, internuclear ophthalmoplegia, horizontal gaze palsies and ocular bobbing) are common findings(Payne et al 1978; Caplan and Goodvin 1982).
• Isolated symptoms such as sixth nerve palsy, sometimes bilateral (Kellen et al 1988), hemisensory or isolated facial sensory(Toratani et al 2008) disturbances, or trigeminal neuropathy (Berlit 1989) have been reported with small hemorrhages. Dystonia may be associated with pontomesencephalic lesions (Loher and Krauss 2009). Recently, phantom arm and leg phenomena have been noted after pontine hemorrhage (Tanaka et al 2008). Early recovery is seen in most patients, and complete resolution of deficits is common. In a study of 80 patients with primary pontine hemorrhage, the initial level of consciousness and the transaxial size of hematoma on CT were strongly related to the outcome (Murata et al 1999).

Question 18

Complex Regional Pain Syndrome

Answer 18

Defn:

Sx: - I) is characterized by severe pain or burning sensation, most commonly in the hand or foot, with associated swelling, trophic skin changes, and signs or symptoms of vasomotor instability. When the hand is affected, there may be associated pain or limitation of the ipsilateral shoulder (shoulder-hand syndrome).

Findings:

Mgmt:

Note:

Question 19

Multiple Sclerosis

Answer 19

Defn: Sx: Findings: Mgmt: Note:

Question 20

Oligoclonal Banding

Answer 20

Defn: Note:

Question 21

L’hermittes Sign

Answer 21

Defn: Note:

Question 22

Parkinsonism (and meds)

Answer 22

Defn: Note: - domperidone is safe as it does not cross BBB

Question 23

Dyskinesia (and meds)

Answer 23

Defn: Note:

Question 24

Dystonia (and meds)

Answer 24

Defn: Note:

Question 25

Neuroleptic Malignant Syndrome

Answer 25

Defn: Due to massive dopamine blockade; risk factors neuroleptics, antiemetics [high-dose or rapid dose increase], concurrent illness dehydration\exhaustion\poor nutrition Sx: FARM (24-72 hours) fever (>39), autonomic hyperreactivity, rigidity, MSE Findings: elevated CK, LDH, AST, leukocytosis, myoglobinuria Mgmt: discontinued drug ( results in 1-2 weeks), aggressive hydration, cooling measures, dantrolene Note:

Question 26

Alcoholic Seizure

Answer 26

Defn: Sx: usually within 12-48 h after last drink, chronic ETOH Findings: TC, usually singular, status rare Mgmt: BZD, phenobarbital Note: Dilantin is ineffective

Question 27

Transient global amnesia

Answer 27

Transient global amnesia is syndrome of reversible anterograde amnesia. Prognosis is generally benign. Anatomically, temporal lobe and hippocampus are sites of neurologic involvement. No clear etiology. Patients are disoriented in time and often repetitively ask questions about date or their environment. Immediate recall is intact, but delayed recall is impaired. Retrograde amnesia is common. A loss of self-awareness excludes TGA. Patients have no problems with complex motor tasks. Treatment is not required.

Attention is spared, visual-spatial skills are intact, and social skills are retained. Symptoms typically last less than 24 hours. As the syndrome resolves, the amnesia improves, but the patient may be left with a distinct lapse of recollection for events during the attack.

Question 28

Ménière’s disease (Endolymphatic Hydrops) think michelin’s man

Answer 28

Defn: distention of the labyrinthine system compressing the perilymphatic spaces Sx: vertigo lasting hours, N&V, since selling euro hearing loss tinnitus and aural fullness Findings: audiometric confirmation is suggestive Mgmt: betahistine Note:

Question 29

Subarachnoid hemorrhage SAH (Diagnosis)

Answer 29

The sensitivity of head CT for detecting SAH is highest in the first 6 to 12 hours after SAH (nearly 100 percent)

Xanthochromia for the detection of cerebral aneurysms had a sensitivity and specificity of 93 and 95 percent. The presence of xanthochromia indicates that blood has been in the CSF for at least two hours. Only limited data





Question 30

Indications for an LP (HA in ED)

Answer 30

• Indications for LP include the following:
• First or worst headache of a patient’s life
• Severe, rapid-onset, recurrent headache
• Progressive headache
• Unresponsive, chronic, intractable headache
• Neuroimaging (CT or MRI scan) should precede LP to rule out a mass lesion and/or increased intracranial pressure.

Question 31

Falx meningioma

Answer 31

Meningiomas can occur anywhere from dura. Seizures common with focal deficits (visual changes), progressive unilateral visual loss, hearing loss (cerebellopontine angle), extremity weakness (parasagittal meningioma), progressive sequence of ipsilateral arm, then leg, then contralateral weakness from foramen magnum meningioma, progressive leg weakness and numbness (spinal).

Question 32

Internal Capsule CVA

Answer 32

Defn: Sx: Findings: Mgmt: Note:

Question 33

ACA CVA

Answer 33

Defn: Sx: Findings: Mgmt: Note:

Question 34

Drug-Induced Hypersensitivity Syndrome

Answer 34

Drug-induced hypersensitivity syndrome — Anticonvulsant hypersensitivity syndrome (AHS) is a rare but serious event associated with aromatic antiepileptics.

idiosyncratic drug reaction, 1 in 1000 to 1 in 10,000 patients treated with phenytoin; fatality of 10 %.

Symptoms - fever, rash, lymphadenopathy, pharingitis and other organ involvement. When severe, the syndrome can include hepatitis, megaloblastic anemia, rhabdomyolysis, and arteritis.

Occurs two months of starting phenytoin. The diagnosis is frequently missed, and must be considered in any patient on phenytoin with suggestive symptoms. Patients who develop the hypersensitivity syndrome are at risk for recurrence and may exhibit similar reactions to other anticonvulsants

Question 35

Diplopia (causes)

Answer 35

• Damage to the third, fourth, or sixth cranial nerves, which control eye movements. • Cancer • Trauma • Multiple sclerosis • Fluoroquinolone antibiotics[6] • Botulism • Guillain-Barré syndrome • Brain tumor • Sinusitis • Abscess • Wernicke’s syndrome • Graves disease • Drunkenness • Orbital myositis • Myasthenia gravis[7] • Salicylism • Strabismus Lyme Disease

Question 36

Carbamazepine

Answer 36

Indication: SEs: Dosage: Notes:

Question 37

Guillain-Barre Synrome

Answer 37

Defn: Sx: Findings: Mgmt: Note: positive babinski r/o

Despite frequent complaints of paresthesias, objective sensory changes are minimal. A well-demarcated sensory level should not be observed in patients with GBS; such a finding calls the diagnosis of GBS into question.

Reflexes are absent or reduced early in the disease course. Hyporeflexia or areflexia of involved areas represents a major clinical finding on examination of the patient with GBS. Pathologic reflexes, such as the Babinski sign, are absent. Hypotonia can be observed with significant weakness.

Question 38

Diabetes (CN 6 palsy)

Answer 38

Defn: Sx: Findings: Mgmt: Note: r/o malignant Otitis externa

Question 39

Central cord syndrome

Answer 39

Defn: Sx: Findings: Mgmt: Note:

is bilateral motor paresis greater in the upper than in the lower extremities. The paresis is usually greater distally than proximally, and there is variable sensory involvement (figure 2). This pattern occurs because the most central portions of the spinal tracts contain fibers from the upper extremities.

Impairment in the upper extremities is usually greater than in the lower extremities and is especially prevalent in the muscles of the hand.

Sensory loss is variable, although sacral sensation is usually present. Anal wink, anal sphincter tone, and Babinski reflexes should be tested.

Muscle stretch reflexes may initially be absent but will eventually return along with variable degrees of spasticity in affected muscles.

Bladder dysfxn is common

Question 40

Diffuse axonal Injury

Answer 40

Defn: Sx: Findings: Mgmt: Note:

Question 41

Cerebellar Tremor

Answer 41

Defn: Sx: Findings: Ddx: MS Other causes include Friedreich’s ataxia, spinocerebellar degeneration, and cerebellar infarction.

Question 42

Wilson’s Disease

Answer 42

Defn: Sx: Findings: Mgmt: Note: Could have all 3 tremors

Question 43

Perilymphatic Fistula symdrome

Answer 43

Perilymphatic or labyrinthine fistula is a condition in which an abnormal communication is present between the perilymphatic space of the inner ear and the middle ear or mastoid. The manifestations of this disease vary in severity and complexity, commonly ranging from very mild to incapacitating. Perilymphatic fistulas (PLFs) may induce hearing loss, tinnitus, aural fullness, vertigo, disequilibrium, or a combination of these symptoms. Weber et al, in a follow-up article, confirmed that surgical repair of perilymph fistula does not result in a significant risk of postoperative hearing loss and that fistula repair may prevent further hearing loss, even in patients in whom a perilymph fistula was not identified at the time of surgery

Question 44

Migraine (Red Flags)

Answer 44

Red flag symptoms:

• thunderclap head ache
• progressive head ache over days to months
• new onset after age 40
• precipitated by valsalva maneurver or exertion, nocturnal occurrence or morning awakening, systemic syptoms, neurologic symptoms, etc.

Question 45

Babinski Response

Answer 45

• Pathologic response
  *

Question 46

Congenital Stenosis (diagnosis)

Question 47

Indications for Carotid endarterectomy (CEA)

Answer 47

CEA should be considered for any patient with carotid artery stenosis in whom surgery will improve the natural history of the disease to a greater degree than the corresponding medical treatment would.[7]

In symptomatic good-risk patients with surgical morbidity and mortality (stroke and death) of less than 6%, proven indications for CEA include the following:

• One or more transient ischemic attacks (TIAs) in the preceding 6 months and carotid artery stenosis exceeding 50%[3]

Acceptable but not proven indications include the following:

• Ipsilateral TIA and carotid artery stenosis exceeding 70%, combined with required coronary artery bypass grafting (CABG)
• Progressive stroke and carotid artery stenosis exceeding 70%

In asymptomatic good-risk patients treated by surgeons with surgical mortality and morbidity of less than 3%, the proven indication for CEA is stenosis exceeding 60%.[4]

Question 48

Drugs that may potentiate physiologic tremor

Answer 48

Beta-adrenergic agonists 
Terbutaline 
Metaproterernol 
Isoetharine 
Epinephrine

Psychiatric drugs 
Lithium 
Neuroleptics 
SSRI

Stimulants 
Amphetamine 
methylphenidate 
midodrine 
cocaine

Heavy metals 
Mercury 
Lead 
Arsenic 
Bismuth 
Methyl bromide

Anticonvulsants 
Valproate sodium

Methylxanthines 
Caffeine 
Theophylline

Question 49

Phenytoin - Dilantin (toxicity)

Answer 49

Phenytoin Toxicity:
-rarely fatal
-neurological symptoms:
-variable: nystagmus → ataxia → coma
-binds to voltage gated sodium channels → increases membrane threshold for depolarization
-excessive inhibition →
incoordination
altered LOC
arrythmia (SA and AV node blocks)
-propylene glycol in which it is storeed → hypotension, cardiac arrhythmias
-70% bioavailable after oral ingestion
-significant protein binding → may be displaced from plasma proteins by other drugs causing toxicity
-increased concentration: protiein binding becomes saturated therfore only a small incremental dose can cause dramatic rise in free phenytoin concentrations → toxicity.

-renal and hepatic clearance so renal failure could impact, but not renally dosed!

Question 50

Carotid Stenosis (primary prevention)

Answer 50

Aspirin for Stroke prevention

• Works if previous TIA, stroke, CAD but not without a prior hx of cerebrovascular or coronary disease, even in those with carotid bruits.
• The AHA/ASA 2011 recommend ASA 75-325mg for patients with obstructive atherosclerosis involving the carotid artery for prevention of ischemic cardiovascular events, acknowlding that “the benefit has not been established for prevention of stroke in asytmpomatic patients”

Question 51

ALS Treatment

Answer 51

Bipap but no prophylactic abx

Question 52

CN III Palsy (findings)

Answer 52

The primary symptom is diplopia from misalignment of the visual axes, and the pattern of image separation is the key to diagnosing which particular cranial nerve (and extraocular muscle) is involved. With unilateral third cranial nerve palsy, the involved eye usually is deviated down and out (infraducted, abducted), and there is ptosis, which may be severe enough to cover the pupil. In addition, pupillary dilatation can cause symptomatic glare in bright light (if the ptotic lid does not cover the pupil), and paralysis of accommodation causes blurred vision for near objects.

Question 53

CN III Palsy (Causes)

Answer 53

• PCA aneurysm
• Cavernous sinus lesion

Question 54

Cavernous Sinus Syndrome (tolosa-hunt)

Answer 54

More diffuse lesions within the cavernous sinus, often inflammatory in nature, typically give rise to simultaneous involvement of the third, fourth, sixth, and first 2 divisions of the fifth cranial nerves in various combinations, which serve to define a cavernous sinus syndrome. Nonspecific or granulomatous inflammation within the cavernous sinus is referred to as Tolosa-Hunt syndrome.

Question 55

Abscence seizures

Question 56

Peroneal nerve injury vs. S1

Answer 56

• Peroneal nerve damage - Examination typically reveals weakness in foot dorsiflexion and foot eversion (deep and superficial peroneal nerve-innervated, respectively), with normal inversion and plantar flexion (posterior tibial nerve). Sensory disturbance is confined to the dorsum of the foot. Reflexes are normal
• S1 radiculopathy – Pain radiates down the posterior aspect of the leg into the foot from the back. Weakness of plantar flexion (gastrocnemius muscle) is specific. Sensation is generally reduced on the posterior aspect of the leg and the lateral edge of the foot. Ankle reflex loss is typical.

Question 57

Sciatic Nerve Injury

Question 58

Wallenberg Syndrome

Question 59

Weber Syndrome

Answer 59

Ventral CN III fascicular syndrome (Weber’s Syndrome, lesion affecting cerebral peduncle)

• Contralateral hemiplegia including lower face d/t CST, CBT involvement
• Ipsilateral oculomotor paresis incl parasympathetic CN III (i.e. mydriasis)

Question 60

Posterior Communicating Artery Aneurysm

Posterior Cerebral Artery Aneurysm

Answer 60

If midbrain involved, (i.e. nuclear or fascicular third nerve lesion) almost always occurs with other neurologic signs or symptoms, which identifies the midbrain location of the lesion:

• Contralateral ataxia will be present if the red nucleus/superior cerebellar peduncle is involved
• Cerebellar tremor may be present in Claude syndrome (ipsilateral third nerve palsy and contralateral cerebellar signs)
• Contralateral hemiparesis may be present in a cerebral peduncle lesion (Weber syndrome)
• Contralateral choreiform movements or tremor are present when red nucleus/substantia nigra involvement occurs (Benedikt syndrome)

Question 61

Vertebrobasilar Stroke Types

Answer 61

Brainstem stroke syndromes refer to a group of syndromes that occur secondary to occlusion of small perforating arteries of the posterior circulation. The resulted infarction has characteristic clinical picture according to the involved area however, generally there is ipsilateral cranial nerve palsy and contralateral hemiplegia/hemiparesis and sensory loss.

Medulla oblongata

lateral medullary syndrome (Wallenberg syndrome)

medial medullary syndrome (Dejerine syndrome)

hemimedullary syndrome (Babinski-Nageotte syndrome)

Pons

lateral pontine syndrome (Marie-Foix syndrome)

inferior medial pontine syndrome (Foville syndrome)

locked-in syndrome

ventral pontine syndromes

ventral pontine syndrome (Raymond syndrome)

ventral pontine syndrome (Millard-Gubler syndrome)

facial colliculus syndrome

Midbrain

Weber syndrome

Benedikt syndrome

Claude syndrome

Question 62

How to grade power

Answer 62

• 5 — Muscle contracts normally against full resistance.
• 4 — Muscle strength is reduced, but muscle contraction can still move joint against resistance.
• 3 — Muscle strength is further reduced such that the joint can be moved only against gravity with the examiner’s resistance completely removed. As an example, the elbow can be moved from full extension to full flexion starting with the arm hanging down at the side.
• 2 — Muscle can move only if the resistance of gravity is removed. As an example, the elbow can be fully flexed only if the arm is maintained in a horizontal plane.
• 1 — Only a trace or flicker of movement is seen or felt in the muscle, or fasciculations are observed in the muscle.
• 0 — No movement is observed.

Question 63

Diabetic Amyotrophy

Answer 63

- Diabetic amyotrophy: L2-L4 and occasionally L5?

• Not a pure lumbosacral plexopathy because it also affects the lumbosacral nerve roots and peripheral nerves
• The most likely cause is ischemic injury from a nonsystemic microvasculitis
• Typically occurs in patients with type 2 diabetes mellitus that has been recently diagnosed (this can be the presenting feature) or has been under fairly good control.
• Traditional features – acute, asymmetric, focal onset of pain followed by weakness involving the proximal leg, with associated autonomic failure and weight loss.
• Progression occurs over months and is followed by partial to full recovery in most patients.
• Diabetic amyotrophy was the presenting feature of diabetes in 21 percent.
• Most patients in the series had proximal and distal sensory loss,

Diabetic lumbosacral plexopathy often occurs in conjunction with significant recent weight loss (commonly 10-40 pounds) and is associated with poor glycemic control.

Question 64

Subacute Combined Degeneration

Answer 64

• Subacute Combined Degeneration

Classic picture of subacute combined degeneration of the dorsal (posterior) and lateral spinal columns (specific for Cbl deficiency, is due to a defect in myelin formation of unknown mechanism. )

• The neuropathy is symmetrical and affects the legs more than the arms.
• It begins with paresthesias and ataxia associated with loss of vibration and position sense, and
• Can progress to severe weakness, spasticity, clonus, paraplegia, and even fecal and urinary incontinence
• Early in the course, poor joint position and vibration sense predominate. Typically, the legs are affected before the arms. Rarely are all limbs affected simultaneously. A Romberg sign is commonly found. The gait may be wide based.
• On presentation, 50% of patients have absent ankle reflexes with relative hyperreflexia at the knees. Plantars are initially flexor and later extensor. A Hoffman sign may be found.
• As the disease progresses, ascending loss of pinprick, light touch, and temperature sensation occurs. Later, depending on the predominance of posterior column versus cortical spinal tract involvement, ataxia or spastic paraplegia predominates. Then, PNS involvement causes distal limb atrophy.
• Cognitive testing may reveal mild impairment or frank dementia.

Question 65

Acoustic neuroma

Answer 65

Vertigo and disequilibrium are uncommon presenting symptoms among patients with acoustic tumors. Rotational vertigo (the illusion of movement or falling) is uncommon and is occasionally seen in patients with small tumors. Disequilibrium (a sense of unsteadiness or imbalance), on the other hand, appears to be more common in larger tumors. Overall, if carefully questioned, approximately 40-50% of patients with an acoustic neuroma report some balance disturbance. However, balance disturbance is the presenting symptom in less than 10% of patients.

Question 66

Subacute Labyrinthitis

Question 67

Burning Mouth Syndrome

Answer 67

Burning mouth syndrome — Burning mouth syndrome is characterized by an intraoral burning sensation for which no medical or dental cause can be found [2]. Prior to making the diagnosis, it is important to rule out oral mucosal diseases, such as herpes simplex and aphthous stomatitis.

A treatment algorithm for neuropathic pain has been proposed based upon a systematic review of randomized clinical trials in various central and peripheral neuropathic pain conditions [41]. The following recommendations were made for central neuropathic pain:

• Tricyclic antidepressants are first-line agents.
• Gabapentin/pregabalin are second choices. However, these agents are first choices in the elderly or when tricyclics are contraindicated.
• Opioids are third-line agents.

When medications of choice fail, a trial-and-error approach can be attempted with other drugs that have been used for neuropathic pain, including but not limited to, phenytoin, carbamazepine, clonazepam,valproate, phenothiazines, serotonin-norepinephrine reuptake inhibitors,mexiletine, and baclofen [6].

Question 68

Posterior circulation syndromes

Question 69

Dilantin Levels (correction)

Answer 69

Corrected Phenytoin = Measured Phenytoin Level / ( (0.2 x albumin) + 0.1)

Question 70

Autonomic Failure (neurologic causes)

Answer 70

Lewy body syndromes — The Lewy body syndromes are characterized by cytoplasmic neuronal inclusions (Lewy bodies) found in the brain and peripheral autonomic nerves of affected patients. There are three different but overlapping phenotypes:

• Pure autonomic failure (PAF) with idiopathic peripheral autonomic failure as the sole clinical finding.
• Parkinson disease (PD) with motor abnormalities and varying degrees of autonomic failure.
• Dementia with Lewy bodies (DLB), with severe cognitive impairment associated with parkinsonism and autonomic dysfunction.
• Some small number of patients with PAF may progress to develop PD or DLB [11], but PAF usually progresses gradually, responds well to therapy, and has a substantially better prognosis than PD or DLB [12].

Multiple system atrophy (MSA) — Another neurodegeneration with autonomic failure is MSA, also referred to as Shy-Drager syndrome, which affects the central nervous system but spares peripheral autonomic neurons. MSA has two phenotypes: parkinsonian and cerebellar, both with prominent autonomic failure.

Neuropathies — Autonomic failure can result from peripheral neuropathies as well as autoimmune and paraneoplastic neuropathy.

• Peripheral neuropathies include those caused by diabetes or amyloidosis that affect postganglionic autonomic nerves.
• Autoimmune autonomic neuropathy with ganglionic nicotinic acetylcholine receptor (nAChR) autoantibodies (autoimmune autonomic ganglionopathy) is usually associated with subacute (less than three months) clinical onset of dysautonomia. In addition to orthostatic hypotension, patients also have dry eyes and dry mouth, severe upper gastrointestinal dysautonomia, large pupils that react poorly to light and accommodation, and neurogenic bladder. There is usually no objective sensory loss or motor or reflex changes. Patients are young to middle aged adults and are more likely to be female than male (ratio 2:1).
• Paraneoplastic autonomic neuropathy occurs commonly in association with anti-Hu antibodies (also known as type 1 anti-neuronal nuclear antibody ANNA-1), most often in patients with small cell lung cancer, but it can be seen in other malignancies. Also associated with paraneoplastic autonomic neuropathy are Purkinje cell cytoplasmic antibodies type 2 (PCA-2) and antibodies to the neuron cytoplasmic protein, collapsin response-mediator protein-5 (CRMP-5). Bowel hypomotility, intestinal obstruction, bladder dysfunction, orthostatic hypotension, pupillomotor and sudomotor dysfunction and xerophthalmia are prominent clinical findings.

Question 71

Lateral Medullary infarction

Answer 71

Lateral Medullary Infarction (Wallenberg Syndrome)

• Most common syndrome related to IC vertebral artery occlusion.
• Vestibulo-cerebellar (nearly always present) - vestibular nuclei and connections, Cerebellar peduncle affected.

Vertiginous sensation (variable forms)

Difficulty sitting upright - often lean towards ipsilateral side.

Hypotonia in ipsilateral arm (a cerebellar sign)

Blurred vision/diplopia, oscillopsia. Nystagmus (both horizontal and rotatory usually)

Ocular torsion - ipsilateral eye tilted downward.

Limb ataxia (ipsilateral)

• Sensory Symptoms
  
  • Pain or unpleasant feeling in ipsilateral face (spinal nucleus of V)
  • Loss of P/T in contralateral trunk and limbs (spinothalamic tract)
  • The most common pattern of sensory abnormality with lateral medullary infarcts is loss of pain and temperature sensation in the ipsilateral face and the contralateral trunk and limbs. The next most frequent combination is hypalgesia in the ipsilateral face and contralateral face, trunk, and limbs.
• Bulbar Muscle Weakness
  
  • Very prominent feature when infarct extends medially (lower CNs)
  • Paralysis of ipsilateral palate, pharynx, larynx - hoarseness/dysphagia, apsiration
  • Ipsilateral Vocal cord paralysis, Uvula deviates to side contralateral to infarct.
• Odine’s Curse - Failure of autonomic respirations, esp during sleep.(Caudal lesions)
• Autonomic Dysfunction - Horner’s in ipsilateral eye, CV insatbility (tachycardia, orthostasis with inappropriate HR response, bradycardia)

Question 72

PICA infarct

Answer 72

POSTERIOR INFERIOR CEREBELLAR ARTERY – Cerebellar infarction in posterior inferior cerebellar artery (PICA) distribution can involve just the vermis, or the lateral surface, or the full PICA territory. Full PICA territory infarcts are often accompanied by edema formation and mass effect (so-called pseudotumoral cerebellar infarcts). The combination of lateral medullary and PICA cerebellar infarction occurs when the intracranial vertebral artery (ICVA) is occluded and blocks the orifice of both PICA and the lateral medullary penetrators. Sometimes medial PICA territory infarcts are accompanied by dorsal medullary infarcts since the medial PICA branch has some supply to the dorsal medulla [4,16,17].

Infarcts limited to the medial vermis in medial PICA territory usually cause a vertiginous labyrinthian syndrome that closely mimics a peripheral vestibulopathy. Severe vertigo with prominent nystagmus are the major findings. Some patients also have truncal lateropulsion characterized by feelings of magnetic pulling of the trunk to the ipsilateral side.

Lateral cerebellar hemisphere PICA territory infarcts are usually characterized by minor degrees of dizziness and gait incoordination with veering to the side of the lesion. Minor limb hypotonia and incoordination are found. A common syndrome is acute unsteadiness with ataxia but without vertigo or dysarthria. Body sway towards the side of the lesion, ipsilateral limb ataxia, and abnormal rapid alternating movements are also common.

When the full PICA cerebellar territory is involved, headache is usually present in the occiput or high neck on the ipsilateral side. The head may also be tilted with the occiput tending to tilt toward the ipsilateral side.

Vomiting, gait ataxia, truncal lateropulsion, and limb incoordination are other common findings. The truncal dysfunction is similar to that found in the lateral medullary syndrome; the body is often tilted or pulled ipsilaterally upon sitting or standing. The limb incoordination consists mostly of hypotonia rather than a rhythmic intention tremor.

Pseudotumoral cerebellar infarction — The syndrome of pseudotumoral cerebellar infarction, with edema formation and mass effect, is most often associated with large full posterior inferior cerebellar artery (PICA) territory infarcts. After the first day or so, patients with this form of cerebellar infarction typically develop increased headache, vomiting, and decreased consciousness, with drowsiness followed by stupor. Bilateral Babinski signs are an early sign of cerebellar mass effect.

Characteristic oculomotor abnormalities of large cerebellar space-taking infarcts can develop and include the following features:

• Most common are a conjugate gaze paresis to the side of the lesion or a paresis of abduction limited to the ipsilateral eye
• Bilateral sixth nerve paresis may occur
• Later bilateral horizontal gaze palsies may develop, often accompanied by ocular bobbing

These signs are due to compression of the pontine tegmentum by the swollen cerebellar infarct. Stupor is followed by deep coma when the oculomotor abnormalities become bilateral.

Question 73

Posterior Cerebral Arteries

Answer 73

POSTERIOR CEREBRAL ARTERIES — Most posterior cerebral artery (PCA) territory infarcts are due to embolism from the heart, aorta, or vertebral arteries. Atherosclerosis and dissection of the PCAs is not common.

The combination of hemisensory loss and hemianopia without paralysis is virtually diagnostic of infarction in the PCA territory. The occlusive lesion is within the PCA before the thalamogeniculate branches to the lateral thalamus. Rarely, occlusion of the proximal portion of the PCA causes a hemiplegia, which is probably due to infarction in the lateral midbrain [3,35-37]. Involvement of the corticospinal and/or corticobulbar tracts in the cerebral peduncles is thought to cause hemiplegia in these cases.

Left PCA territory symptoms and signs — When the left PCA territory is infarcted, alexia without agraphia, anomic aphasia or transcortical sensory aphasia, and Gerstmann syndrome (acalculia, agraphia, finger agnosia, and right-left disorientation) may be found. Defective acquisition of new memories is common when both medial temporal lobes are damaged but also occurs in lesions limited to the left temporal lobe. The memory deficit in patients with unilateral lesions is usually not permanent but may last up to six months. Patients cannot recall what has happened recently and, when given new information, they do not recall it moments later. They often repeat statements and questions spoken only minutes before. Some patients with left PCA territory infarction have difficulty in understanding the nature and use of objects presented visually (associative visual agnosia). They can trace with their fingers and copy objects, demonstrating that visual perception is preserved; they can name objects presented in their hand and explored by touch or when verbally described.

Right PCA territory symptoms and signs — Infarcts of the right PCA territory are often accompanied by prosopagnosia, which is difficulty in recognizing familiar faces. Disorientation to place and an inability to recall routes or to read or visualize the location of places on maps are also common. Patients with right occipitotemporal infarcts also may have difficulty visualizing what a given object or person look like. Dreams may be devoid of visual imagery. Visual neglect is much more common after lesions of the right than of the left PCA territory.

Question 74

Horner’s Syndrome

(Ddx)

Answer 74

Anatomic approach

Central

• Hypothalamus
• brainstem (Wallenberg’s) / spinal cord (syringomyelia/myelitis)

pre-ganglionic

• pulmonary: Pancoast tumor
• cervical rib, aneurysm or dissection of the aorta, lymph nodes, neuroblastoma
• Iatrogenic: CT placement, CVC

post-ganglionic

• Int. carotid dissection
• para trigeminal syndrome
• carotid cavernous fistula
• herpes zoster

NOTE: imp to rule out cervicothoracic dissection or acute cavernous sinus syndrome

Question 75

Tabes Dorsalis

Question 76

Hereditary Motor Sensory neuropathy (Charcot-Marie-Tooth)

Answer 76

Hereditary motor sensory neuropathy (HMSN), also known as Charcot-Marie-Tooth (CMT) disease, is really a spectrum of disorders caused by a specific mutation in one of several myelin genes that result in defects in myelin structure, maintenance, and formation. Hereditary motor sensory neuropathy (Charcot-Marie-Tooth disease) has been classified as CMT types 1 through 7 and is genetically heterogeneous with more than 40 genes/loci identified to date. The major division comprises type 1 and type 2, which together are the most common hereditary peripheral neuropathies [4], with an estimated prevalence of 40 per 100,000. Common features include both motor and sensory nerve manifestations with distal leg weakness, foot deformities (pes cavus, hammer toes), and sensory deficits.

• CMT-1: onset first and second decade. The most common. Early complaints may include frequent sprained ankles caused by distal muscle weakness or difficulty running and keeping up with peers. The only obvious physical findings may be loss of reflexes, pes cavus foot deformity, and hammer toes.
• Distal calf muscle atrophy often occurs, causing the classic “stork leg deformity.” Walking is clumsy because of both muscle weakness and sensory loss. Sensory loss is gradual and mainly involves proprioception and vibration.
• Later changes include atrophy of the intrinsic hand and foot muscles. Palpable enlargement of the peripheral nerves may occur secondary to nerve hypertrophy. In addition, kyphosis or scoliosis often develops.
• Ambulation is variable but usually is maintained through life. Life expectancy is not affected.
• Disease exacerbation can occur in pregnancy, an effect that may be mediated by increased plasma progesterone.
• In addition to the typical findings discussed above, patients with type 1A HMSN may have associated sleep apnea.

Nerve conduction studies (NCS) - severe slowing of conduction velocity in both the motor and sensory nerves to less than 60 percent of normal, degree often does not correlated with physical findings.

Sural nerve biopsy shows demyelination that affects primarily the large nerve fibers. Onion bulbs are a characteristic feature; they reflect repeated demyelination and remyelination and represent redundant Schwann cells, collagen, and fibroblasts. Secondary axonal changes are present.

The classic features of CMT2 include distal weakness, atrophy, sensory loss, decreased deep tendon reflexes and variable foot deformity [37]. The onset of symptoms usually is in the second or third decade of life. The clinical course is similar to that of CMT1, but sensory symptoms, with loss of vibration and proprioception, typically predominate over motor symptoms, and peripheral nerves are not palpably enlarged. Distal trophic ulcerations in the feet may occur.

Management of CMT is supportive. Specific disease-modifying therapy is not available. Daily stretching exercises early in the course of the disease may help delay ankle contractures. Orthotics are often used to help stabilize the ankles. Orthopedic foot surgery often is required by the time the patient reaches adolescence to treat the pes cavus deformity and hammer toes.

Question 77

Dopamine Agonist (PD)

Answer 77

DOPAMINE AGONISTS — The dopamine agonists (DAs) are a group of synthetic agents that directly stimulate dopamine receptors. The drugs currently approved by the United States Food and Drug Administration (FDA) include bromocriptine, pramipexole, ropinirole, rotigotine, and injectable apomorphine (table 1). Pergolide has been voluntarily withdrawn from the United States market and is best avoided because it is associated with a risk of cardiac valve problems.

Apomorphine and lisuride are additional DAs that can be administered parenterally for “rescue therapy” in patients experiencing sudden akinetic episodes. Lisuride is not currently approved in the United States, but is available in Europe. Injectable apomorphine has been approved by the United States FDA for treatment of motor fluctuations in PD [50]. Apomorphine infusion pumps may also be useful, but are not available in the United States.

Unlike carbidopa-levodopa (Sinemet), these drugs are direct agonists that do not require metabolic conversion, do not compete with amino acids for transport across the gut or into the brain, and do not depend upon neuronal uptake and release. An additional advantage over immediate-release forms of levodopa is the longer duration of action of most of these agents.

Monotherapy — Dopamine agonists (DAs) were initially introduced as adjunctive treatment for advanced PD complicated by reduced levodopa response, motor fluctuations, dyskinesia, and other adverse effects of levodopa. However, the hypothetical concern that free radicals generated by the oxidative metabolism of dopamine contribute further to the degeneration of dopaminergic neurons has prompted some investigators, despite lack of conclusive evidence, to advocate the early use of DAs as an levodopa-sparing strategy [51,52].

With this approach, treatment with levodopa can be postponed and saved for a later time in the course of the disease, when disability worsens and the less effective agonists no longer provide adequate benefit. This strategy is based upon the unproven concept that the long-term duration of a given patient’s responsiveness to levodopa is finite and that the drug, like money in a savings or retirement account, should be rationed. However, whether reduced responsiveness to levodopa over time is due to a decline in drug response or progression of underlying PD is currently uncertain.

Given the potential that DAs are associated with fewer motor fluctuations and the evidence that there is a higher incidence of levodopa-related dyskinesia in young-onset PD, some experts suggest using DAs as initial treatment for PD in patients younger than age 60, and using the more effective agent levodopa in patients 60 and older [53], although other factors should be weighed in making this treatment decision.

Effectiveness of DAs — Controlled trials have shown that bromocriptine, pergolide, pramipexole, and ropinirole are all effective in patients with advanced PD complicated by motor fluctuations and dyskinesia. Other studies have found that pramipexole, ropinirole, transdermal rotigotine, and pergolide are effective as monotherapy in patients with early disease. However, DAs are ineffective in patients who have shown no therapeutic response to levodopa.

The decision to initiate symptomatic medical therapy in patients with PD is determined by the degree to which the patient is functionally impaired. The timing of this decision varies greatly among patients but is influenced by a number of factors, including [1]:

• The effect of disease on the dominant hand
• The degree to which the disease interferes with work, activities of daily living, or social and leisure function
• The presence of significant bradykinesia or gait disturbance
• Personal philosophy regarding the use of drugs

The major drugs available for symptomatic therapy include:

• Levodopa
• MAO B inhibitors
• Dopamine agonists
• COMT inhibitors
• Anticholinergic agents
• Amantadine

Either levodopa or a dopamine agonist (DA) can be used initially for patients who require symptomatic therapy [2-4]. Practitioners should always try to find the lowest but still effective dose of dopaminergic medication, either singly or in combination, for patients with PD, each of whom must be evaluated and managed in a highly individual way.

In addition to these agents, low-dose estrogen may be helpful as adjunctive therapy in postmenopausal women.

Most patients with PD can go without antiparkinson medications when oral intake is temporarily restricted (eg, when perioperative, or when NPO for testing), or when seriously ill. In patients who are critically ill and bedbound, the parkinsonian symptoms are typically overshadowed by the burden of other medical problems, and antiparkinson medications may not provide any clear benefit.

There have been reports of patients with PD who developed neuroleptic malignant syndrome in the context of sudden withdrawal of levodopa or dopamine agonists. However, the occurrence of neuroleptic malignant syndrome in this situation is highly unusual and is typically of more theoretical than real concern. Brief withdrawals (ie, for <24 hours) of these medications under careful supervision during hospitalization should not be an issue.

When treatment is still desired for patients who are NPO, options include transdermal rotigotine and apomorphine by injection or continuous infusion. The use of apomorphine requires a test dose prior to ongoing treatment. Transdermal rotigotine is available in Europe, but in the United States the drug has been recalled and future availability is uncertain.

Levodopa (L-dopa) is well established as the most effective drug for the symptomatic treatment of idiopathic or Lewy body PD. It is particularly effective for the management of akinetic symptoms and should be introduced when these become disabling and are uncontrolled by other antiparkinsonian drugs. Tremor and rigidity can also respond to levodopa therapy, but postural instability is less likely to do so.

Adverse Effects: Nausea, somnolence, dizziness, and headache are among the more common early side effects that may accompany treatment with levodopa, but they are not likely to be serious in most patients. More serious adverse reactions to levodopa (mainly in older patients) may include confusion, hallucinations, delusions, agitation, and psychosis. A substantial number of patients develop levodopa-induced complications within several years of starting levodopa. These include motor fluctuations (the wearing-off phenomenon), involuntary movements known as dyskinesia, abnormal cramps and postures of the extremities and trunk known as dystonia, and a variety of complex fluctuations in motor function. It is estimated that such motor complications occur in at least 50 percent of patients after 5 to 10 years of treatment

Question 78

MAO B Inhibitors

Answer 78

MAO B INHIBITORS — Selegiline (Eldepryl), a selective monoamine oxidase (MAO) type B inhibitor (table 1) [38], is modestly effective as symptomatic treatment for PD [39] and may have neuroprotective properties. In many individuals, however, selegiline monotherapy does not produce a functionally significant benefit. However, the use of selegiline in early PD is a reasonable option as long as the patient understands its limitations.

In terms of MAOB inhibitors, Selegiline has mild symptomatic benefit, and it may be used in patients with early PD. Its use should be limited to patients with early disease since the symptomatic benefits are unlikely to be significant in those with more advanced PD.

Question 79

Progressive Multifocal Leukoencephalopathy

Answer 79

<tt>Progressive Multifocal leukoencephalopathy: </tt>

Progressive multifocal leukoencephalopathy

• JC Virus
• Lesions usually in white matter: cortical symptoms.
• Asymmetric, focal neurologic symptoms
• Motor deficits (hemiparesis or monoparesis), limb ataxia, gait ataxia, and visual symptoms such as hemianopia and diplopia.

Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system that is caused by reactivation of the polyomavirus JC (JC virus). Asymptomatic primary infection with JC virus occurs in childhood and antibodies can be found in 86 percent of adults [4]. In most individuals, JC virus remains latent in kidneys and lymphoid organs, but, in the context of profound cellular immunosuppression, JC virus can reactivate, spread to the brain, and induce a lytic infection of oligodendrocytes, which are the CNS myelin-producing cells.

Progressive multifocal leukoencephalopathy occurs almost exclusively in immunosuppressed individuals. There are only isolated case reports of PML in patients without apparent immunosuppression. There are, however, reports of PML affecting patients who have conditions associated with minimal or occult immunosuppression, such as hepatic cirrhosis and renal failure.

PML was initially described in patients with lymphoproliferative and myeloproliferative diseases such as chronic lymphocytic leukemia, chronic myeloid leukemia, and Hodgkin lymphoma [11-16]. It was subsequently reported in rare patients with solid organ malignancies, granulomatous and inflammatory diseases, solid organ transplant recipients, and with the use of certain drugs. Prior to the widespread use of highly active antiretroviral therapy (HAART) in the United States and Europe, PML was recognized as a major opportunistic infection associated with AIDS in adults, with a prevalence of 1 to 5 percent. PML has also been reported in patients treated with belatacept [32,33], efalizumab [34,35], fludarabine [12-16], infliximab [36], rituximab [37-39], mycophenolate [40-42], and glucocorticoids.

PML usually manifests with subacute neurologic deficits including altered mental status, motor deficits (hemiparesis or monoparesis), limb ataxia, gait ataxia, and visual symptoms such as hemianopia and diplopia. Most patients with HIV infection and PML are profoundly immunosuppressed with CD4-positive T-cell counts <200 per mm³. Initial symptoms may vary greatly from patient to patient, depending on the location of their lesions in the central nervous system (CNS) white matter. PML typically spares the optic nerves and the spinal cord, although incidental finding of PML lesions in the spinal cord has been reported in the post-mortem examination of one patient with HIV infection who died from hemispheric PML [46].

Although PML lesions are located mainly in the white matter, PML symptoms are frequently indicative of a cortical disorder. These cortical symptoms have been attributed to white matter lesions that undercut relevant cortical areas. As examples, aphasia may result from lesions underlying the language areas in the left frontal and temporal lobes, and lesions in the occipital lobe white matter can mimic symptoms of cortical blindness. However, neuropathologic study has revealed that cortical demyelination is a frequent finding with PML, suggesting that cortical symptoms in PML may arise from cortical demyelinating lesions.

Seizures are usually thought to be a manifestation of cortical injury rather than white matter disease. Nonetheless, seizures occur in up to 18 percent of patients with PML, mainly when PML lesions are located immediately adjacent to the cortex [47].

The disease course of PML is usually progressive and fatal. The median survival of patients without HIV infection is only a few months. Survival in the era of highly active antiretroviral therapy is longer, but survivors are often left with severe neurologic sequelae.

Question 80

Progressive Supranuclear Palsy

Answer 80

The diagnosis of PSP is clinical. Key features typically develop over time. Participants in a National Institute of Neurological Disorders and Stroke (NINDS)/Society for PSP conference have formulated and validated clinical research criteria for the diagnosis of PSP.[1] In this system, criteria for possible PSP are as follows:

Gradually progressive disorder with onset at age 40 years or older

Either vertical supranuclear palsy or both slowing of vertical saccades and prominent postural instability with falls in the first year of onset

No evidence of other diseases that can explain the clinical features

Question 81

Optic neuritis

Answer 81

Vision loss and eye pain

Afferent pupillary defect

Central scotoma

Papillitis with hyperemia and swelling of disk, blurring of disk margin, loss of colour vision

Question 82

Papilledema

Answer 82

While rarely the presenting symptom, visual symptoms are common in patients with papilledema. These are typically visual obscurations that clear completely, are often unilateral, and typically very brief (just seconds) They may occur spontaneously or with changes in position, and they are believed to represent transient fluctuations in nerve head perfusion. Their presence correlates with the degree of intracranial pressure elevation. Increasing intensity, frequency, and duration of these symptoms can be a prognostic sign for visual loss.

In papilledema, it is unusual for patients to have deficits of visual acuity or field loss until quite late in the course. Untreated, chronic papilledema can lead to progressive visual field loss in the form of peripheral field contraction, nerve fiber bundle defects, and even blindness

Question 83

DDx of Ptosis

Answer 83

PTOSIS

Congenital Ptosis (levator muscle problem)

Amblyopia, strabismus also common

Aponeurotic ptosis

Horner Syndrome

Ipsilateral Miotic Pupil

Myasthemia

Variable, Fatigable. Diplopia/EOM abn often +.

Muscle disease

May also affect Orbicularis/Extraocular/Bulbar muscle

CN 3 Palsy (many causes)

Nucleus

Ipsi complete 3^rd, Ctrl Ptosis + SR weakness

Infarct, Hemorrhage, Tumor

Fascicles

Ipsi ataxia; Ctrl tremor or hemiparesis (i.e. midbrain syndromes)

Infarct, Hemorrhage, Tumor, Demyelination

Subarachnoid space

Typically Isolated Ptosis. +/- headache, orbital pain

ICA/PCom/Basilar/PCA Aneurysm, Microvascular (Ischemic), Tumor (Pit), Meningitis, Herniation, Trauma

Cavernous Sinus

Horner. Prominent Pain

Tumor, Inflmmation, Carotid aneurysm, Ischemic, Thrombotic, AV fistula

Orbital Apex

Proptosis, Visual loss, CN IV, VI, V1, V@ also affected, Horner, Pain

Tumor, Trauma, Inflammation, Infection (Fungal)

Question 84

Carpal Tunnel syndrome / CTS

Answer 84

• Earlier symptoms of CTS are pain and numbness with repetitive wrist motion.
• Numbness along the Palmer aspect of the first to fourth digits; weakness comes later
• Phalen’s and Tinels have Se50% Sp75%

Diagnostic Criteria for Carpel Tunnel:

• One or more of the following symptoms affecting at least part of the median nerve distribution of the hand: paresthesias; hypesthesia; pain; or numbness
• One or more of the following objective findings: physical findings of median nerve compression including a positive Hoffman-Tinel sign or a positive Phalen test; diminished or absent sensation to pin prick in the median nerve distribution; or electrodiagnostic findings indicative of median nerve dysfunction across the carpal tunnel.

Question 85

Bell’s Palsy

Answer 85

Given the available trial data, we recommend early treatment with oral glucocorticoids for all patients with Bell’s palsy. Treatment should preferably begin within three days of symptom onset. Our suggested regimen is prednisone (60 to 80 mg/day) for one week.

For patients with severe facial palsy, we suggest early combined therapy with prednisone (60 to 80 mg per day) plus valacyclovir (1000 mg three times daily) for one week.

Question 86

Spinal Stenosis

Answer 86

Nerve root entrapment in lumbar spinal stenosis is caused by narrowing of the spinal canal (congenital or acquired), nerve root canals, or intervertebral foramina. This narrowing is usually caused by bony hypertrophic changes in the facet joints and by thickening of the ligamentum flavum. Disc bulging and spondylolisthesis may contribute.

Symptoms of significant lumbar spinal stenosis include back pain, transient tingling in the legs, and ambulation-induced pain localized to the calf and distal lower extremity, resolving with rest.

Question 87

Brain Death

Criteria

Answer 87

• There are four prerequisites before one can begin considering a patient “brain dead”

1. the cause of brain death / coma should be known.
2. Exclusion of complicating medical conditions or drug intoxication or poisoning, which may confound the clinical assessment
3. Core temperature 36ºC (90ºF). There is little evidence base for a choice of threshold temperature. Because of this, Canadian forum recommendations published in 2006 substituted 34ºC as a standard
4. Abnormal Neurologic exam (any findings)
   * Examination must demonstrate absent cerebral or brainstem function with all of the following findings:
   - Coma
   - Absent motor response
   - Absent pupillary light reflex; pupils are midposition or dilated (4 to 9 mm)
   - Absent corneal reflexes
   - Absent oculocephalic reflexes (Doll’s eyes)
   - Absent oculovestibular reflexes (caloric responses)
   - Absent gag reflex
   - Absent cough with tracheal suctioning
   - Absent sucking or rooting reflexes
   - Apnea as demonstrated by apnea test, described below
   * Following above, can do apnea test (spontaneous breathing trial)
   - Pre-oxygenate with 100% O2 for 10min
   - Disconnect from vent
   - Observe for resp efforts x 8-10min
   - Do ABG and reconnect to vent
   - Need PaCO2 to be > 60 and > 20 from baseline

Uniform Determination of Death Act, which has been adopted by most state laws in the United States.

Question 88

Parkinson’s (Treatment)

Answer 88

1. Carbidopa levodopa; SEs (delusion, hallucinations, dyskinesia);
2. DA: are all effective in patients with advanced PD complicated by motor fluctuations and dyskinesia; can be used in monotherapy (only if not refractory to levodopa)
3. MAOI: Selegiline; modest effective and does not significantly improve fxn.

Question 89

Normal Neurologic Changes with Aging

Question 90

Retinal Ischemia Findings

Question 91

Internuclear Ophalmoplegia

Answer 91

Bilateral adduction difficulties = INO

Internuclear ophthalmoplegia (INO) is a specific gaze abnormality, characterized by impaired horizontal eye movement with weak adduction of the affected eye and abduction nystagmus of the contralateral eye. In typical cases, convergence is spared. Abnormalities of vertical gaze are common but are not required features of an INO

Question 92

Causes of sensory neuropathy

Answer 92

paraneoplastic syndrome

Question 93

Amantadine

Answer 93

is a relatively weak antiparkinsonian drug with low toxicity that is most useful in treating patients with early or mild PD and perhaps later when dyskinesia becomes problematic.

Question 94

Charcot Marie Tooth

Question 95

Etiology of Seizures

Answer 95

REfeeding Syndrome (low phosphate)

Question 96

Falx Meningioma

Question 97

Vegetative states

What are they?

Answer 97

• Vegetative state—complete unawareness of the self and environment despite preservation of some brain functions (often cardiovascular and autonomic control).
  
  • Persistent vegetative state >1 month after initial injury.
  • Permanent vegetative state >6 months of unresponsiveness.
• Minimally Concious state - In contrast to PVS, they may intermittently demonstrate limited interaction with the environment by visually tracking, following simple commands, signaling yes or no (not necessarily accurately), or having intelligible verbalization or restricted purposeful behavior.
• Brain Death

Question 98

NIHSS

Answer 98

The NIHSS is a 15-item impairment scale, intended to evaluate neurologic outcome and degree of recovery for patients with stroke. The scale assesses level of consciousness, extraocular movements, visual fields, facial muscle function, extremity strength, sensory function, coordination (ataxia), language (aphasia), speech (dysarthria), and hemi-inattention (neglect)

Question 99

Indications for Thrombolysis in Acute Stroke

Question 100

Contraindications to Thrombolysis in Acute Stroke

Question 101

TIA of PCA

Answer 101

PCA

• Most due to embolism from the heart, aorta, or vertebral arteries
• Most frequent complaint is hemianopia. Superior quadrant field defect - just the lower bank of the calcarine fissure (the lingual gyrus). Inferior quadrantanopia if lesion affects the cuneus on the upper bank of the calcarine fissure.
• Lateral thalamic infarction is the major reason for somatosensory symptoms and signs
• The combination of hemisensory loss and hemianopia without paralysis is virtually diagnostic of infarction in the PCA territory.

Question 102

TIA of Int carotids

Answer 102

Retinal Ischemia more commonly associated with embolism from ipsilateral carotid rather than heart disease (in contrast to TIAs involving cerebral hemisphere). Only about 23.8% experience the typical altitudinal visual loss, but when present, highly suggestive of retinal embolism. Episodes generally last from seconds to minutes, rarely more than 15 minutes

When contralateral hemispheric symptoms (motor, sensory, aphasia) accompany visual loss, carotid disease is very likely; however, TMVL is more often an isolated symptom

Question 103

Stroke guidelines (antiplatelets)

Answer 103

Question 104

Mini Mental Status Exam MMSE

Answer 104

Domains of MMSE

Question 105

Lacunar syndromes

Answer 105

Question 106

Wernicke’s Encephalopathy

Answer 106

a. The classic triad of Wernicke’s encephalopathy (WE) includes:
1. gait ataxia 2. encephalopathy
3. occulomotor dysfunction (nystagmus and/or palsy).

WE is diagnosed in patients with two of the following four Caine criteria:

• Dietary deficiency
• Oculomotor abnormalities
• Cerebellar dysfunction
• Either altered mental status or mild memory impairment

Rx: Recommended regimen 500 mg of thiamine IV over 30 minutes, TID x 2 days then 500 mg IV/IM daily x 5 additional days in combination with other B vitamins

Expect ocular palsies to improve within hours-days; Gait ataxia – 2 weeks; confusion – days to weeks

Question 107

Stroke guidelines (anticoagulation)

Answer 107

The available evidence suggests that early anticoagulation with heparin or low molecular weight heparin is associated with a higher mortality and worse outcomes compared with aspirin treatment initiated within 48 hours of ischemic stroke onset.

Although full anticoagulant therapy was associated with about nine fewer recurrent ischemic strokes per 1000 patients treated, it was also associated with a nine per 1000 increase in symptomatic intracranial hemorrhage. Similarly, anticoagulants avoided about four pulmonary emboli per 1000, but this benefit was offset by an extra nine major extracranial hemorrhages per 1000

Question 108

TIA of Temporal Lobe

Answer 108

features that are relatively specific for TLE, including a rising epigastric sensation (often likened to a “roller coaster” sensation), and psychic or experiential phenomena, such as deja vu, jamais vu, or fear. taste and smell are less common but are also relatively specific for TLE. Unilateral automatisms are usually ipsilateral to the seizure focus, while dystonic posturing almost invariably occurs on the contralateral side. Head deviation at seizure onset is usually ipsilateral to the seizure; when it occurs later, it is contralateral

Question 109

Radiculopathy (Compressive)

Answer 109

• L4-L5 and L5-S1 - about 90 to 95 percent of compressive radiculopathies (evenly split)
• Next in frequency is L4. Other levels are uncommon. (And also unlikely to cause bilateral findings?)
• Far lateral herniations are seen more often at the L2-4 levels. They may affect the rostral root. (rare, as only 10 percent of far lateral herniations will result in nerve root compression)

Question 110

Plexopathy

Question 111

Diabetes Mellitus (Neurologic Complications)

Answer 111

• Symmetric Polyneuropahty, Autonomic Neuropathy, Polyradiculopathies (of which amyotrophy is one), mononeuropathies (CN, Peripheral nerve, MN)

Question 112

Disorders of Consciousness

Types?

Answer 112

• Neither (mimics) - can experience PAIN!
  
  • Locked-in: pons injury from basilar artery infarct; only blinks + vert eye movmt
  • Akinetic mutism: prefrontal/premotor injury; tracks only
• Arousal: located in the ARAS (pons, midbrain); 4 subtypes
  
  • confusion/delirium: “grumpy”
  • drowsiness: “dopey” - can wake up
  • stupor: “sleepy” - wakes up only with noxious stimuli
  • coma: “comatosey” - can’t wake up
• Awareness: Cerebrum with multiple inputs
  
  • Abulia: no will - frontal/prefrontal/basal ganglia, ACA, psych
  • catatonia: no movm’t - NMS
  • conversion disorder: no dz
  • dissociative state: no one (multiple personality)

Question 113

Approach to Decr’ LOC

Question 114

Decorticate vs Derebrate postures

Answer 114

Lesions in the bilateral midbrain or pontine areas result in a decerebrate posturing, characterized by opisthotonus and extension with internal rotation of all four extremities. A hemispheric disease or upper midbrain lesions (classically above the red nucleus) can result in a decorticate posturing, characterized by extension with internal rotation of the lower extremities and flexion with adduction of the upper extremities.

Question 115

Herpes Simplex Virus Infection HSV (Neurologic Sequalea)

Question 116

Pseudobulbar Palsy

Answer 116

Pseudobulbar palsy results from an upper motor neuron lesion to the corticobulbar pathways in the pyramidal tract. Patients have difficulty chewing, swallowing and demonstrate slurred speech (often initial presentation). Individuals with pseudobulbar palsy also demonstrate inappropriate emotional outbursts.

Causes

• Vascular causes: Bilateral hemisphere infarction, CADASIL syndrome
• Progressive Supranuclear Palsy
• Amyotrophic lateral sclerosis (ALS)
• Parkinson’s disease
• Multiple System Atrophy (related to Parkinson’s disease)
• Degenerative disorders: motor neuron disease
• Inflammatory disorders: Multiple sclerosis
• Malignancy: High brain stem tumors
• Metabolic causes: osmotic demyelination syndrome^[1]
• Brain trauma

Symptoms

These include:

• Dysphagia (difficulty in swallowing)
• Labile affect^[2]
• Dysarthria

Signs

These include:

• Speech is slow, thick and indistinct
• Gag reflex is normal, exaggerated or absent
• Tongue is small, stiff and spastic
• Jaw jerk is brisk
• There may be upper motor neuron lesion of the limbs.

Bulbar palsy is a similar disorder but is caused by lower motor neuron lesions

Question 117

Unprovoked Seizures (Approach)

Answer 117

1. Epileptic Seizures:
   -Congenital - brain malformations, inborn errors of metabolism
   -Drugs - withdrawal, overdose, illicit
   -Infections - menigintis, febrile siezures.
   -Structural - tumour, infarct, stroke, cerebral degeneration, truama
2. Physiologic Non-epileptic:
   -Metabolic - electrolyte, hypo/hyperglycemia (non-ketotic), hypoxia,
   hyperthryoid
   -Other - arrhythmia, acute intermittent porphyria
3. Psychogenic Non-epileptic - major emotional trauma, stressful psychological conflicts.
4. Seizure Mimics - syncope/TIA/migraine/BPV/hypoglycemia/sleepdisorders/periodic
   paralysis/ breath holding spell.

Types - 1) Simple (conscious), 2) Complex (reduced LOC), 3) partical
(part cortex), 4, generalized - bilateral cortex, unconscious

Epilepsy >1 unprovoked seizure
Status epilepticus - >30 min generalized seizure and warrants admission.

First seizure: History, Seizure preceiptiatnts (strong emotions,
intesnse exercise, loud music, flasshing lights, fever, menstrual
period, lack of sleep, stress) auras (simple partial complex) ,
history seizures, post-ictal sate.

Then labs: Screning to rule out metabolic, toxic.
Then LP: essential if history suggestive of infection or cancer that
might met to mengines - rule out space occupying lesion with CT!

EEG - essential to diagnosis. if abnromal, routin interictal EEG may
aid in supporting diagnosis. suggest general vs partial.
-abnormal in 23% presenting with first sieuzre —> increases
likelihoood of secont seizure in 2 years.
-can also use sleep depreivation and provokative meausres to increase yield.
-IF NORMAL DOES NOT RULE OUT EPILEPSY.
-many are non-specifically abnormal.

Then neuro imaging.

When to Treat?
-risk of reucrrent seizures is high.
-Risk with single unprovoked seizure - 14% at oneyear, 29% at 3 years,
34% t 5 years. 2 year recurrecen is 40-50%.
3 RF for recurrence: 1) Epileptiform activity on EEG, 2) remote
symptomatic cause - history or neuroimaging, 3) abnormal neuro exam.
-consider AED if any high RF after first seizures, otherwise,
generally only start after second seizure —> 75% risk of recurrence.
-OTHER RFS for reucrrence: status epilepticus, multi seizures in one
day, prior history of febrile seizures, family history, occurs during
sleep.
-no difference between various AED.
-beware of TENS/SJS with arbemazepine, valproate, phenytoin.

Question 118

Headaches

How many types can you describe?

Answer 118

TMC’s: tension migraine cluster and chronic

• Tension-type; most common type; Bilateral, dull and viselike/band; non-pharm tx
• Migraine headache, 2nd most common, worse with exertion, has four phases:

The prodrome—premonitory phenomena occurring hours to days prior to headache onset

The aura—focal neurologic symptoms (visual), usually <60 minutes.

The headache—unilateral, throbbing, mod-severe intensity, and aggravated by exertion. Other symptoms: nausea, vomiting, sensory excitability, systemic symptoms (i.e., anorexia, diarrhea, abdominal cramps, diaphoresis, lightheadedness, irritability). Hemicranial.

The postdrome—feeling tired, washed out, or depressed.

• Chronic daily headache—any type of headache that occurs ≥15 days per month.

Medication overuse headaches, when related to overuse of medications (e.g., nonsteroidal anti-inflammatory drugs). Tx is to limit drugs to <=2 days/wk

• Cluster headache—≥5 attacks/d of severe, stabbing, unilateral, periorbital, and/or temporal pain lasting 15-180 minutes assoc ipsilateral lacrimation, conjunctival injection, rhinorrhea, nasal congestion, forehead and facial sweating, miosis, ptosis, or eyelid edema. Tx is 100% O2; Li or verapamil to abort.
• Thunderclap headache—maximal at onset “explosive”. Global
• Hemicrania continua—continuous, daily unilateral headache, associated with autonomic features (miosis, ptosis, lacrimation, rhinorrhea). Responds to Indomethacin.
• Trigeminal Neuralgia, brief paroxysms, shock-like w/ facial triggers
• GCA, temporal, tender scalp, jaw claudications and visual changes; PMR

Question 119

Migraine without Aura

Criteria?

Answer 119

Remember 1- 2-3-4-5!! one of the phobias, 2/4, up to 3 days, at least 5 attacks

five attacks are needed, each lasting 4 to 72 hours and having two of the following four characteristics: unilateral location, pulsating quality, moderate to severe intensity, and aggravation by routine physical activity. In addition, the attacks must have at least one of the following: nausea (and/or vomiting), photophobia, or phonophobia.

Question 120

Migraine therapy (abortive)

Answer 120

For initial treatment of patients who present to the hospital emergency department with severe migraine, particularly if the migraine is accompanied by severe nausea or vomiting, we suggest treatment with intravenous (IV) metoclopramide or prochlorperazine

• Triptans (Serotonin 1b/1d agonists, inhibit the release of vasoactive peptides, promote vasoconstriction, and block pain pathways in the brainstem); Triptan for outpatients with moderate to severe migraine. There are no efficacy data that definitively support use of one triptan versus another. Based on limited evidence, it is still recommended that ?? Based on limited evidence, it is still recommended that triptans be avoided in patients with familial hemiplegic migraine, basilar migraine, ischemic stroke, ischemic heart disease, Prinzmetal’s angina, uncontrolled hypertension, and pregnancy. Also recommend giving first dose under observation if over age 40 and risk factor for CHD even if no known CHD.
• Ergotamine: drug of choice in few patients with migraine because of issues of efficacy and side effects. Ergots should be avoided in patients with coronary artery disease because they cause sustained coronary artery constriction, peripheral vascular disease, hypertension, and hepatic or renal disease.
• Analgesics, antiemetics, anxiolytics

In a small randomized trial, prochlorperazine was shown to be as effective as subcutaneous sumatriptan

Cleveland clinic Review

Question 121

Migraine therapy (Chronic)

Answer 121

• How to select patients for prophylaxis?
• Preventive medications for migraines include antidepressants, anticonvulsants, beta-blockers, and calcium channel blockers.
• Level A evidence is available for antiepileptic drugs (AEDs; divalproex sodium, sodium valproate, topiramate), beta blockers (metoprolol, propranolol, timolol), and triptans (frovatriptan for short-term menstrual-associated migraines).
• Among the beta-blockers, propranolol appears to be the most effective, probably because it penetrates the blood–brain barrier the best, but is no longer the most recommended migraine preventive medication (unless >4 /month).
• Among the AEDs, valproic acid is probably the most effective, but the side effects of hair loss and lack of safety in pregnancy make it difficult to use in the patient population that is more likely to have migraines—young women. Gabapentin is another useful anticonvulsant that is not metabolized by the liver and has a minimal side effect profile. Among the tricyclic antidepressants, amitriptyline is widely used, with a good level of supportive evidence, but it also has the most side effects: dry mouth, constipation, blurred vision, and some degree of confusion and drowsiness.
• Onabotulinumtoxin A, the first agent to receive U.S. Food and Drug Administration approval for chronic migraine, is likely safe and well tolerated.

Question 122

Headaches (ED approach)

Answer 122

LP!!!

Question 123

Polyneuropathy (Caused by drugs)

Answer 123

Colchicine:

• Neurological examination shows predominantly proximal muscle weakness (more in LE than UE), which is accompanied by distal areflexia and minor distal sensory loss.
• CK levels are almost always elevated, usually 10 to 20-fold above normal.
• Electromyography shows myopathic changes, while electron microscopy of muscle biopsy samples shows vacuolar changes and an accumulation of lysosomes and autophagic vacuoles.
• Myopathy generally disappears a few weeks after the cessation of medication, but neuropathy may persist for a long time.

Question 124

Stroke (clinical classification)

Answer 124

ANTERIOR CEREBRAL ARTERY (embolic >thrombotic):

• motor and sensory deficit (leg >face, arm)
• frontal release signs (grasp, snout, root, and suckling reflexes)
• abulia, paratonic rigidity, gait apraxia, personality D

MIDDLE CEREBRAL ARTERY (left dominant hemi- spheric, embolic >thrombotic):

• aphasia, right hemiparesis, and sensory deficit (face, arm >leg)
• may be complete hemiplegia if internal capsule involved
• right spatial neglect, right homonymous hemianopia, impaired right conjugate gaze

MIDDLE CEREBRAL ARTERY (right non-dominant hemi- spheric, embolic >thrombotic):

• anosognosia, left motor and sensory deficit (face, arm >leg)
• left spatial neglect, left homonymous hemianopia, impaired left conjugate gaze

DEEP (SUBCORTICAL/LACUNAR) HEMISPHERE OR BRAIN STEM (small artery infarct):

• hemiparesis (pure motor stroke); sensory loss (pure sensory stroke)
• dysarthria and clumsy hand; ataxic, hemiparesis
• No abnormalities of cognition, lan- guage, or vision

POSTERIOR CEREBRAL ARTERY (embolic >thrombotic):

• homonymous hemianopia with macular sparing
• alexia without agraphia (dominant hemisphere), visual hallucinations, visual perseverations (calcarine cortex), choreoathetosis, spontaneous pain (thala- mus), third nerve palsy, paresis of vertical eye movement, sensory loss, motor deficit (cerebral peduncle, midbrain)

VERTEBROBASILAR ARTERY (brain stem, embolic =thrombotic):

• motor or sensory loss in ALL 4 limbs; crossed signs (ipsilateral cranial nerve palsy with contralateral motor/sensory deficit), dysconju- gate gaze, nystagmus, ataxia, dysarthria, dysphagia

CEREBELLUM:

• ipsilateral limb ataxia, gait ataxia

INTERNAL CAROTID ARTERY (thrombotic >embolic):

• progressive or stuttering onset of MCA syndrome
• occasionally ACA syndrome as well

Question 125

Bulbar Palsy

Answer 125

refers to impairment of function of the cranial nerves IX, X, XI and XII, which occurs due to an upper and lower motor neuron lesion either at nuclear or fascicular level in the medulla oblongata or from lesions of the lower cranial nerves outside the brainstem.

Question 126

Progressive supranuclear Palsy

Answer 126

PSP:

The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.

Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms.

Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down.

Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles,sleep disruption, urinary incontinence and constipation.

The visual symptoms are of particular importance in the diagnosis of this disorder. Notably, the ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. Involuntary eye movement, as elicited by Bell’s phenomenon, for instance, may be closer to normal. On close inspection, eye movements called “square-wave jerks” may be visible when the patient fixes at distance. These are fine movements, that can be mistaken for nystagmus, except that they are saccadic in nature, with no smooth phase. Difficulties with convergence (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia(double vision) when reading.

Cardinal manifestations:

• Supranuclear ophthalmoplegia
• Neck dystonia
• Parkinsonism
• Pseudobulbar palsy
• Behavioral and Cognitive impairment
• Imbalance and Difficulties walking
• Frequent falls

Prognosis

There is currently no effective treatment or cure for PSP, although some of the symptoms can respond to nonspecific measures. The average age at symptoms onset is 63 and survival from onset averages 7 years with a wide variance.

Differential diagnosis

PSP is frequently misdiagnosed as Parkinson’s disease because of the slowed movements and gait difficulty, or as Alzheimer’s disease because of the behavioral changes. It is one of a number of diseases collectively referred to as Parkinson plus syndromes. A poor response to levodopa along with symmetrial onset can help differentiate this disease from PD.^[4]

Question 127

Foot Drop (What is it?)

Question 128

Confusional Assessment method CAM

Answer 128

The Confusion Assessment Method (CAM) is a simple tool that can be used by clinicians to integrate their observations and identify when delirium is the most probable diagnosis.

CAM questionaire if positive —> strongly argues for delerium +LR 10.3
CAM questionnaire if negative → argues against -LR 0.2

Need BOTH major criteria and one minior for test to be positive.

Question 129

Critical Illness Neuropathy

Question 130

Steppage Gait (Causes)

Question 131

Ataxia (Ddx)

Question 132

Thiamine Deficiency (Neurologic Manifestations)

Question 133

Mid Brain Peduncle lesion

Answer 133

Horner syndrome is the clinical combination of ptosis, anhydrosis (decreased sweating) and miosis (constriction of pupil). It is caused by a lesion or compression on one side of the cervical or thoracic sympathetic chain.

In order to get that plus a L body weakness (UMN) the lesion would need to be high up and likely prior to decussation (at the medulla oblangata). So you are looking at where the sympathetic chain meets a motor tract above the medulla.

The cerebral peduncle is a region of the midbrain. It carries the corticospinal (pyramidal [carries motor signals from the brain to the spinal cord) and corticobulbar tracts (motor information from the cranial nerve nuclei to the spinal cord).

Question 134

Gait Disorders (Approach)

Answer 134

Overview of gait disorders:

• UMN gait: When walking, the toes do not adequately clear the ground because the hip flexors are weak, and the toes scuff with each step. The strategy of circumduction at the hip helps with toe clearance. Inspection of the patient’s shoes may show wearing of the soles at the tips.
• LMN gait: In a patient with LMN foot drop, the gait is high stepping because the hip flexors are strong and compensate for the weakness to allow foot clearance. There may be an audible slap as the foot hits the ground.
• Myopathic gait: In myopathy, because of limb girdle weakness, the gait is waddling and there is abnormal pelvic tilt with each step.
• Parkinson’s: shuffling gait.

In B12 deficiency, the neuropathy is symmetrical and affects the legs more than the arms. It begins with paresthesias and ataxia associated with loss of vibration and position sense, and can progress to severe weakness, spasticity, clonus, paraplegia, and even fecal and urinary incontinence.

Question 135

Complex Migraines (symptoms)

Question 136

Describe this

Answer 136

Picture of blurred optic disc, inc central scotoma. This is likely optic neuritis associated with MS.

MS – Common symptoms include viual loss, diplopia, sensory symptoms in limbs or face, acute or subacute motor weakness, gait disturbance and balance problems, Lhermitte’s sign (electric shock-like sensations that run down the back and/or limbs upon flexion of the neck), bladder problems, limb ataxia, acute transverse myelopathy, and pain.

In MS, optic neuritis (ON) is the most common type of involvement of the visual pathways. It usually presents as acute or subacute unilateral eye pain that is accentuated by ocular movements. This is followed by a variable degree of visual loss (scotoma) affecting mainly central vision. In ON, disc edema (papilledema) may be observed on fundus examination when the acute ON lesion involves the head of the optic nerve

Other eye findings in MS are internuclear ophthalmoplegia (INO) refers to abnormal horizontal ocular movements with lost or delayed adduction and horizontal nystagmus of the abducting eye. It is caused by a lesion of the medial longitudinal fasciculus on the side of diminished adduction.

Question 137

Name the CN and the muscle

Answer 137

CN VII innervates the platysma. However, it does not decrease hearing. If anything, since it innervates the stapedius, sounds will seem louder (hyperacusis). If the defect was from a central cause, then no facial asymmetry with spontaneous smile.

Bell’s palsy typically presents with the sudden onset (usually over hours) of unilateral facial paralysis. Common findings include the eyebrow sagging, inability to close the eye, disappearance of the nasolabial fold, and the mouth drawn to the non affected side. Associated manifestations may include decreased tearing, hyperacusis, and/or loss of taste sensation on the anterior two-thirds of the tongue.

Central Peripheral Lesion

Location UMNContralateral motor cortex Descending pyramidal tractsLMNPeripheral nerve / facial nucleus in the ipsilateral ponsWeakness Predominantly lower facial musclesBoth upper and lower facial muscles

Movements affected: All facial movements on the side affected

Causes Unilateral- Stroke, demyelination (MS)- Cerebral tumours

Bilateral- Pseudobulbar palsy, poliomyelitis - motor neuron disease Pontine (ass. with CN 5 and 6 lesions)- Aneurysm, Tumour, Syringobulbia, MSPosterior fossa (ass. with CN 8)- Acoustic neuroma, meningioma, meningitisPetrous Temporal Bone (Facial Canal)- Bell’s Palsy, Ramsay-Hunt, Otitis mediaass with hyperacusis and/or loss of tasteParotid Gland- Tumour, SarcoidBilateral- Sarcoid, GBS, MG, Myopathies

Question 138

Status Epileticus

Answer 138

status = seizures longer than 5 min

tx: ativan 0.1 mg/kg / diazepam (can be given rectally)

Question 139

Partial Seizures (Treatment)

Answer 139

1st Line: Levetiracetam

Question 140

Seizures / Epilepsy

Driving Recommendations

Answer 140

LAW OF 3’s (Three Stooges)

• Single, unprovoked OR sz w/ med change: Investigations and 3 months
• Epilepsy on meds: No sz for 6 months (except for the following)
• Epilepsy postop: No sz for 12 months
• Epilepsy diagnosed: No sz for 2 yrs; otherwise wait 12 months
• Epilepsy drug taper: No sz for 5 yrs (off meds) and clear EEG in prev 6 mos
• ETOH: abstinent AND sz free for 6 mos after rehab program
• Auras/Simple partial: Stable dz for 12 months

https://www.cma.ca/secure/assets/assets-library/document/en/CMA-Drivers-Guide-8th-edition-e.pdf

Question 141

Seizures - unprovoked

How to investigate

Answer 141

• Neurologic Exam: Definitely
• Drug screen: Considered but NO evidence of benefit
• Neuroimaging: Definitely; MRI preferred but emergent CT may be considered
• EEG: Definitely; may be done as outpt except in non-convulsive status (useful for prognosis as well as tx determination)
• ECG: Definitely; sz-like attacks with a cardiovascular cause may be misdiagnosed as epilepsy

http://www.epilepsytoronto.org/…/download.php?…Recommendations%20in%…

Question 142

Seizures

AED

Answer 142

LAW of FIVE

1. ONE drug whenever possible
2. TWO drugs together only when switching and tapering off
3. THREE important Drugs:
   
   • Valproate
   • Carbamazepine
   • Phenytoin
4. FOUR important reactions:
   
   1. Pregnancy/teratogenicity
   2. STS/TENS: carbamazepine in Hans Chinese
   3. Hepatotoxicity
   4. Renal Injury
5. FIVE Reasons to monitor levels:
   
   1. Therapeutic Range
   2. Compliance
   3. Pregnancy
   4. Hospitalization or comorbid dz
   5. New meds

​http://www.epilepsytoronto.org/…/download.php?…Recommendations%20in%…

[NICE clinical guidelines, 2012]

http://www.ncbi.nlm.nih.gov/books/NBK2597/#ch14.s1

Question 143

Seizures

What are the mimics?

Answer 143

• Myoclonic Epilepsy
• Pseudoseizures
• Hypnagogic Hallucinations
• Absence Seizures
• Encephalopathy
  *

Question 144

CNS Tumor

What is the most common type and how to treat it?

Question 145

Intracranial Tumors

Ddx

Answer 145

• Non-tumors (ABCD/QRST): Abscess, Blood vessels (AVM), Cysts, Demyelinating DZ; “Quirky” toxo, radiation induced, Sarcoidosis, TB
• Tumors: Primary vs. Mets
  
  • Mets: multifocal with ring enhancement @ GW jxn; Breast, Lung, melanoma, Lymphoma
  • Primary: Histological (or benign vs malignant)
    
    • Glioma (30%): may enhance and can be bihemispheric; astocytoma/gliobalstoma (bleeding), ependymoma, oligodendroglioma (honeycomb pattern)
    • Meningioma (35%): Extradural, calcified, “light bulb” lesions
    • Schwannoma: Cerebropontine angle
    • Medulloblastoma: Posterior fossa +/- hydrocephalus
    • Craniopharyngioma (1%)
    • Pituitary (12%)/Pineal
• Notes:
  
  • Rule of 1/3’s: two of the major types are ~30% each; 30% of all types are malignant and 5yr mortality are ~30%
  • Always get family hx: FAP, Li-Fraumeni, neurofibromatosis
  • MMSE must >24 in order to be considered to drive

MKSAP 16

Question 146

Horner’s Syndrome

How to localize?

Question 147

Stroke in the young

Risk Factors?

Question 148

Amaurosis Fugax

What is it?

Answer 148

• Common presentation of carotid artery embolism
• Temporary monocular blindness “shade over vision”
• medical emergency

Question 149

Types of dizziness

Answer 149

1. vertigo: illusion of movement often related to a disorder of the vestibular system
2. near syncope: lightheadedness related to inadequate cerebral perfusion
3. disequilibrium: and unsteadiness related to a lack of sensory input or processing

Question 150

Tilt Table Testing
Indication?

Answer 150

is for neurocardiogenic or supected cardiac syncope

Question 151

Concussion

Management

Answer 151

definition: traumatically induced transient disturbance of brain function, involving a complex pathophysiological process.

• immediate cessation from activities and prompt assessment by a licensed health care provider.
• Initial assessment should be guided by a symptom checklist, cognitive evaluation, balance test, and for the neurologic examination.
• In suspected or diagnosed concussion the individual should be monitored for deterioration
• hypnotics should be avoided in the first 10 hours
• patient cannot return to activity on the same day, even without headaches
• routine imaging is not indicated and is reserved for patients with neurologic symptoms

Question 152

Restless legs syndrome

secondary causes?

Answer 152

1. Iron deficiency anemia
2. diabetes
3. end-stage renal disease

Question 153

Ulnar neuropathy versus C8 radiculopathy

Answer 153

ulnar neuropathies are characterized by normal grip, triceps reflex and lack of the Tinel sign at the elbow.

Question 154

Myotonic dystrophy

what is it?

Answer 154

• M.D., most common form of muscular dystrophy and adults, affects one in 8000 persons worldwide. it has a variable presentation with an autosomal dominant inheritance pattern. It is a multiorgan disease that is incurable.
• Type I (Steinert): DM, primary hypogonadism, and testicular atrophy are common problems. Cardiac arrhythmias may also be seen.
• Type II: generally milder in symptoms

Question 155

Best treatment for acute MS flare

Answer 155

IV methyl prednisolone 1 g daily 3-5 days; no conclusive evidence that it reduces disability at six months but it may shorten symptoms.

Question 156

benign positional vertigo / BPPV

Answer 156

Defn: calcium debris in semicircular canal Sx: Brief vertigo after changes in position; nystagmus +/-nausea vomiting Findings: Dix-Hallpike Mgmt: Epley maneuver

Note: can be caused by head trauma

Question 157

Vertigo

Peripheral and Central Causes

Answer 157

• Peripheral
  
  • BPV,
  • labyrinthitis - ativan, antinauseants,
  • vestibular neuritis, recurrent vestibulopathy,
    
    • toxic vestibulopathy: AG, loop diuretics, NSAIDs, cytotoxtic, AED
      *

Question 158

Charcot neuropathic osteoarthropathy

Answer 158

• a.k.a. “rocker bottom” foot
• etiology: diabetic neuropathy related inflammation
• x-ray should be the initial imaging modality: subtle fractures or subluxations

Question 159

Higher cortical functions

Answer 159

• Apraxia (inability to perform multistep task) is an early indication of frontal parietal problems such as Alzheimer’s disease

Question 160

Nerve conduction studies

Question 161

Gallium scan

Question 162

Somatosensory evoked potentials