Main

Neurology

Editor: Hunter Hewitt, MD

Reviewed by Christopher Lee, MD

Neurologic Emergencies

Stroke

Background

  • Preferred term: Stroke (CVA is like saying heart attack instead of STEMI)

  • Sudden onset, focal (usually one-sided) neurologic deficits: weakness, sensory loss, vision loss, ataxia/unsteadiness, vertigo, double vision, facial droop, dysarthria, aphasia

  • Differential:

  • stroke (ischemic or hemorrhagic)

  • seizure or post-ictal paralysis

  • headache phenomena (complex migraine)

  • cervical spinal cord lesions, though these more commonly cause bilateral symptoms

  • Stroke-like symptoms can also develop as recrudescence – previous stroke or brain lesion symptoms worsening with systemic toxic/metabolic/infectious processes or hypotension

Evaluation

  • Critical decision-making information: last known normal (LKN), time symptoms first observed, anticoagulation status, recent surgeries, history of bleeding (severe GIB or ICH), recent medications, platelet count, and baseline neuro exam

  • If symptoms developed with LKN within 24 hours -> stroke alert!

  • If > 24 hours, can request Neuroalert Instead

  • VUMC: call 11111 and tell the operator stroke alert and current patient location

  • NAVA: call an RRT and stat page 835-5137, include in the page 911 at the end of the call back number to signal it is a stroke alert

  • Stat head CTP (order CTH/CTA) for consideration of tPA or endovascular therapy
  • If renal function is abnormal, discuss with neurology

  • Generally, go for CTA if the patient is a thrombectomy candidate (within 24 hrs of onset)

  • MRI/MRA is an option but takes longer (MRAs are also better with Gadolinium)

  • Neurology service should be leading this portion

Management

  • Blood pressure goals
  • Ischemic stroke:

    • In general aim for SBP \<220

    • Patients with intracranial atherosclerosis may require higher BP to maintain perfusion

  • Hemorrhagic stroke:

    • SBP \< 140 (BP management is key)

    • These pts are managed in Neuro ICU

    • Reverse coagulopathies and keep platelets >100,000

Status Epilepticus

Background

  • Either a single seizure >5 minutes or ≥2 seizures occurring without a return to baseline in between

  • Differentiating convulsive seizures from non-epileptic events (“pseudoseizure”):

  • Features that suggest non-epileptic/psychogenic event include moaning or talking throughout the event, “no-no” head shake, repetitive movements of opposing muscle groups, very arrhythmic or purposeful-looking movements, or seizures that have been ongoing for “hours”

Evaluation

  • Fingerstick glucose, BMP/CBC, and UDS

  • Consult Neurology

  • EEG (start with 2hr) to determine if it is seizure or not and for titration of medications

  • Consider a non-contrasted head CT; MRI cannot be obtained while EEG is attached

  • Up to half of pts presenting in status epilepticus have no history of seizure, so they need urgent head imaging, consideration for lumbar puncture, infectious and toxic workup, tox screen, and sometimes rheumatologic or paraneoplastic workup

  • If Hx of seizure or on Antiseizure meds (ASMs) please order trough levels

Management

  • ABCs! Start with benzos:
  • 2 mg lorazepam IV then repeat q1-3 minutes up to 0.1 mg/kg OR

  • 5 mg of diazepam IV every minute (takes longer to give diazepam so would give concurrent ASM)

  • 10 mg IM midazolam if no IV access

  • After 2 rounds of benzos, would shift to antiepileptics if still in status (neurology should be contacted here if not already):
  • IV fosphenytoin 20 mg/kg

  • IV levetiracetam 30-60 mg/kg (generally 3g, up to 4.5g max)

  • IV valproic acid 30 mg/kg

  • If still seizing at this point, the patient will likely need intubation

  • These pts MUST be placed on EEG if they get paralyzed or sedated because convulsive status often continues as nonconvulsive status, which still damages the brain!

  • If still seizing, patients should be on midazolam, propofol or barbiturate infusions

  • Focal seizures, such as arm or face twitching with retained awareness do not always need to be treated to the point of initiating coma

Myasthenia Gravis (MG) and Lambert-Eaton Myasthenic Syndrome (LEMS)

Background

  • Disorders of the neuromuscular junction

  • MG affects the post-synaptic cleft at the acetylcholine receptor

  • LEMS affects the pre-synaptic cleft at the calcium channels

  • Many cases are paraneoplastic (classically small cell lung carcinoma)

Presentation

  • Double vision, ptosis, dysarthria, dysphagia

  • Dyspnea looks different than in other conditions: air hunger, usually also with dysphagia

  • Initially, the patient may not look sick or distressed, but may have a short inspiratory time or difficulty speaking in complete sentences due to shallow breathing

  • Most pts have a known history of myasthenia, but up to 20% present initially with crisis

Evaluation

  • Exam
  • Look closely for ptosis, nasal speech, weak neck flexion/extension (same nerve roots as diaphragm), interrupted speech to take extra breaths

  • These patients do not exhibit “huffing and puffing” like in COPD/asthma exacerbations

    • Patients with NMJ disease can go from talking to intubated within several hours!
  • LEMS: less ocular weakness, but do have extremity weakness and absent reflexes.

  • Pulmonary compromise is very rare in LEMS

  • EMG/NCS
  • MG: decremental response to repetitive stimulation

  • LEMS: increased amplitude in response to repetitive stimulation

  • Labs
  • Myasthenia antibody panels (send prior to IVIG/PLEX being given)
  • Imaging
  • Consider chest CT to look for thymic hyperplasia

Management

  • Monitor NIF (negative inspiratory force) at baseline and q4h
  • Measure of diaphragmatic strength

  • Normal is \<-60

  • If below -30, consider elective intubation

  • Note that patient effort will affect NIF values

  • IVIG or PLEX
  • Both have similar supportive evidence; IVIG is usually easier to do

  • PLEX has the risks you would expect with dialysis (e.g. fluid shifts) and coagulopathy

  • IVIG -> check IgA levels. Can increase risk of DVT, has risk of aseptic meningitis and provides significant fluid load so not ideal for pts with CHF

  • Steroids
  • Usually up-titrated SLOWLY (by 10-20 mg prednisone daily)

  • Rapid increases in steroids can worsen patients with MG**, so talk to neuro before adjusting**

  • Pyridostigmine
  • Typically continue at their home dose

  • Too much pyridostigmine can make patients worse (more secretions), so for those doing poorly on >90 mg per dose, consider lowering the dose

  • Treat underlying causes of exacerbations: usually infections or other toxic/metabolic insults

  • Remove/avoid exacerbating medications: fluoroquinolones, aminoglycosides, beta blockers, and magnesium

  • LEMS specific management:

  • 3,4-diaminopyridine

  • Can respond to IVIG or pyridostigmine

  • Workup for underlying neoplasm

Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

Background

  • Rapid onset polyneuropathy that manifests most often with ascending weakness and numbness that can involve the respiratory and facial musculature

  • Usually preceded by infectious illness a few weeks prior

  • Patients are much more likely to get AIDP from an infection than any vaccine, weak vaccine links to AIDP are an addition 1-2 cases per million flu vaccines.

Presentation

  • Acute, progressive extremity weakness, weak or absent reflexes, and potentially subjective sensatory changes, especially back pain
  • There are a TON of variants of AIDP, with some having cranial nerve weakness early, proximal weakness, sensory ataxia, primarily sensory loss, or rapid muscle weakness; do not use lack of classic ascending weakness to dismiss the idea of AIDP

  • Sensory loss is common in an ascending pattern too

Evaluation

  • LP – albuminocytologic dissociation = high protein with normal cell count
  • One exception is HIV, which can cause AIDP but also have a high cell count and high protein count
  • EMG/NCS
  • Demyelinating pattern (temporal dispersion and decreased conduction velocities).

  • This can be normal in the first few days, and is most useful 2+ weeks out if the diagnosis is still unclear

  • Differential diagnosis: spinal cord lesions, LEMS, MG, acute HIV or HCV, viral myelitis (entero/West Nile)

Management

  • ABCs! NIF at baseline then Q4-6 hours
  • NIF \< -30 with good effort generally warrants ICU monitoring
  • IVIG or PLEX

  • Can get worse with steroids

Elevated Intracranial Pressure (ICP) and Hydrocephalus

Background

  • Communicating hydrocephalus (i.e. non-obstructive)
  • Causes: subarachnoid granule scarring after subarachnoid hemorrhage or meningitis, ependymoma producing excess CSF, venous sinus thrombosis
  • Non-communicating hydrocephalus (i.e. obstructive)
  • Causes: tumor, abscess, or hematoma in the midline ventricular structures
  • Eventually, elevated ICP will cause brain herniation

Presentation

  • Headache, blurred vision, visual field reduction, enlarged blind spot, nausea, vomiting, syncope, coma

  • Sixth nerve palsies are common

  • Third nerve palsies (blown pupil) are classically associated with uncal herniation

Evaluation

  • Good visual exam: visual fields, enlarged blind spot, papilledema (may not be present if very rapid ICP increase, even with vision loss), and 6th nerve palsies

  • Stat head CT to look for obstructions, mass lesions

  • NSGY evaluation if obstructive lesion (removal vs ventricular drain)
  • CTV or MRV to look for venous sinus thrombosis
  • Venous sinus thrombosis needs AC, even if there is some degree of hemorrhagic infarction
  • If no obstructive lesion LP with opening pressure
  • If workup is otherwise normal, except for elevated opening pressure diagnosis = IIH

Management

  • Idiopathic intracranial hypertension (IIH)
  • Diamox and/or topiramate

  • Ophthalmology evaluation emergently for consideration of nerve sheath fenestrations or urgent VPS placement if severe disc edema

  • If there is clinical concern for herniation:

    • Mannitol: 50g IV, can be given peripherally. Has risks of renal injury

    • Hypertonic saline: 3%, 7% or 23% saline can be given, needs central access

    • Maintain head of bed at least 30° and loosen neck obstructions (c-collars) as able

    • NSGY consult for shunt consideration

  • Hyperventilation can be done with goal PaCO2 30-34 mm Hg or ETCO2 20-30 mmHg but is only a temporizing measure and risks rebound edema

    • After 4-6 hrs, compensatory pH changes in the blood prevent vasoconstrictive affects

Common Neurologic Problems

Altered Mental Status (AMS)

Background

  • Definition: Change in a patient’s baseline cognition
  • Can be hypoactive (lethargic) or hyperactive (agitated)
  • Risk factors: Functional impairment, age > 75, dementia, depression, ETOH/substance use disorder, sensory impairment, recent surgery

Etiologies: Consider MOVE STUPID mnemonic

  • Metabolic (Hypo/hypernatremia, Hypercalcemia)

  • Oxygen (Hypoxia)

  • Vascular (CVA, Bleed, MI, CHF)

  • Endocrine (Hypoglycemia, Thyroid, Adrenal)

  • Seizure (postictal state)

  • Trauma

  • Uremia

  • Psychiatric

  • Infection

  • Drugs – intoxication, withdrawal, or medications

  • Delirium – see “Delirium” section in psychiatry

Evaluation

  • Consider broad toxic, metabolic, and infectious workup as appropriate
  • TSH, Vitamin B12, CBC, CMP, UA, CXR, VBG, blood Cx, glucose, UDS
  • Review of medications
  • Sedatives, anticholinergics, benzos/EtOH toxicity or withdrawal
  • Head imaging in the setting of focal neurologic findings
  • Start with head CT – note strokes take up to 24 hours to show up on CT

  • Consider MRI if high concern for stroke, inflammatory changes or infection

  • LP should be performed if there is any concern for meningitis

  • EEG is reasonable with fluctuating mental status or seizure-like activity

Management

  • First line: nonpharmacologic interventions: HOMMEEESS
  • Hydration/nutrition: ensure patient fed, rule out constipation and urinary retention

  • Orientation

  • Mobilize out of bed 3x/daily as able

  • Manage pain

  • Eliminate unnecessary devices (restraints, catheters, tele, lines) and meds

  • Environmental modification: minimize devices, lights on/windows open during day

  • Engage family

  • Sensory restoration: use eyeglasses and hearing aids and reorient

  • Sleep protocol: minimize nighttime vitals, earplugs, sleep mask and no TV at night

  • Second line: Pharmacologic approaches, see "Delirium" in section in psychiatry

Seizure without Status Epilepticus

Background

  • Risk factors: birth trauma, prematurity, TBI with loss of awareness > 1 hours or penetrating wound, strokes/tumors/abscesses, history of meningitis/encephalitis

  • Key for seizures: stereotyped event with sudden onset/offset

  • Generally, if full body systems are involved (e.g., jerking or tonic activity), then there will also be loss of awareness

Evaluation

  • A clear description or recording of seizure semiology is helpful

  • Provoked seizures can develop with medications, hypo/hyperglycemia, significant electrolyte abnormalities (e.g. hyponatremia), and CNS infections

  • EEG is necessary for spell capture

  • MRI brain with and without contrast once stable

Management

AED Side effects
Levetiracetam (Keppra) (PO/IV)

Sedation and agitation, worsening of underlying mood disorders.

Can trial B6 supplementation to help with mood effects

Valproic acid (Depakote) (PO/IV) Sedation, hirsutism, PCOS, P450 inhibitor, nausea, liver injury, hyperammonemia
Phenytoin (Dilantin) (PO/Fosphenytoin IV) Sedation, gingival hyperplasia
Lacosamide (Vimpat) (PO/IV) Heart block, dizziness, ataxia
Topiramate (Topamax) (PO) Kidney stones, metabolic acidosis, paresthesias, weight loss, cognitive slowing
Carbamazepine (Tegretol) (PO) Hyponatremia, SJS (in Han Chinese check HLA), bone marrow suppression (rare)
Oxcarbazepine (Trileptal) (PO) Similar to carbamazepine
Lamotrigine (Lamictal) (PO) SJS/TEN, nausea. Least sedating
Zonisamide (Zonegran) (PO) Sedation, ataxia, nausea, confusion

Non-Epileptic Spells (aka PNES, psychogenic non-epileptic spells)

  • Can be very difficult to distinguish from epileptic seizures

  • Features more common in PNES

  • Retained awareness with bilateral extremity “seizing”

  • Opisthotonus (arching the back)

  • Talking during a spell

  • Excessively long spells (e.g. lasts hour or days)

  • Forced eye closure

  • Coachability during a spell or reacting to external stimuli

  • Heavy breathing during a spell with lots of rigorous movement

  • Immediately returning to normal after a spell

  • Features more common in epileptic seizures:
  • Seizures arising out of sleep

  • Highly stereotyped

  • Incontinence

  • Severe injuries (e.g. burns)

  • Management
  • Try to avoid excessive BZD use

  • This requires good clinical judgement as you wouldn't want to withhold Ativan and discover that the pt was having true atypical seizures. The compromise would be: do not repeatedly administer BZDs when there is suspicion for PNES as well as no evidence of response to prior BZD administration.

Syncopal Convulsions

  • Very common, can present with posturing and tonic-clonic movements happening for a few moments after syncope
  • Should not last for more than 30 seconds

  • These are just related to syncope and do not typically require seizure medications

  • Workup:

    • Two-hour EEG and MRI (with and without contrast)

    • Infectious workup, BMP, CBC, blood glucose, toxicology/drug screen

    • If there is concern for convulsive syncope, (carefully) check orthostatic vitals

Inpatient Headache (HA)

Background

  • Important to distinguish primary and secondary headache

  • Red flags for secondary headaches (SNOOPP): Systemic symptoms, Neurologic symptoms, Onset that is sudden (thunderclap), Older age (new headache >40), Progression or evolution in previous headaches, Postural component

  • Other red flags: preceding trauma, headache awakening patient from sleep, no headache-free intervals, thunderclap headache (maximal intensity develops within 11 minutes or less)

Evaluation

  • Get a good description of where the pain is, when, associated symptoms, and assess for “red flag” features listed above

  • If there are any red flag features imaging and workup are necessary

  • Imaging depends on highest suspicions, but CTA head/neck is appropriate to evaluate for aneurysm (including neck to consider dissection). If any focal signs, MRI is generally preferred; venous imaging can be beneficial in headaches with features of elevated ICP

  • If no red flag features, then workup is not necessary, and focus is on treatment

Management

  • NSAIDs and Tylenol for infrequent headaches, but consistent use (>2-3 a week) runs the risk of rebound headaches

  • Triptans for migraine, but contraindicated in patients with CAD, uncontrolled HTN, previous stroke. They must be used within 6 hour of onset

  • There are theoretical concerns of serotonin syndrome when used with SSRI/SNRIs
  • Migraines:
  • “Migraine cocktail”: 1L fluid bolus, 4g Mg, IV Compazine(10mg) OR Phenergan(20mg) with Benadryl (25mg)

  • 2nd line: Depakote 1000 mg IV, decadron 10mg IV , ± toradol 30mg IV, flexeril 10mg PO

  • Cluster headache:
  • Triptans, high flow O2 (>10 L), sometimes intranasal lidocaine if no arrhythmia history

Outpatient Headache

Type Presentation First line meds
Tension type (most common) Generally bilateral, pressure/tightness, mild/moderate pain, no significant photophobia, phonophobia, or nausea Acetaminophen, TCAs, SNRIs
Migraine

Unilateral, pulsating, moderate-to-severe pain, lasts 4-72hr, worse with activity and improves with sleep

Associated with nausea, photophobia or phonophobia

± Aura

Acute: triptans

Preventive: TCAs, propranolol, topiramate, VPA

Cluster Severe, often extreme unilateral orbital/supra-orbital/temporal pain, often with lacrimation, rhinorrhea, sweating, swelling of face, visual changes Acute: 100% FiO2 at 12L/min for at least 15 mins, triptans, Indomethacin
Medication Overuse HA at least ½ the days of the month, w/medication intake at least ½ the days of the month; often presents as worsening HA despite increased intake of medication. Often seen with meds that include caffeine (Excedrin, fioricet) \*STOP offending medication, typically via taper. HA will worsen before it gets better, start concurrent daily prophylactic headache medication

Medication Overview:

  • Abortive
  • Triptans (e.g. sumatriptan or rizatriptan)

    • Cannot be used more than 10 days/month

    • Avoid in pts with hypertension/CAD

  • Preventative
  • Amitriptyline: indicated for both migraine and tension-type. Helps with sleep and comorbid depression. Most common side effects (SE) = dry mouth, sedation

  • Topiramate: has the best evidence among migraine meds. Can theoretically help with weight loss. Most common SE = sodas taste bad, sedation, parasthesias

  • Propranolol: useful for relative lack of interactions. Mild cardiac/blood pressure effects compared to other beta-blockers. Most common SE = drowsiness

  • Magnesium oxide: reduces headache frequency with almost no SE. Start 400mg daily, can go up to 800mg BID. Patients can increase dose until they get diarrhea.

  • Riboflavin (vitamin B2): mild effect but effectively has no side effects. 400mg daily.

  • Gabapentin: can be useful if HAs have stabbing/electric quality. Main SE = sedation

  • Venlafaxine: useful for migraines with significant vestibular symptoms (dizziness). SE = hypertension/tachycardia.

  • Verapamil: can be used for migraine and cluster headaches. Can use ER formulation

  • Botox: can be administered every 3 months. Can be very effective, but pts generally will have had to fail multiple medications for insurance to approve refer to neuro resident clinic

  • CGRP receptor modulators (mostly injections) such as Rimegepant are newer options

Parkinson’s Disease

Clinical Presentation

  • Resting tremor is typically a very early symptom, often worse on one side

  • Cogwheel rigidity; can be confused for paratonia, which is seen in demented or encephalopathic patients who have involuntary variable resistance movements during passive ROM assessment

  • Speech changes (low volume), hand-writing changes (slow small movements)

  • Gait changes

  • Festination – slow start with movements that gradually build up speed

  • En bloc turning – taking multiple steps to turn around

  • Anosmia and REM behavior sleep disorders are very common

Evaluation

  • Clinical diagnosis; there are some supportive imaging studies like DaTscan that looks for activity of substantia nigra (usually not necessary)

  • Clinical response to dopamine replacement is so typical that if a patient does not respond, it is important to consider a Parkinson plus syndrome (see below)

Management

  • Dopamine replacement – carbidopa/levodopa; dosed at regular intervals several times a day. These generally do not need to be held on admission.
  • If pt is altered, can hold anticholinergics, MAO-B inhibitors or COMT inhibitors
  • Dopamine agonists – can cause confusion, hallucinations, dyskinesias

  • MAO-B inhibitors (MAOIs): can cause confusion, hallucinations, insomnia and dyskinesias

  • COMT inhibitors – can cause confusion, hallucinations, insomnia, and dyskinesias

  • Anticholinergics – useful for tremor when there is not much bradykinesia or gait disturbances. In older pts cognitive changes are a bigger concern along with hallucinations

  • PD medications are rarely titrated in the hospital because acute medical illness makes PD symptoms worse and everything will need to be re-adjusted as an outpatient

  • Be cautious with PRN anti-emetics in patients with PD. Many work via dopamine antagonism. Zofran is generally the safest option.

  • Similarly, many antipsychotics have dopamine antagonism. Safest option is seroquel

Parkinson Plus Syndromes

Evaluation

  • Consider if atypical features such as bilateral symmetric onset, early cognitive/personality changes, cerebellar findings, or prominent autonomic dysfunction early

Types

  • Progressive Supranuclear Palsy
  • PD symptoms with early falls and minimal tremor

  • Vertical eye movement abnormalities

  • Multisystem Atrophy
  • Profound orthostatic hypotension without any increase in HR

  • Three types:

    • MSA-A – autonomic features prominent (previously Shy-Drager Syndrome)

    • MSA-P – prominent atypical Parkinsonism features

    • MSA-C – prominent cerebellar dysfunction

  • Lewy Body Dementia
  • Parkinsonism with prominent early cognitive impairment and hallucinations

Brain Masses

Background

  • Neoplasm is the biggest concern
  • 90% of malignant brain masses are metastatic

    • Most commonly: lung, RCC, breast, melanoma

    • Highest bleeding risk: melanoma, thyroid, choriocarcinoma, RCC

  • Primary brain tumors

    • Gliomas: WHO Grade I-IV

      • Glioblastoma multiforme (GBM) – WHO Grade IV; large heterogenous masses with edema; heterogenous contrast enhancement; can cross the corpus callosum

      • Lower grade gliomas – include oligodendrogliomas and astrocytomas

    • Meningioma: usually low grade

      • Can be left alone and monitored with yearly MRI

      • If symptomatic, may need resection/radiation

    • Ependymoma: uncommon. Can cause CSF outflow obstruction

    • CNS lymphoma – diffuse WM involvement, with mass effect, restricts diffusion on MRI with prominent contrast enhancement. Can also cross the corpus callosum

      • Usually B-cell, initially responds significantly to steroids

Presentation

  • A significant number of brain lesions are detected incidentally

  • If a patient has a first-time seizure, brain mass needs to be ruled out

  • HA (usually constant, severe), seizure, and focal neurologic deficits

Evaluation/Management

  • Imaging: MRI w/ and w/o contrast provides the most information
  • Findings suggesting malignant lesions: marked edema, multifocal lesions, or presence at gray-white junctions
  • LP may be indicated if herniation risk is low, particularly if concerned for infection

  • Biopsy will ultimately be needed in most cases, which is done by NSGY

Management

  • Work up for primary malignancy, CT C/A/P + PET

  • Steroids are generally indicated for treatment of edema

  • Decadron 10 mg IV to start; then transition to 4mg IV q6h with SSI
  • Symptomatic tumors need eval by NSGY for resection consideration and radiation oncology

Amyotrophic Lateral Sclerosis (ALS)

Background

  • Progressive weakness, often asymmetric and profound with no sensory loss

  • Combination of LMN and UMN findings

  • LMN findings: weakness, flaccidity, absent/decreased reflexes, fasciculations

  • UMN findings: weakness, spasticity, hyperreflexia, pathologic reflexes (e.g. extensor plantar response), slow movements

  • Pts with bulbar symptoms may complain of difficulty swallowing, changes in their voice, or increased saliva production (they just aren’t swallowing it)

  • Tongue fasciculations are fairly uncommon in other disorders and is a good clue for ALS if present (these can be hard to be certain of – tongue movements that are symmetric are typically not fasciculations)

  • Pseudobulbar affect – inappropriate laughing or crying; relatively common in ALS

  • FVC is an important marker for function

Evaluation

  • EMG/NCS is gold standard; El Escorial Criteria looks for evidence of UMN and LMN findings at cranial, cervical, thoracic and lumbosacral levels to determine if ALS or not

  • Exclude mimicking lesions, which may be treatable (see below)

Management

  • Patients with ALS are ideally treated with a multidisciplinary team of Neurologists, Pulmonologists, Speech therapists, PT, OT and mobility

  • Two medications have been shown to add a few months to survival:

  • Riluzole - PO med; have to track CBC/LFTs. Side effects: GI and general weakness

  • Edaravone (Radicava) – IV, given daily for 2 weeks every month. SE = HA, bruising

Mimics of ALS

  • High cervical spine lesions –UMN changes in upper extremity with LMN pattern in LE

  • Primary Lateral Sclerosis – UMN-only disease, much less common with much slower progression. Many transition to ALS in the first 1-2 years, which then is likely a spectrum of the same disease

  • Multifocal Motor Neuropathy – rare autoimmune disorder that looks like ALS with LMN signs only, responds to IVIG. Can have anti-GM1 Ab in blood. EMG/NCS pattern is different with conduction block

  • Kennedy Disease – X-linked genetic disorder with progressive LMN pattern of weakness and endocrine disorders w/androgen resistance profile (gynecomastia, defective spermatogenesis)

  • Inclusion Body Myositis – can be asymmetric with grip weakness and quadriceps weakness. Biopsy and atrophy pattern usually distinguishes it. CPK 500-800.

  • Polymyositis/dermatomyositis – proximal weakness in arms/legs, CPK > 1000, usually younger onset (30-40s), no UMN signs

Multiple Sclerosis

Background

  • Progressive inflammatory disorder primarily manifesting with demyelination of the central white matter

  • Optic neuritis and transverse myelitis (spinal cord lesion) are common presentations

  • Generally develop over a few days; very uncommon to happen suddenly (e.g., patients will complain about a dark spot appearing in their vision that expands over several days)

Evaluation

  • MRI w/ and w/o contrast can identify plaques and determine if they are more acute
  • “Active” MS plaque will enhance, and continues to for weeks (even after treatment)
  • Modified MacDonald Criteria: ≥3 characteristic demyelinating lesions (>1 cm, periventricular, infratentorial (brainstem/cerebellum/cord) or juxtacortical in location) with evidence of separation in time (active and chronic)

  • LP with studies for oligoclonal bands, IgG index, cell count and protein, anti-mog, anti-aqp4

Management

  • Treat flares and optic neuritis with steroids
  • Speeds up recovery, but does not improve the degree of recovery

  • Often dosing starts with methylpred 1g

  • If a patient with known MS has worsening symptoms that are not new, then recrudescence is the likely cause infectious/toxic/metabolic workup and imaging is needed

  • There are several long-term medications; common side effects listed below:

  • Interferon (SQ injections) – flu-like symptoms, injection site reactions

  • Glatiramer acetate (SQ) – injection site reactions

  • Fingolimod (PO) – macular edema, liver injury, increased risk of skin cancer

  • Teriflunomide (PO) – liver injury, hair loss, immunosuppression, teratogenic

  • Dimethyl fumarate (PO) – GI side effects, lymphocytopenia, liver injury

  • Natalizumab (IV) – PML concern, immunosuppression

  • Ocrelizumab (IV) – contraindicated in active HBV infection, cannot give live vaccines

  • Alemtuzumab (IV) – autoimmune disease, rash, headache

Neuromyelitis Optica and Spectrum Disorder

  • Demyelinating disease due to Ab against aquaporin-4 (on oligodendrocytes)

  • Classically causes optic neuritis and longitudinally extensive transverse myelitis

  • Can be more aggressive than MS
  • Diagnose with NMO antibodies and MS workup as above

  • Treated with steroids, but in severe or refractory cases may require PLEX

Vertigo

Background

  • Clinical Presentation
  • Sensation of the room spinning or moving, or themselves spinning or moving

  • Must distinguish from orthostasis/presyncope via history

  • Causes: most easily differentiated based on chronicity and triggers rather than description of symptoms
  • BPPV - short duration, very positional, classically nystagmus is horizontal and torsional with the posterior canal being most commonly involved

  • Vestibular neuronitis/labyrinthitis – typically follows URI or ear infections; often self-limited, but can be severe/prolonged, in which case it is treated with steroids

  • Menière’s Disease – tinnitus and low range frequency hearing loss, generally progressive and treated with diuretics, meclizine and sometimes surgeries or intratympanic injections

  • Endolymphatic Leak – usually following trauma or concussive blasts, requires ENT eval and management. Classically a loud sound will cause vertigo and nystagmus

  • vertiginous migraine: usual migraine triggers, may have aura. Episodic nature and often positive family hx of migraine or even vertiginous migraine.

  • Stroke – sudden onset, ask about vascular risk factors, rare cause of isolated vertigo

Evaluation/Management

  • Careful exam of vertigo will be more helpful than random scans, but pt has to be symptomatic for exam to mean anything:
  • HINTS Battery – Head Impulse test, Nystagmus pattern, Test of skew

    • Central pattern – no corrective saccade, multidirectional nystagmus, skew present

    • Peripheral pattern – corrective saccade, unidirectional nystagmus, no skew present

  • Dix-Hallpike Test

  • Cerebellar testing: FNF, HKS, mirroring, gait

  • Outpatient Vestibular function testing with ENT

  • Central patterns will need head and vessel imaging (looking for vertebral dissection or basilar clots)
  • Often, central vertigo is due to centrally acting medications
  • Peripheral causes are varied and often require eval by ENT as an outpatient

  • Treatment with anticholinergics like meclizine or scopolamine is often helpful

  • Vestibular therapy with OT is also very beneficial


Last update: 2022-06-09 16:32:41