Internal Medicine EOR - Neuro

Autosomal dominant disorder that prevents with early-onset dementia (< 50), chorea (rapid involuntary movements) and behavioral changes; may also have gait abnormalities, incontinence

Huntington Disease

Diagnosis of Huntington's Disease

- Clinical evaluation

- Family hx + genetic testing

- Neuroimaging (CT scan)

Management of Huntington Disease

supportive measures, genetic counseling for relatives; can give antidopaminergics for chorea, antipyschotics and benzoes.

NO CURE, USUALLY FATAL WITHIN 15-20 YEARS AFTER PRESENTATION

Postural, bilateral, ACTION tremor of the hands, forearms, head neck or voice; worsened with emotional stress and intentional movement, shortly relieved with EtOH ingestion; WILL HAVE NO OTHER NEURO FINDINGS

essential benign/familial tremor

Management of essential tremor

Tx usually not needed, but can give propranolol if severe or situational

Clinical manifestations of Parkinson disease

1. TREMOR - resting tremor, "pill rolling" worse with rest and lessened with activity/intentional movement

2. BRADYKINESIA - slowness of voluntary movements, decreased automatic movements (i.e. shuffling gait, lack of swinging arms when walking)

3. RIGIDITY - "cogwheel" flexed posture, festination

4. FACE INVOLVEMENT - fixed facial expressions, widened palpebral fissure, decreased blinking

5. INSTABILITY - postural instability (usually a late finding)

How is Parkinson's diagnosed?

clinically; usually confirmed with response to dopaminergic therapy

Medical management options in Parkinson disease

1. LEVODOPA/CARBIDOPA - most effective treatment

2. Dopamine agonists (i.e. Bromocriptine, Pramipexole) - sometimes used in young patients to delay use of levodopa/carbidopa

3. Anticholinergics (benztropine, trihexyphenidyl) - used only in pts < 70 with tremor predominance

+ Amantadine, MAO-B inhibitors, COMT inhibitors

Condition that presents with mixed upper and motor neuron symptoms; including spasticity, stiffness, hyperreflexia, b/l fasciculations, muscle atrophy, muscle weakness, hyporeflexia; eventually leads to dysphagia, dysarthria, respiratory dysfunction and death

amyotropic lateral sclerosis (ALS)

Symmetric, ASCENDING weakness and paresthesias that also presents with decreased DTRs and autonomic dysfunction (i.e. tachycardia, hypotension, breathng difficulties)

Guillain Barre Syndrome (GBS)

There is increased incidence of GBS following which infection?

Campylobacter jejuni (but can also be preceded by other respiratory or GI infections)

Diagnosis of GBS

- clinical evaluation

- LP w/ CSF (high protein, normal cell count)

- if necessary, can also do electrophysiologic studies

Management of GBS

Intensive supportive care + IVIG/plasmapharesis

About 10-20% of patients with GBS are left with permanent disability known as

chronic inflammatory demyelinating polyneuropathy (CIDP)

Neuro disorder that presents in young women as diplopia, ptosis, and generalized muscle weakness that WORSENS with use/throughout the day

myasthenia gravis

75% of patients with MG have what?

thymic abnormality (thymoma or hyperplasia)

Pathophysiology of MG

autoimmune antibodies against acetylcholine postsynaptic receptor at the neuromusclar junction

Diagnosis of MG

- (+) acetylcholine receptor antibodies

- Endrophonium test --> rapid response to short acting IV Endrophonium in limb MG

- CT scan or MRI may show thymoma or thymus gland abnormality

- ice pack test = place on eyelid for 10 min, improvement of ptosis in ocular MG

Management of Myasthenia Gravis

1. ACETYLCHOLINESTERASE INHIBITORS (i.e. pyridostigmine, neostigmine): increases acetylcholine by decreasing its breakdown, can cause cholinergic crisis if too much

*for myasthenic crisis (respiratory depression), use plasmapharesis or IVIG

*if there is a thymoma, thymectomy

Syndrome similar to MG that is associated with small cell lung cancer; presents with weakness that IMPROVES with repeated use

Lambert-Eaton

Neuro condition (MC in young women) that presents with headaches (+ trigeminal neuralgia), pain, fatigue, numbness, paresthesias in the limbs, muscle cramping, unilateral eye pain/vision changes, bladder/bowel/sexual dysfunction

Multiple sclerosis

Uhthoff's phenomenon

MS symptoms that worsen with heat (i.e. exercise, fever, hot shower, jacuzzi)

Lhermitte's sign

neck flexion causing lightening shock-type pain radiating from the spine down the leg; seen in MS

Optic neuritis

unilateral eye pain that is worse with movements, diplopia, central scotomas, vision loss (especially color)

*seen in MS

Marcus Gunn pupil

Afferent pupillary defect (e.g., due to optic nerve damage or retinal detachment); Decreased bilateral pupillary constriction when light is shone in affected eye

*seen in MS

Diagnosis of MS

MRI w/ contrast (of brain AND spine) = test of choice to confirm MS

*will see white matter plaques

*can also get LP

What will you see in the CSF of someone with MS?

oligoclonal bands (inc IgG)

Management of ACUTE exacerbation of multiple sclerosis

IV corticosteroids (methylprednisolone 1000mg IV once a day for 3-5 days)

Disease-modifying therapies for multiple sclerosis

beta-interferon or glatiramer acetate (Copaxone); new drugs include Fingolimod, Natalizumab

*can use Baclofen for spasticity, Gabapentin for paresthesias, amantadine for fatigue

Sudden onset of ipsilateral ear pain --> unilateral facial paralysis +/- taste disturbance

Bell palsy

*usually cannot wrinkle forehead, smile on affected side; if they can wrinkle both sides of forehead, it is NOT Bell palsy

Risk factors for bell palsy

DM, pregnancy, post-URI***, dental nerve block

*bell palsy thought to be related to herpes simplex reactivation

Management of Bell Palsy

no tx in most cases; if so, may use prednisone (esp effective within first 72 hours)

MC type of headache; presents as bilateral, tight, band-like constant daily headache that is worsened with stress and fatigue; will NOT have any N/V or focal neurologic symptoms

tension headache

Management of tension headaches

1st line = NSAIDS, aspirin, acetaminophen

2nd line = triptans, ergotamines

*in severe or recurrent cases, can use TCAs, B-blockers, etc

Presents as a lateralized, pulsatile/throbbing headache associated with N/V, photophobia, phonophonia; is worse with activity, stress, EtOH, chocolate, OCPs/menstruation

migraine headache

What are the auras that can be associated with migraines?

light flashes (photopsia), zig zag lines of light (teichopsia), scotomas (blind spots), aphasia, weakness, numbness; usually come before headache, last for 5-20 min

Management of migraine headache

SYMPTOMATIC:

- if mild, use NSAIDS

- moderate, most commonly use Triptans or Ergotamines

- for N/V, give dopamine blockers IV (usually metoclopramide)

PROPHYLACTIC: anti-HTN meds (B-blockers, CCBs), TCAs, anticonvulsants (i.e. Valproic acid)

Headache in young males that presents with severe sharp UNILATERAL periorbital/temporal pain lasting about 2 hrs with spontaneous remission; associated with ipsilateral Horner's syndrome, rhinorrhea, lacrimation

Cluster headache

Triggers for cluster headache

worse at night***, EtOH, stress, ingestion of specific foods

Management of cluster headaches

100% O2 (first line) +/- SQ Sumatriptan

Prophylaxis of cluster headaches

Verapamil

Seen in obese women of childbearing age; presents as headache (worse with straining), retrobulbar pain, N/V, tinnitus, and visual changes; have papilledema on fundoscopic exam

idiopathic intracranial HTN (pseudotumor cerebri)

Diagnosis of pseudotumor cerebri

1st --> CT scan (to r/o mass)

2nd --> LP (will show inc OP, otherwise normal)

Management of pseudotumor cerebri

acetazolamide

Classic triad of dementia, gait disturbance (wide-based shuffling) and urinary incontinence

normal pressure hydrocephalus

Diagnosis of NPH

MRI > CT (will show enlarged ventricles)

Management of NPH

ventriculoperitoneal shunt

Characteristics specific to DELIRIUM

ONSET - sudden****

DURATION - days to weeks

CAUSE - other condition (i.e. infection, drugs, etc)

COURSE - reversible***

ATTENTION - greatly impaired***

LEVEL OF CONSCIOUSNESS, ORIENTATION - fluctuating***

SPEECH - inappropriate, incoherent

MEMORY - usually intact

Characteristics specific to DEMENTIA

ONSET - gradual****

DURATION - permanent**

CAUSE - chronic brain disorder

COURSE - irreversible***

ATTENTION - unimpaired

LEVEL OF CONSCIOUSNESS - unimpaired***

ORIENTATION - impaired***

SPEECH - sometimes difficulty finding right word

MEMORY - LOST!****

Acute, abrupt, transient confused state due to an identifiable cause; associated with fluctuating mental status, marked deficit in attention and short-term memory

delirium

Progressive, chronic, intellectual deterioration of selective functions, especially memory loss (but can also include loss of impulse control, motor and cognitive functions)

dementia

Most common type of dementia

Alzheimer's Disease (AD)

Pathophysiology/pathology of Alzheimer's disease

Extracellular beta-amyloid deposits (in senile plaques)

Intracellular neurofibrillary tangles (tau protein, paired helical filaments)

Clinical manifestations of Alzheimer's disease

- Loss of short-term memory (eg, asking repetitive questions, frequently misplacing objects or forgetting appointments)

- Impaired reasoning, difficulty handling complex tasks, and poor judgment (eg, being unable to manage bank account, making poor financial decisions)

- Language dysfunction (eg, difficulty thinking of common words, errors speaking and/or writing)

- Visuospatial dysfunction (eg, inability to recognize faces or common objects)

- Behavior disorders (eg, wandering, agitation, yelling, persecutory ideation)

Usual age of onset for Alzheimer's

≥ 65 yr

Diagnosis of Alzheimer's disease

- Formal mental status examination

- History and physical examination (clinical criteria)

- Neuroimaging (cerebral cortex atrophy on CT)

ONLY DEFINITIVE DX IS POST MORTEM

Traditional diagnostic criteria for Alzheimer disease include all of the following:

1. Dementia established clinically and documented by a formal mental status examination

2. Deficits in ≥ 2 areas of cognition

3. Gradual onset (ie, over months to years, rather than days or weeks) and progressive worsening of memory and other cognitive functions

4. No disturbance of consciousness

5. Onset after age 40, most often after age 65

6. No systemic or brain disorders (eg, tumor, stroke) that could account for the progressive deficits in memory and cognition

7. Biomarkers (beta-amyloid or tau protein in CSF)

8. Beta-amyloid deposits and/or decreased cerebral metabolism in the temporoparietal cortex on PET scan

Management of Alzheimer's disease

1. Acetylcholinesterase inhibitors (donepezil, tacrine, rivastigmine)

2. NMDA antagonist (i.e. Memantine)

2nd MC type of dementia; disease caused by chronic ischemia, multiple infarctions ("lacunar infarcts"), with HTN as an important risk factor

Vascular dementia

Presentation of vascular dementia

*depends on where ischemia is located

CORTICAL: forgetfulness, confusion, amnesia, executive difficulties, speech abnormality

SUBCORTICAL: motor deficits, gait abnormalities, urinary difficulties, personality changes

Confirmation of vascular dementia requires...

a history of stroke or evidence of a vascular cause for dementia detected by neuroimaging (CT or MRI)

Management of vascular dementia

Safety and supportive measures;

- smoking cessation

- BP control

- Cholesterol-lowering therapy

- Regulation of plasma glucose (90 to 150 mg/dL)

Rare type of dementia that presents mostly with personality changes such as apathy, disinhibition; another type may present with speech changes (aphasia)

Frontotemporal dementia

How does Lewy body dementia present?

EXTRAPYRAMIDAL SYMPTOMS: rigidity, bradykinesia, repeated falls, and gait instability; tremor late onset

COGNITIVE: fluctuating orientation, memory problems, deficits in attention/alertness, visuospatial and visuoconstructional abilities, visual hallucinations

+++++ autonomic dysfunction (syncope), REM sleep behavior disorder

Most common primary CNS tumor in adults

grade IV astrocytoma ("glioblastoma")

How does a CNS tumor usually present?

headaches (usually worse in late night/early AM, positional, worse with coughing/sneezing/straining); cranial nerve deficits, AMS, ataxia, vision changes, weakness

SYMPTOMS D/T INC ICP ---> headache, N/V, papilledema, drowsiness, stupor

Diagnosis of Astrocytoma

CT or MRI w/ contrast --> brain biopsy (imaging guided) with histopathology

Management of astrocytoma

1. Pilocytic (Grade I) - surgical excision

2. Diffuse (Grade II) - surgery (if tumor is accessible), radiation adjunct for unresectable tumors

3. Anaplastic (Grade III) - surgery --> radiation therapy +/- chemotherapy

4. Glioblastoma (Grade IV) - radiation + chemo

Cushing's Reflex/Triad

Intercranial pressure that pushes the brainstem and midbrain through the foramen magnum; classic triad of irregular respirations, hypertension & bradycardia

How does glioblastoma appear on neuroimaging?

nonhomogenous mass with a hypodense center and a variable ring of enhacement surrounded by edema; if it crosses corpus callosum "butterfly glioma"

Management of glioblastoma

surgical excision, radiotherapy and adjuvant chemotherapy

Benign tumors arising from the meninges; associated with neurofibromatosis, dural infection, etc.

meningiomas

Symptoms of meningiomas

most often asymptomatic; if symptoms, can have seizures, progressive spasticity, weakness/sensation loss; symptoms d/t increased ICP not as common!!!

Diagnosis of meningiomas

CT/MRI with contrast: intensity enhancing, well-defined lesion often attached to the dura (will eventually lead to brain biopsy)

Management of meningioma

observation (if small/asymptomatic) or surgical removal

Primary vs. secondary CNS lymphoma

Primary - variant of NHL, less common than secondary

Secondary - METS from another site, especially diffuse B cell lymphoma (90%) and Burkitt lymphoma (10%)

90% of CNS lymphoma patients have positive titers of...

EBV

Diagnosis and management of CNS lymphoma

DX: CT/MRI with contrast shows ring-enhancing lesion in deep white matter, biopsy via imaging; workup usually includes CT abdomen/pelvis, PET scan, bone marrow biopsy, slit lamp exam

TX: chemotherapy (methotrexate), radiation, corticosteroids

CNS tumor with histology that shows "fried egg shaped" tumor cells, chicken wire capillary pattern

oligodendroglioma

abnormal build-up of blood vessels in the skin or internal organs; make up 2% of all primary brain tumors

hemangioma

CNS tumor arising from blood vessel lining; benign, slow growing, well defined tumors, most often found in brainstem and cerebellum

hemangioblastoma

Dx and Tx of hemangioma/hemangioblastoma

DX: CT/MRI with contrast ---> brain biopsy

TX: surgical resection

DEFICITS: motor lower extremity > upper extremity, pain/light touch/temperature sensation, urinary continence

PRESERVED: proprioception, vibration, pressure

anterior cord syndrome

DEFICITS: motor upper extremity > lower extremity, pain/temperature deficit (upper > lower extremity), "shawl distribution"

PRESERVED: proprioception, vibration, pressure

central cord syndrome

DEFICITS: loss of proprioception and vibratory sense ONLY

PRESERVED: pain, light touch, no motor deficits

posterior cord syndrome

IPSILATERAL DEFICITS: motor, vibration, proprioception

CONTRALATERAL DEFICITS: pain, temperature

Brown Sequard syndrome

Transient episode of neurologic deficits caused by focal brain, spinal cord, or retinal ischemia without acute infarction; lasts < 24 hrs, most last 30-60 min

Transient Ischemic Attack (TIA)

*most commonly from emboli

Clinical presentation of TIA

INTERNAL CAROTID: monocular vision loss/temporary "lamp shade down on one eye," weakness in contralateral hand

ICA/MCA/ACA: cerebral hemisphere dysfunction (see "CVA" flashcards)

PCA: somatosensory deficits

VERTEBROBASILAR: gait and proprioception dysfunction, vertigo, N/V

Diagnosis of TIA

IMAGING - CT scan w/o contrast best initial test, followed by CTA/MRA, carotid doppler, echo (for emboli)

LABS: blood glucose, electrolytes, CBC, coag studies, ECG

Management of TIA

aspirin + clopidogrel; place in supine position, don't lower BP too quickly, modify reducible risk factors

*THROMBOLYTICS CI

Presentation of lacunar infarct(s)

1) pure motor MC (hemiplegia, hemiparesis)

2) ataxic hemiparesis and clumsiness

3) dysarthria (clumsy hand syndrome)

4) pure sensory loss (numbness, paresthesias)

Over 80% of patients who have lacunar infarct have a history of...

HTN

MCA ischemic stroke symptoms (MC, 70%)

- contralateral sensory/motor loss/hemiparesis (face, arm >>>> leg, foot)

- contralateral homonymous hemianopsia (gaze preference toward side of lesion)

- if on DOMINANT side (usually LEFT) ---> Broca or Wernicke aphasia, agraphia, no math comprehension

- if on NONDOMINANT side (usually RIGHT) -->

dysarthria, spatial defects, left-sided neglect, anosognosia, apraxia

ACA ischemic stroke symptoms (2%)

- contralateral sensory/motor loss/hemiparesis (leg foot >>>>>>>>>> upper extremity, so leads to abnormal gait)

- impaired judgment, confusion, personality changes

- urinary incontinence

- gaze preference toward side of lesion

*****face and speech preservation spared

PCA ischemic stroke symptoms

- visual hallucinations

- contralateral homonymous hemianopsia

- "crossed symptoms" ---> ipsilateral CN deficit, contralateral muscle weakness

- coma, drop attacks

Basilar artery ischemic stroke symptoms

cerebellar dysfunction, CN palsies, decreased vision, decreased sensation bilaterally

Vertebral artery ischemic stroke symptoms

vertigo, nystagmus, N/V, diplopia, ipsilateral ataxia

Diagnosis of CVA

1st ---> CT head w/o contrast (to try to see if there is bleed. THEN ---> CTA/MRA and possible other imaging/labs

Ischemic stroke management

1. Thrombolytics i.e. tPA (within 3 hours of symptom onset preferred)

2. Antiplatelet therapy (aspirin + clopidogrel)

Most common cause of spontaneous ICH

HTN

*most common location in basal ganglia