Neurology Clinical Medicine Exam

Alert (conscious)

appearance of wakefulness, awareness of the self and the environment

Lethargy

mild reduction in alertness

Obtundation

Moderate reduction in alertness, increased response time to stimuli

Stupor

Deep sleep, patient can be aroused only by vigorous (“pain”) and repetitive stimulation, returns to deep sleep when not continually stimulated (requires constant stimulation to stay awake)

Coma (unconscious)

sleep like appearance and behaviorally unresponsive to all external stimuli (unarousable unresponsiveness, eyes closed)

Cheyne-Stokes respiratory pattern

episodes of deep breathing alternate with periods of apnea

• bihemispheric or diencephalic disease; metabolic disorders

Hyperventilation respiratory pattern

brainstem tegmentum (central posterior part of brain stem)

Apneustic breathing respiratory pattern

prominent end-respiratory pauses

• damage at pontine level; basilar artery occlusion

Atactic breathing respiratory pattern

completely irregular pattern of breathing

• lower pontine tegmetnum and medulla damage

Trigeminal Neuralgia pathology

the trigeminal nerve supplies sensation to the face and sensory/motor fxn for muscles of mastication (masseter and temporalis)

V1- Opthalmic

V2- Maxillary

V3- Mandibular

Trigeminal neuralgia location

• Most common: compression of the trigeminal nerve by inter cranial artery

• Less common: venous compression, tumor, AV malformation, aneurysm, MS plaque

Trigeminal neuralgia

• Risks: aging, women, MS pts at a younger age (suspect if less than 40)

• S/S: lancinating face pain, pain has triggers (touch, movement, eating), pt holding face, usually unilateral, can have autonomic symptoms (lacrimation, conjunctival injection, rhinnorhea)

• Dx: clinical (hx and PE), bilateral= red flag and needs further testing

Trigeminal neuralgia tx

• 1st line: Anti-seizure- Oxcarbazepine (Trileptal) or carbamazepine (Tegretol)

• Alternative: phenobarbital, lamotrigine (Lamictal), gabapentin (Neurontin)

• Referral for surgical decompression in refractory cases

Herpes zoster (shingles) when occurring at the trigeminal nerve is the MCC of

post herpetic neuralgia

Types of herpetic neuralgia

• Acute: pain preceding or accompanying rash or eruption, up to 30 days

• Subacute: continues after rash is gone but goes away before 4 months

• Post: pain persists longer than 4 months after infection

Post herpetic neuralgia

• Risks: herpes zoster infection, elderly/immunocompromised, severity/duration of rash, V1 infection

• S/S: burning/pruritic pain, constant or intermittent, allodynia, sensory changes

Post herpetic neuralgia treatment

• 1st line: gabapentinoids

  • Gabapentin (Neurontin)

  • Pregabalin (Lyrica)

• 2nd line: TCAs

  • Amitriptyline (Elavil)

• Can use topical application of capsaicin cream or topical lidocaine

Prevention of post herpetic neuralgia

• Tx of HSV with antivirals within 72 hours of rash eruption

• Zoster vaccine

Glossopharyngeal neuralgia

• pain in distribution of the glossopharyngeal nerve and auricular/pharyngeal branch of the vagus nerve

  • causes a pain in the throat, tonsillar fossa, ear/back tongue

• S/S: severe/shooting pain, precipitated by chewing swallowing yawning, and pain associated syncope

• Dx: clinical and PE

• Tx

  • 1st line: oxcarbazepine, carbamazepine

  • Sx in refractory cases

Stroke syndrome of the anterior cerebral artery

• contralateral foot and leg

• gait

• bladder incontinence

• AMS (frontal lobe)

Stroke syndrome of the middle cerebral artery

• MC location of stroke

• contralateral arm and face aphasia

• Non dominant hemisphere: speech and comprehension preserved, unilateral neglect or constructional and visual spacial deficits

• Broca’s aphasia (superior): expressive

• Wernicke’s aphasia (inferior): receptive

Stroke syndrome of the posterior cerebral artery

homonymous hemianopsia (loss of half of the field of view on the same side in both eyes), cortical blindness, and loss of memory

• Alexia without agraphia

• Thalamic pain syndrome

Vertebrobasilar artery symptoms by distribution

diplopia, vertigo, nystagmus, syncope, and nausea

Head CT criteria for concussion/head injury

• GCS lesser than 15

• Focal neurological deficit

• Seizure

• Coagulopathy

• 65+

• skull fracture

• persistent N/V

• retrograde amnesia more than 30 mins

• Intoxication (inaccurate exam)

• High mechanism of trauma injury

Atypical face pain

Pain without typical features of trigeminal neuralgia; constant often burning pain that may have a restricted distribution at its onset but spreads to rest of face on affected side and sometimes involves the other side

• Risks: Middle aged women (depressed)

• Dx: Clinical and PE

• Tx: Simple analgesics (NSAID), TCA’s (Elavil- Amytriptyline), carbamazepine, oxcarbamazpine

Bell’s Palsy (aka Facial nerve palsy CNVII)

Idiopathic; swelling of the facial nerve due to an immune or viral disorder; various other disorders include diabetes, Lyme dz, sarcoidosis

• S/S: abrupt facial paresis, face is stiff and pulled to one side, difficulty eating, fine facial movement, disturbance of taste

• Tx: Usually watch/wait, can do a prednisone taper (controversial)

Primary headache

headache with no underlying medical condition causing it

Secondary headache

headache caused by a pre-existing medical condition

Migrane headache pathology

Altered central neuronal processing and involvement of the trigeminovascular system which causes inflammation of cranial vessels and dura mater which leads to increased sensitivity of the nervous system, genetic factors, roles of the NT CGRP-calcitonin gene related peptide involvement in the brainstem and the hypothalamus

• Risks: females, increase levels of CGRP, triggers (red wine, skipping meals, sleep deprivation, hormones, stress, foods)

• S/S: headache (unilateral, frontotemporal, pulsatile/throbbing), N/V, photophobia, sonophobia, osmophobia

Prodrome of migraine

sensation migraine is coming; change in mood, neck pain, food cravings, loss of appetite, nausea

Aura of migraine

temporary neurologic disturbance affecting sensation, balance, muscle coordination, speech and vision

• right before a migraine (minutes)

Migraine equivalent (migraine variant)

patient has aura but no headache

Basilar artery migraine (Migraine with brainstem aura) (migraine variant)

blindness/visual disturbances in both visual fields, dysarthria/disequilibruim/tinnitus/perioral parathesia, unilateral headache, N/V

• sort of resembles a stroke

Opthalmoplegic medicine (migraine variant)

headache, eye pain, CN III (opthalmic) palsy

Hemiplegic migraine (migraine variant)

causes weakness on one side of the body

Migraine headache Dx and Tx

• Dx: clinical (pHx, fHx, pattern)

• Tx: Lifestyle modification combined with medication

Abortive Tx for migraines

• Acute, nonspecific

  • Analgesics (ASA, NSAIDs, Caffeine)

  • Anti-nausea (Ondansetron- Zofran, Prochlorperazine- Compazine, Metocloperamide- Regian)

• Acute, specific

  • Ergotamine

  • Triptans “tan”

  • CGRP antagonists (Ubrelvy, Nurtec, Zavpret)

Prophylaxis Tx for Migraines

Migraine more than 2-3 month, take abortive meds with breakthrough headache

• Anti-epileptics: Topiramate (Topamax), Valproic acid (Depakote)

• CV: Candesartan (ARB), Propanolol (B-Blocker), Verapamil (CCB)

• Antidepressants: Amitriptyline- Elavil (TCA), Venlafaxine- Effextor (SNRI)

• Monoclonal Aby against CGRP: Erenumab (Aimovig), Fremanezumab (Ajovy), Galcanezumab (Emgality)

• Adjunct: Botox

Cluster headache (primary headache)

Attacks of severe unilateral pain in the orbit or surrounding areas of both lasting 5-180 minutes, recurring once every other day to 8 times per day

• Risks: middle aged males, alcohol, smoking, stress, nitroglycerin, chocolate/food

• S/S: ipsilateral (on headache side)- conjunctival injection, lacrimation, nasal congestion, ptosis, miosis, facial sweating

• Tx: Avoidance of triggers

  • Abortive

    • Sumatriptan (Triptan), Ergotamine

    • 100% high flow O2

    • high dose prednisone during headaches

    • intranasal lidocaine

  • Prophylaxis

    • Antidepressants: TCAs and SSRIs

    • Anticonvulsant: Lithium and Topamax

    • CCB- Verapamil

Chronic paroxysmal hemicrania

Attacks largely with some characteristics of pain and associated symptoms as cluster headaches but are shorter lasting and more frequent

• Risks: females

• Tx: oral NSAIDs, indomethacin

Cluster headaches versus chronic paroxysmal hemicrania

Cluster Headaches

• male, longer with less frequency

Chronic Paroxysmal Hemicrania

• female, shorter with more frequency

Tension headaches (primary)

Recurrent episodes of headache lasting minutes→days

• S/S: mild/moderate intensity, bilateral (widespread), photo/phonophobia, no physical aggravation

  • pain in upper back, neck, back of head, ears, jaw, love the eyes

  • chronic= more than 15 days per month over 6 months

• Dx: Clinical/PE

• Tx: Lifestyle modification

  • Acute/Abortive: NSAIDs

  • Chronic/Preventative: TCA/SSRIs

Post-Traumatic Headache (secondary)

Traumatic event, headache arises within 1-2 days of the event

• S/S: constant, dull headache, throbbing, N/V, scintillating scotomas

• Tx: Analgesics, severe may need future prophylaxis

Primary cough headache (secondary headache)

severe head pain caused by cough (strain, laugh, sneeze), a few minutes or less

• Risks: intercranial lesions, tumors

• Dx: CT/MRI in all pts

• Tx: indomethacin 75-150 mg orally

Medication overuse headaches

Chronic pain or severe headaches unresponsive to medication

• >10 day/month: Ergotamine, Triptans, Butabital, Opioids

• >15 day/month: Acetaminophen, ASA, NSAIDs

Giant cell arteritis (Temporal Arteritis)

Immune response leading to chronic inflammation and arterial wall destruction

• Risks: >50 yo, women, polyalgia rheumatica

• S/S: new onset temporal headache, scalp tenderness, jaw claudication, blurry vision, diplopia, amaurosis fugax

• Dx: high ESR/CRP, temporal artery biopsy

• Tx: high dose corticosteroids (IV methylprednisone)

Pseudotumor Cerebri (Idiopathic Intercranial Hypertension)

Idiopathic, high pressure with no obvious cause

• Risks: overweight, women 20-44

  • Rare: venous sinus thrombosis, mastoiditis, COPD, CT disorder

• S/S: headache, diplopia, abducens VI dysfunction, pulsatile tinnitus

• Dx: CT/MRI of brain, LP (normal fluid, high pressure)

• Tx: weight loss, med to decreased CSF pressure (acetazolamide, topiramate-Topamax)

  • Refractory= Sx for VP shunt

Red flags for headache

• First or worst headache

• >55 yo

• AMS

• change in HA pattern

• Instant onset (subarachnoid headache)

• Temporal artery tenderness (GCA)

• Persistent HA in child

• Recent head injury

• Suspected meningitis

  • S/S: fever, nuchal rigidity, HTN, weight loss, poor arterial pulse papilledema, lymphadenopathy

Causes of abrupt coma

subarachnoid hemorrhage, brainstem stroke, intracerebral hemorrhage

Causes of slow onset coma

structural mass/lesion, metabolic, agitated delirium

Coma

state of unarousable unresponsiveness, reflex movements and posturing may be present; caused by injury to bilateral cerebral hemispheres or the brainstem

Vegetative state

occur after serve bilateral hemispheric injury after recovery from a coma

• unawareness of self or environment

• preserved sleep/wake cycles, complete/partial preservation of hypothalamic and brain stem autonomic fxn

Permanent vegetative state (aka: akinetic mutism, apallic state, coma vigil)

• 3 mo after non-TBI or 1 yr after TBI

• most pts will die within months→ years, there are some rare cases of spontaneous recovery

Minimally conscious state

Severely impaired consciousness w/ minimal but definite behavioral evidence of awareness

• inconsistent consciousness, some degree of functional recovery of behaviors

• after 12 months, likely to be a permanent disability

Locked-In syndrome

(brain is ok, loss of motor activity) state of de-efferenation w/ quadriplegia and loss of lower CN function but preservation of sensation, cognition, and eye movements

• Risks: acute destructive lesions (infarction, hemorrhage, demyelination, encephalitis)

• Fully aware of surroundings, prognosis is poor

Catatonia

psychiatric disease; behavioral disturbances, unresponsiveness

GCS- Glasgow Coma Scale

E (eye) V (verbal) M (motor)- 4, 5, 6

• Mild: 13-15

• Moderate: 9-12

• Severe: less than 8

Upon presentation of altered consciousness

• ABCs (airway, lines), blood (glucose, lytes, calcium), ABGs, liver and kidney function, toxicology report

• Dextrose, naloxone, thiamine IV (banana bag)

• URGENT non-contrast head CT

Painful stimuli- examining impaired consciousness

• Withdrawal: sensory and motor pathways intact

• Unilateral absence: corticospinal lesion

• Bilateral absence: brainstem lesion, bilateral pyramidal tract lesion, psychogenic unresponsiveness

• Decorticate (flexor): lesions of the internal capsule and rostral cerebral peduncle

• Decerebrate (extensor): dysfunction of midbrain and rostal pons

Oculocephalic Reflex- examining impaired consciousness

• Normal: doll eyes (eyes move opposite of head turn)

• Brainstem lesion: reflex absent

Caloric stimulation- examining impaired consciousness

Irrigation with ice water

• Unconscious with intact brainstem: eyes deviate towards irrigated side

• Impaired: response is perverted or fixed

Brain death

irreversibly comatose patient lost all brainstem reflex responses for longer than 6 hours after last medication was given

• pupillary, corneal, oculovestibular, oculocephalic, oropharyngeal, and respiratory reflexes

• Ongoing seizure or decorticate posturing is NOT brain death

Tests for brain death

1. Apnea test: paCO2 60 mmHg< or increase in 20 mmHg from baseline (demonstrating ability to respirate)

2. EEG: confirmed dx; absence of brain activity

Mild TBI (concussion)

alteration in mental status caused by trauma with or without loss of consciousness

• Risks: athletics, risky behaviors

• S/S: (varies greatly) vacant state, delayed verbal response, slurred speech, inability to concentrate, disorientation, memory deficits, headache, N/V

• Dx: clinical and Hx, examine for other injuries (lacerations)

  • progressive decline/change: urgent imaging and neurosurgery consult

• Tx: Home with caregiver if no CT needed

  • If CT: admit until CT normal, CGS is 15, and no seizures or bleeding occur only if caregiver is present

  • promote resolution of concussive symptoms before pt should return to athletics and ample time off of occupation

Post concussive syndrome (complication of mild TBI)

S/S persist weeks→ months after the injury

• MRI and individualized tx

Second impact syndrome (complication of mild TBI)

Recurrent concussion after not healing the first one

• can cause fatal cerebral edema

Chronic Traumatic Encephalopathy- CTE (complication of mild TBI)

Very common in pro athletes

• repeated concussions cause cumulative neuropsychological deficits

• Prevention: helmets, athletic safety, safe work settings, minimize risk behavior

Moderate/Severe TBI

start with CT/MRI to evaluate bleeding (SDH, EH)

• Tx: ABCs in the ED, scans, consult neurosurgery, consult rehab

Basilar skull fracture

Fracture through petrous portion of temporal bone

• S/S: raccoon sign (bruising around the eyes), blood in the external acoustic meatus (battle sign), CSF leak from the ears and nose, CN palsy (1-8), head and neck immobilized

• STAT neuro surgery consult

Epidural hematoma

acute collection of blood b/t the inner table of skull and dura; MC due to the middle meningeal artery being lacerated through a skull fracture

• S/S: loss of consciousness after high impact head injury, may have lucid stage then deteriorate quickly, fixed/dilated pupil on affected side, contralateral hemiparesis on opposite side, high pressure bleed can cause herniation

• Dx: CT head- “football shaped”

• Tx: STAT consult for neurosurgery

Epidural hematoma vs Subdural hematoma

Epidural hematoma

• arterial

• more emergent/acute

Subdural hematoma

• venous

• can be more chronic/slow

Subdural hematoma

Collection of venous blood between the dura and the arachnoid; tears of the bridging artery

• common accelerate-decelerate (slosh)

• elderly and alcohol users are more susceptible

• Dx: CT of head- crescent shaped

Acute subdural hematoma

within 14 days of injury

• most have s/s within 24 hours

• S/S: headache, N/V, weakness, dizzy

Chronic subdural hematoma

>2 weeks after injury

• may not recall trauma (elderly fall)

• insidious s/s

CNS tumors

brain and spinal cord tumors

• Primary: originate from CNS tissue (neuroglia, neurons, cells of blood vessels, CT, meninges)

• Secondary: metastatic from other tissue

• Infiltrative (spread into tissue) vs space occupying (distinct mass)

• WHO classification: Grade I (low proliferative)→ Grade 4 (aggressive malignant tumor)

Primary intracranial tumors

1/3 meningioma, ¼ gliomas, pituitary adenoma, neurofibroma

• 1/3 are malignant (85% glioma, low (astrocytoma)→ high (glioblastoma) grade), lymphoma, metastatic

• HIV/immunocompromised are at risk for PCNSL- primary central nervous system lymphoma

Meningioma (1/3 of primary intracranial tumors)- space occupying

tumor of the meninges; originates from dura or arachnoid, size varies greatly

• Risks: age

• S/S: vary with location and size, usually benign, possible calcification and bone erosion

• Dx: Head CT, x-ray for bone erosion and calcification

• Tx: surgical

• Good prognosis with complete removal

Pyramids decussate in the

lower medulla

• damage above: contralateral deficit

• damage below: ipsilateral deficit

Glioblastoma Multiforme (GBM)

(most common malignant primary brain tumor, high grade glioma); tumor of neuroepithelial origin that includes the surrounding parenchyma

• Risks: non-hispanic whites, slight male predominance

• S/S: high ICP, nonspecific

• Dx: CT/MRI

• Tx: surgical removal, radiation with tezolamide (Temodar)

Astrocytoma

(low grade glioma); tumor of neuroepithelial origin; similar to GBM but slow growing

• Tx: surgical resection, radiation and temozolamide in in higher grade tumors

Primary CNS lymphoma

rare subtype of non-Hodgkin lymphoma

• Risks: AIDS, immunodeficiency

• S/S: focal deficits, disturbance of cognition and consciousness

• Tx: high dose methotrexate and corticosteroids with radiation

Frontal lobe lesion

intellectual decline, slow mental activity, personality change, anosmia (CN I),

• expressive aphasia (Broca’s area)

Temporal lobe lesion

Olfactory/gustatory hallucination, decreased music perception

• deja vu

• lip smacking

• Wernicke’s- receptive aphasia

Parietal lobe lesion

contralateral disturbances of sensation, astereogenesis, thalamic syndrome (extensive lesion)

Occipital lobe lesion

visual hallucination, visual agnosia for objects and color, inability to identify familiar face, inability to interpret a scene

Brainstem and cerebellar lesions

CN palsies, ataxis, nystagmus, vertigo, diplopia

Herniation syndromes

if pressure is increased in one compartment, brain tissue may herniate into a compartment with lower pressure

• MC is the temporal lobe through the tensorial hiatus

• can lead to apnea, circulatory collapse and death

Arteriography in lesions (aka angiography)

• pre surgical embolization of highly vascular tumors

• distinguish adenoma vs aneurysm

Labs and studies for lesions

• Glial neoplasms require biopsy and molecular analysis (WHO grade)

• NO LUMBAR PUNCTURE

Tx for lesions

• Benign= serial annual imaging

• Malignant/symptomatic= complete surgical removal or debulking

• Sx shunt for hydrocephalus

• corticosteroids can reduce cerebral edema

• Herniation- IV dexamethasone and IV mannitol

Metastatic Intercranial tumors

MC source is lung cancer (others include breast, kidney, skin, GI); most are supratentorial (located in the cerebrum)

• Dx: MRI with contrast

• Tx: surgical resection, radiation, chemo, immunotherapy

  • corticosteroids for cerebral edema

Leptomeningeal Carcinomatosis (metastatic tumor)

From breast, lung, lymphoma, and leukemia; cancer in the leptomeninges (membranes of Pia and arachnoid mater)

• S/S: multifocal neurologic deficits

• Dx: MRI with contrast, CSF cytology (LP)

• Tx: intrathecal chemotherapy (directly into the CSF of the brain or spinal cord) and radiation

Primary CNS lymphoma (metastatic tumor)

Rare subtype of diffuse large B-Cell lymphoma

• Risks: AIDs, immunosuppressed, EBV infection

• S/S: disturbance in cognition or consciousness, focal motor or sensory deficits, aphasia, seizures, CN neuropathy

• Dx: often confused with cerebral toxoplasmosis (common AIDs complication) on imaging- trial tx then repeat scan

  • Biopsy if no improvement

• Tx: Corticosteroids, high-dose methotrexate, radiation

Intramedullary spinal tumors

10% of spinal tumors

• Ependyoma MC

Extramedullary spinal tumors

Can be extra/intra dural

• Neurofibromas and meningiomas (benign)- intra or extradural

• Cancer metastases, lymphomatous, leukemic deposits- usually extradural

Spinal metastases is due to

prostate, breast, lung, and kidney cancers

Spinal tumors

• S/S: insidious, localized/achey/dull, worse with cough, sneeze, strain or lying, motor weakness, radiculopathy, paresthesia, numbness

  • LMN: dermatomal sensation change at level of lesion

  • UMN: sensory below level of lesion

• Dx: MRA or CT myelography and CSF will have high protein with normal cells and glucose

• Tx:

  • Intramedullary: decompression and sx excision with radiation

  • Epidural spinal metastases: Sx, radiation, or both

    • high dose dexamethasone for swelling

    • radiation alone for radiosensitive tumor

TIA pathology

blood flow to the brain is temporarily blocked

• CC: blood clots, narrow blood vessels, restricted blood flow of the carotid artery

• focal neurological deficit of acute onset, clinical deficits resolves completely within 24 hours

TIA risk factors that lead to a subsequent stroke

RF of cardiovascular dz, can lead to future stroke

• Subsequent stroke: highest in the first 3 months (1st month especially) and 48 hours after TIA

• Carotid TIA is MC to lead to stroke than a vertebrobasilar TIA

• Other risks include pts older than 60, diabetics, TIA longer than 10 minutes

TIA Etiology

• Embolic

  • Cardiac causes: PFO, A-fib, heart failure, endocarditis, atrial myxoma, MI, mural thrombi

• Abnormalities of blood vessels

• Hematologic

• Subclavian steal syndrome