543 - Block Exam 1 (Neuro)

Trigeminal neuralgia

definition:

unilateral electric shock-like pains of the face

etiology:

compression of trigeminal nerve root CN V (V2, V3)

↳MC few mm entry into pons

types:

classic: related to vascular compression

secondary: underlying disease

clinical manifestations:

shock/stabbing pains

↳triggered by chewing/brushing teeth

tx:

-1st line: carbamazepine or oxcarbazepine

Bell's Palsy

definition:

unilateral facial paralysis & weakness (CN VII)

etiology:

-viral (HSV/Zoster) MC

clinical manifestations:

-inability to wrinkle brow

-drooping eyelid; inability to close eye

-inability to puff cheeks

-dropping mouth; inability to smile

dx:

-House-Brackmann grading scale

tx:

prednisone

Tension HA

definition:

MC type of HA

types:

episodic: occurs randomly

chronic: cyclical pattern

etiology:

-multifactoral; muscular and psychogenic

clinical manifestations:

-non-pulsatile (bilateral) band distribution

-no focal neuro sx

tx:

-tylenol or NSAIDs

Migraine

types:

classic (aura)

common (no aura)

etiology:

-family hx; autosomal dominant

clinical manifestations:

classic:

↳prodromal aura < 60 mins

↳visual sx MC; Scintillating scotoma

↳throbbing, pulsatile

↳N/V

common:

↳unilateral & localized pain MC frontotemporal & ocular areas

dx:

1st line: CT

tx:

mild: NSAIDs, tylenol, ASA

moderate: triptans

Cluster HA

definition:

idiopathic and unilateral HA often causing periorbital/temporal pain

etiology:

episodic: 2 cluster phases lasting 7 days - 1 year

chronic: > 1/year w/out remission

clinical manifestations:

-sudden onset of severe unilateral periorbital/temporal pain

-pain lasts < 2 hrs which spontaneously resolves

tx:

-1st line active: 100% O2 6-10 L via NRM

-1st line prophylaxis: verapamil

Post-Traumatic HA

etiology:

HA from trauma to head or neck

↳typically from whiplash (MVC) or TBI

dx:

-mental status evaluation

tx:

-tylenol and ibuprofen

Viral meningitis

definition:

inflammation of the leptomeninges as a manifestation of CNS infection

etiology:

HSV 2 MC cause

clinical manifestions:

-parotitis THEN triad:

↳fever

↳vomiting

↳HA

-pericarditis, myocarditis, conjunctivitis, HFMD

-Morbilliform rash

dx:

LP & CT

-LP first if no focal neuro sx

-CT head first if hx mass lesion, stroke, AMS

cell count: < 2,000 lymphocytes

glucose: normal

protein: < 150

tx:

empiric ampicillin + ceftriaxone + acyclovir

Bacterial meningitis

etiology:

-adults: strep pneumonia MC

-children: H. influenza MC

-neonates: group B strep MC

clinical manifestations:

triad

↳fever

↳HA + AMS

↳nuchal rigidity (stiff neck)

-Brudzinski's & Kernig's signs

-bulging fontanelles

dx:

LP & CT

-LP first if no focal neuro sx

-CT head first if hx mass lesion, stroke, AMS

cell count: > 1000 - 2000 neutrophils

glucose: < 45 (low)

protein: > 250

tx:

ages 0-4 wks: ampicillin + cefotaxime

age 1 mo - 50 YO: vanco + ceftriaxone

age > 50 YO: vanco + ampicillin + ceftriaxone

Brain abscess

definition:

focal purulent collection in brain parenchyma

etiology:

-streptococcus MC cause

clinical manifestations:

-HA MC complaint

dx:

-MRI brain w/ contrast study of choice

↳round-like encapsulated mass w/ central necrosis and surrounding edema

-surgical removal and bx

tx:

-neurosurgical aspiration

-ceftriaxone

Normal Pressure Hydrocephalus

defintion:

dilation of the cerebral ventricles w/ normal opening pressures on LP

etiology:

-impaired CSF absorption after CNS injury

-MC elderly

clinical manifestation:

triad:

↳dementia/cognitive dysfunction (wacky)

↳gait disturbance (wobbly) earliest sx

↳urinary incontinence (wet)

dx:

MRI

tx:

tx of choice: ventriculoperitoneal shunt

Encephalitis

definition:

infection of brain parenchyma

etiology:

HSV and arborviruses MC

neonates: HFE HSV-1 MC

adults & peds: HFE HSV-2 MC

clinical manifestations:

-photophobia**

-vesicular lesions**

-hemiparesis

-focal seizures

dx:

LP & MRI

-LP; viral cultures & PCR for HSV

↳perform first if no focal sx, AMS or ↑ ICP

↳↑ WBC

↳↑ protein

↳ nml glucose

-MRI w/ and w/out contrast

↳ cortical blurring

tx:

acyclovir

Parkinson's disease

definition:

neurodegenerative disease which results in idiopathic dopamine depletion

etiology:

genetic link; autosomal dominant (MC)

↳ LRRK2 or SNCA

clinical manifestations:

triad

↳ tremor

↳ bradykinesia

↳ rigidity (cogwheel)

hallmark

↳ loss of pigmented dopaminergic neurons of substantia nigra pars compacta

↳ (+) Lewy bodies

-visual hallucinations MC psychotic sx

-stooped posture

-shuffling gait

dx:

(+) Lewy body on bx

tx:

-1st line: levodopa-carbidopa

↳Let's PARK the Car

Huntington's disease

definition:

-incurable, adult-onset inherited disorder

etiology:

-defective CAG; autosomal dominant

clinical manifestations:

triad

↳ behavioral disturbances

↳ extrapyramidal movement

↳ dementia

-optokinetic nystagmus

dx:

genetic testing (CAG allele)

tx:

chorea: tetrabenazine 1st line

Essential tremor

definition:

rhythmic, oscillatory movement

etiology:

-genetic

clinical manifestations:

-tremors which worsens w/ intentional movement (stress)

-tremor improves w/ small amount of EtOH & rest

tx:

if needed, propanolol 1st line

Tourette's syndrome

definition:

neuro disorder manifested by sudden, brief, intermittent motor and phonic tics

etiology:

excess dopamine + GABA deficiency

clinical manifestations:

-hallmark: tics

dx:

-motor tics + phonic tics

PLUS

-onset of tics before 21 YO

tx:

1st line: habit reversal

Benign neoplasm (Brain)

definition:

Neoplasm arising from meninges

etiology:

-meningioma

-Schwannoma

-pituitary adenoma

dx:

study of choice: MRI brain w/ & w/o gadolinium contrast

Meningioma

definition:

-slow growing tumor that forms on meninges

etiology:

childhood exposure to dx head CTs ↑ risk of brain tumor

clinical manifestations:

-underlying parenchyma: (+) Hoffman & Babinski sign

-dominant L parietal lobe: Gerstmann syndrome

-non dominant R parietal lobe: tactile & visual extinction

-occipital: congruent homonymous hemaniopsia

dx:

study of choice: MRI brain w/ & w/o gadolinium contrast

tx:

surgical resection

Hemangioma/Hemangioblastoma

etiology:

-hemangioma: buildup of blood vessels IN skin/internal organs

-hemangioblastoma: growth from stromal cells blood vessel lining

-MC posterior fossa

-Von Hippel-Lindau disease

clinical manifestations:

-retinal hemangioblastoma: loss of visual acuity

dx:

-bx

-CT or MRI w/contrast

tx:

-surgical resection

-radiation if tumor attached to brainstem

Pilocytic Astrocytoma

definition:

Benign tumor of astrocytes which originates in glial cells

types:

-diffuse (II): low grade, slow growth

-anaplastic (III): rare & aggressive

-glioblastoma (IV): MC primary CNS tumor in adults

-subependymal giant cell astrocytoma: ventricular tumors associated with/ tuberculosis sclerosis

clinical manifestations:

-PE findings depends on tumor location

dx:

-study of choice: MRI w/ & w/out contrast

↳Grade I & II: non-enhancing

↳Grade III & IV: enhancing

-bx

↳grade I: sacs of fluid

↳grade II: microcysts

↳grade III: tentacle-like projections

↳grade IV: cystic material & calcium deposits

tx:

grade I & II: surgical excision

grade III & IV: surgical excision + radiation tx

Glioblastoma

definition:

highly malignant (grade IV) astrocytoma

↳most lethal & MC primary brain tumor

etiology:

-primary GBM MC (60%)

-secondary (40%)

-arises from poorly differentiated neoplastic astrocytes

clinical manifestations:

-HA**

-focal neuro deficit MC

-focal seizure MC in primary

dx:

-fundoscopy: papilledema

-confirmatory & essential: bx

↳malignant astrocytes + necrotizing hemorrhagic center

-initial study of choice: MRI brain w/ & w/o contrast

↳butterfly gliomas

tx:

maximal surgical resection

-for seizures: keppra 1st line

Medulloblastoma

definition:

-embryonal tumor originating from fetal brain cells

-MC malignant peds brain tumor

etiology:

-wingless/integrated signaling pathway (WNT)

-SHH protein

clinical manifestations:

-sx of ↑ ICP & cerebellar dysfunction

-truncal/gait ataxia

dx:

-genetic testing for WNT & SHH

-study of choice: MRI brain w/ & w/o contrast

-CT: classic finding is hyperdense mass

tx:

-surgical resection, radiation, chemo

Retinoblastoma

definition:

-rare malignant tumor of retina that affects peds

etiology:

-MC primary ocular cancer of peds

-genetic: germline RB1 gene mutation

-nongenetic: somatic RB1 gene mutation

clinical manifestation:

-vision loss MC complaint

↳leukocoria MC initial presentation

↳strabismus

dx:

-ophthalmic evaluation under anesthesia

↳chalky, off-white retinal mass

-ocular US

↳echogenic soft tissue mass

-study of choice: MRI brain & orbit

-bx

↳Flexner-Wintersteiner rosettes classic finding

↳spoke & wheel shaped cell formation

tx:

-1st line: focal techniques

↳cryotherapy

↳laser photoablation

↳enucleation

↳radioactive plaques

Oligodendroglioma

etiology:

-commonly found in white matter and brain cortex

-MC frontal & temporal lobe

-chromosomal abnormality: 1p & 19q

clinical manifestations:

-focal neuro deficits

↳corticospinal defects

↳spinocerebellar defects

dx:

-confirmatory: bx

↳fried-egg apperance/chicken wire vessels

-study of choice: MRI brain w/ & w/o contrast

↳calcifications on enhancing mass

tx:

standard & intial: surgical resection

Ependymoma

etiology:

-arises from ependymal cells in CNS

↳MC in children

↳MC in 4th ventricle, spinal cord, & medulla

clinical manifestations:

supratentorial (MC in peds)

↳increased head size

infratentorial (MC in adults)

↳N/V & HA

dx:

-confirmatory: bx

↳perivascular pseudorosettes

-study of choice: MRI brain w/ & w/o contrast

tx:

mainstay: surgical resection & adjuvant RT

CNS lymphoma

definition:

-malignant cells form in the lymph tissue in CNS

etiology:

-secondary MC (mets)

-EBV (+) in 90% of cases

clinical manifestations:

-immunocompetent: focal sx indicative of mass lesion

-immunoCOMPROMISED: encephalopathy

dx:

-study of choice: MRI brain w/ contrast

↳if immunocompromised, test for toxoplasma and LP

-SPECT

tx:

-1st line: methotrexate + folinic acid

Metastatic brain cancer

definition:

MC intracranial malignancy in adults

↳MC hematogenous spread found at gray-white matter junction

etiology:

-lung cancer

-breast cancer

-kidney cancer

-colorectal cancer

-melanoma

dx:

-confirmatory: brain bx

-study of choice: MRI brain w/ contrast

↳multiple lesions

↳lesions @ gray-white matter junction

↳vasogenic edema

Delirium

definition:

-acute, abrupt, transient confused state

etiology

-identifiable cause; drugs, infections, brain disorders

clinical manifestations:

-disturbance in attention & awareness

-irritability, anxiety

dx:

-MMSE

-investigate medical etiologies

tx:

tx underlying cause

Dementia

definition:

-short-term memory impairment PLUS either

↳aphasia/apraxia/agnosia/abstract thinking

-MC Alzheimer's & Lewy body dementia

Alzheimer's Disease

definition:

-neurodegenerative disorder characterized by cognitive decline

etiology:

-age & Down's syndrome (trisomy 21)

-neuritic plaques (extracellular)

-neurofibrillary tangles (intracellular)

-cholinergic system degeneration

clinical manifestations

-initial sx: short term memory loss

dx:

-montreal cognitive assessment (MoCA)

-study of choice: MRI brain

↳volume loss in hippocampus & temporal lobe

tx:

donepezil

Diffuse Lewy Body Disease

definition:

-progressive dementia

etiology:

abnormal alpha synuclein proteins

clinical manifestations:

-fluctuating cognition

-visual hallucinations

-Parkinsonism

-REM sleep disorder

dx:

bx: cortical Lewy bodies

tx:

-tx of Parkinsonism may exacerbate neuropsychiatric sx

Vascular dementia

definition:

-sudden decline in functions w/ stepwise progression of sx

etiology:

-chronic ischemia and multiple infarcts

-HTN biggest risk factor

clinical manifestations:

-cortical: executive dysfunction

-subcortical: focal motor deficit

dx:

study of choice: MRI brain

↳white matter lesions, cortical/subcortical infarcts

tx:

donepezil

Frontotemporal dementia (Pick's disease)

definition:

-localized brain degeneration of frontotemporal lob

clinical manifestations:

-changes in social behavior, personality, and language

↳inappropriate sexual remarks, invading personal space, urinating in public

dx:

bx: pick bodies

Multiple Sclerosis

definition:

-immune-mediated inflammatory demyelinating disease of CNS

-relapsing-remitting MC type

etiology:

idiopathic origin

clinical manifestations:

-sensory deficits (pain, paresthesia)

-motor deficits (weakness, gait, balance)

-trigeminal neuralgia

-optic neuritis and/or diplopia

↳Marcus Gunn pupil

-Upward babinski

-Lhermitte's sign

dx:

-LP; ↑ IgG & oligoclonal bands

-study of choice: MRI

↳hallmark: hyperintense white matter plaque

tx:

1st line: high dose glucocorticoid (methylprednisone)

1st line for spasticity: baclofen

Myasthenia gravis

definition:

-autoimmune disorder characterized by fatigable weakness of skeletal muscles

etiology:

-MC associated to thymus gland

↳HLA:B8 and DR3

clinical manifestations:

hallmark: fluctuating skeletal muscle weakness

↳ocular

↳bulbar (oropharyngeal)

↳limb

↳respiratory

dx:

-AChR test

-MuSK Ab

-RF/ANA

+ chest imaging

tx:

1st line: Pyridostigmine

crisis tx: IVIg + plasmapheresis

Cerebral palsy

definition:

Heterogeneous group of conditions involving permanent, nonprogressive motor dysfunction which affects posture +/- movement

etiology:

injury or abnormal brain development in immature brain

clinical manifestations:

-MC abnormal muscle tone

-hypotonic to spastic tone

dx:

-hx of gross motor developmental delay in 1st year of life

-MRI

tx:

multidisciplinary team; maximize functional abilities & independence

Amyotrophic lateral sclerosis (ALS/Lou Gehrig's disease)

definition:

Progressive, incurable, neurodegenerative disorder which causes death of neurons of voluntary muscles

etiology: unclear

clinical manifestations:

-Asymmetric limb weakness MC sx

-upper extremity: begins w/ hand weakness

-lower extremity: begins w/ foot drop

-lower motor neuron sx:

↳weakness, atrophy, fasiculations

-upper motor neuron sx:

↳spasticity/stiffness

↳pseudobulbar affect (inappropriate laugh, crying, forced yawning)

dx:

preferred imaging: MRI

tx:

riluzole

prognosis:

-progressive neuromuscular respiratory failure MC cause of death

Stroke of Middle Cerebral Artery (MCA)

etiology:

-most common artery

↳MC type of ischemic stroke

clinical manifestations:

-hemiplegia

-contralateral sensory loss

-contralateral homonymous hemianopia

left (dominant)

-aphasia

↳Broca's: expressive

↳Wernicke's: receptive

right (non-dominant)

-unilateral neglect

Stroke of Anterior Cerebral Artery (ACA)

classic signs:

↳contralateral leg weakness & sensory loss

↳incontinence

Stroke of Posterior Cerebral Artery

clinical manifestations:

-visual deficits

↳contralateral homonymous hemianopia

Cerebellar Stroke

clinical manifestations:

-impaired balance & coordination

Brainstem stroke

etiology:

-rare but devastating

-pts often critically ill

Arteriovenous Malformation (AVM)

definition:

direct arterial to venous connection WITHOUT capillary "bridge"

etiology:

autosomal dominant; HHT; Osler-Weber-Rendu syndrome

clinical manifestations:

MC intraparenchymal (intracranial hemorrhage)

dx:

-cerebral CT angiogram/MRA

tx:

small: observation

large: clot evacuation

Transient Ischemic Attack (TIA)

definition:

-brief interruption of blood flow to part of brain, spinal cord or retina.

-temporary stroke like sx which lasts minutes up to 24 hrs

etiology:

-embolic

-lacunar

-large artery

clinical manifestations:

-amaurosis fugax

dx:

best initial: CT head WITHOUT contrast

most accurate: MRI brain

tx:

-control HTN

-if carotid artery stenosis > 50%; carotid endarterectomy

Ischemic Stroke (Ischemic CerebroVascular Accident)

definition:

MC type of stroke (~90%)

etiology:

-MC thrombotic occlusion

clinical manifestations:

-balance loss

-eyesight changes

-facial droop

-arm weakness

-slurred speech

dx:

-NIHSS stroke scale

-best initial: CT head WITHOUT contrast

-most accurate: MRI brain

tx:

-mechanical thrombectomy

-IV tPA if onset within last 3-4.5 hrs

Epidural hemorrhage

etiology:

MC arterial bleed due to trauma

clinical manifestations:

-brief LOC

-lucid interval → coma

dx:

CT

↳convex bleed; temporal area MC

tx:

-neurovascular surgery; evacuation of hematoma

Subdural hemorrhage

etiology:

MC venous bleed due to blunt force trauma

↳associated w/ contre-coup injury

clinical manifestations:

focal deficits

dx:

CT

↳concave bleed

tx:

-if hematoma > 5 mm mid-line shift (massive)

↳evacuation

Subarachnoid hemorrhage (SAH)

etiology:

arterial bleed; MC berry aneurysm (saccular)

clinical manifestations:

-thunderclap HA MC

dx:

best initial: CT

↳if CT (-), LP

↳ Xanthochromia

gold standard: SAH 4 vessel angiogram

Intracerebral hemorrhage

etiology:

HTN MC overall cause

dx:

best initial: CT head w/out contrast

tx:

gradual BP reduction

Interventricular hemorrhage

etiology:

-MC in pre-mature infants

-in adults, trauma MC cause

clinical manifestations:

bulging fontanelles

dx:

CT Head

Brain Aneurysm

etiology:

MC in anterior circulation (~85%)

-saccular aneurysms in males: MC ACoA or ACA

-saccular aneurysms in females: MC PCoA

clinical manifestations:

-ACoA: unable to move/speak

-ACA: motor deficits; contralateral leg weakness & sensory loss

dx:

-CTA-MRA; aneurysmal SAH detected 90-95%

tx:

control HTN

TBI

etiology:

direct impact

↳coup: occur at area of direct impact to skull

↳contra-coup: located opposite site of direct impact

↳diffuse axonal injury: whole brain involved or wide spread damage

clinical manifestations:

Cushing's Triad:

↳ widened pulse pressure

↳ bradycardia

↳ irregular respirations

-decorticate posturing: injury above brainstem

-decerebrate posturing: injury at brainstem

dx:

-CT

Diffuse axonal injury

definition:

TBI where whole brain is involved

etiology:

MC w/ high speed MVC

clinical manifestations:

-vascular injury

-hypoxic/ischemic damage

-swelling

dx:

MRI study of choice

Uncomplicated concussion

definition:

mild traumatic brain injury w/ GCS 13-15

↳often leads to AMS

etiology:

-young: MVC MC

-elderly: Falls MC

clinical manifestations:

hallmark sx

↳confusion

↳amnesia

↳LOC

seizure typically NOT seen with mild TBI & concussion

Complicated concussion

definition:

cortical contusions + development of intracranial hemorrhage

clinical manifestations:

-neuro deterioration or focal neuro sx

-unequal pupils (aniscoria)

epidural/intracranial hemorrhage:

↳arterial

subdural

↳searing of bridging veins or dura

dx:

-neuro assessment

-westmead post-traumatic amnesia scale

-head CT w/o contrast

tx:

-neuro checks Q2H

Post-concussion syndrome

definition:

common sequelae of TBI/minor head trauma

clinical manifestations:

-cognitive deficits up to 6mo post injury

-persistent HA

dx:

Head CT

tx:

supportive care; pain control w/ analgesics

Pediatric Head Trauma

etiology:

-infants: abuse

-toddlers: abuse & falls

clinical manifestations:

-shaken baby syndrome

↳retina hemorrhage

↳subdural hematoma

↳brain swelling

dx:

PECARN Head CT rule

↳GCS must NOT be < 14

tx:

protective equipment

Peripheral Neuropathy

definition:

-synonymous w/ polyneuropathy

etiology:

-DM significant cause

clinical manifestations:

-loss of sensation MC

↳starts in distal LE and then ascends

-stocking glove distribution

dx:

-Electromyography (EMG)

(+/-) Nerve Conduction Study (NCS)

tx:

Gabapentin

Complex Regional Pain Syndrome (CRPS)

definition:

-disorder of the body characterized by pain, swelling, typically after a bone fracture

etiology:

-MC inciting events; fractures

-type 1 (MC): w/o evidence of peripheral nerve injury

-type 2: w/ evidence of peripheral nerve injury

clinical manifestations:

-pain, hyperalgesia

-motor impairment

dx:

-Budapest criteria

-immunoelectrophoresis

tx:

NSAIDs

Carpal Tunnel Syndrome (CTS)

definition:

median nerve entrapment and compression at carpal tunnel

etiology:

-increased incidence in pregnancy & DM

-repetitive hand and wrist use

clinical manifestations:

-numbness/tingling into first 3 fingers

dx:

-phalens and/or tinels test

-EMG (+/-) NCS

tx:

-wrist splint

Guillain-Barre syndrome

definition:

acute/chronic inflammatory demyelinating polyneuropathy

etiology:

-MC cause of acute flaccid paralysis in US due to provocation by preceding infection (URI or GIT)

types:

-acute inflammatory demyelinating polyneuropathy (AIDP): Schwann cells

↳peripheral N. myelin is the target

↳triggered immune response begins at nerve roots

-acute motor axonal neuropathy: Nodes of Ranvier

↳Campylobacter Jejuni infection

↳DTR may be preserved

↳no sensory or peripheral involvement

clinical manifestations:

-symmetric, ascending weakness & sensory changes

-starts in distal LE; may develop weakness of respiratory muscles

-MC sensory deficit is CN 7 palsy

↓ or absent DTRs

dx:

-progressive weakness of legs or arms + areflexia or ↓ reflexes in affected limbs

-LP; ↑ CSF protein w/ normal CSF cell counts

-NCS

-Plasmapheresis

tx:

none :(