Kaplan Flashcards
port wine stain of face, seizrues, CNS abnormalities like homonymous hemianopsia, hemiparesis and mental subnormality
Sturge-Weber Syndrome
Seizures, progressive psychomotor retardation & mental deterioration, adenoma sebaceum (reddened facial nodules), shagreen patches (leathery plaques on the trunk), ash leaf (hypopigmented) patches, retinal lesions and cardiac rhabdomyomas
Tuberous Sclerosis
Of all the parkinsons drugs, which one is most likely to slow the progression of parkinsonism
Mao inhibitors like selegiline are assoc with the possibility of retarding the progression of parkinsonism
When a pt with severe parkinsonism being treated on levodopa/carbidopa, ropinirole and tocalpone and develops psychoses? How to treat?
CANNOT stop meds b/c if you stop meds in someone with severe parkinsonism can cause “locked in” with severe bradykinesia
START anti-psychotic -the one with the least EPS symptoms (secondary antipsychotic)
Why is sumatriptan a 5-HT agonist used only to abort migraines as opposed to being used ppx?
during migraine attacks, it is believed that it is assoc with decreased 5HT therefore sumatriptan can help. Sumatriptan causes cerebral vasoconstriction. In ppl with migraines when they are migraine free, that is assoc with increased 5-HT. Therefore, sumatriptan has no use ppx.
When should sumatriptan be avoided in aborting migraines?
if pts have underlying heart disease
Characterize nonproliferative retinopathy vs proliferative retinopathy caused by diabetes
nonproliferative: dilation of veins, microaneurysms, retinal edema, retinal hemorrhages. On funduscopic exam, hard exudates and “cotton-wool” spots are classic findings.
proliferative: more advanced, will see neovascularization
What on PFT makes one nervous about a myasthenia gravis crisis?
decrease FVC
- can do elective intubation
- treat with either IVIG or plasmapheresis for acute exacerbation
Sjogren syndrome is a chronic autoimmune inflammatory disorder that can occur either alone or assoc with other autoimmune disease (most commonly RA). How to histologically and clinically characterize sjogren? What autoantibodies can you find?
histologically: lymphocytic infiltration of exocrine glands (salivary and lacrimal)
clinical: dryness of mouth (xerostomia) can lead to difficulty swalling, abnormality in taste and smell, dental caries, hoarseness; keratoconjunctivitis (dry eyes)
can be anti-Ro and anti-La positive
Define cauda equina syndrome
- acute onset of lower back pain
- saddle anesthesia/diminished anal sphincter tone
- bowel and bladder incontinence
- hyporeflexia
- most commonly caused by cancer
- surgical emergency requiring immediate decompression
Diabetic with either nonprol or proliferative diabetic retinopathy should have what to prevent vision loss?
laser photocoagulation
Pt with proptosis, headaches, blurry vision and CT shows mass arising from lacrimal gland –> significant local tissue destruction. It is an aggressive carcinoma of lacrimal gland is?
adenoid cystic carcinoma
What is the most common cause of neonatal seizures? Neonatal seizures are usually focal, multifocal, tonic, myoclonic or subtle. The nervous system isn’t mature so not commonly generalized tonic-clonic
hypoxic-ischemic encephalopathy
clues to point to hypoxic ischemic encephalopathy as cause of seizures are: presence of severe late decelerations, acidotic cord pH, low Apgar scores
CSF finding of someone with GBS
CSF is elevated protein levels with normal WBC count aka albuminocytologic dissociation.
GBS is characterized by ascending paralysis with diminished or absent reflexes. It may be preceded by an infection and has been assoc with camplyobacter and HIV.
Type 1 spinal motor atrophy aka Werdnig-Hoffman disease
a degenerative disease of motor neurons that begins in fetal life and progresses in early infancy assoc with progressive denervation of muscle and subsequent atrophy of muscle fibers
clinically: an infant in first months of life presents with severe progressive hypotonia and generalized weakness with involvement of the face, jaw muscles and tongue. No DTRs, fasciculations of the tongue, can develop feeding difficulties and respiratory distress. Death occurs in early infancy or by 2 years of age.
confirm by muscle biopsy showing perineural denervation. But most simply can find SMN gene mutation in blood
Dacryostenosis -symptoms and treatment
blocked tear duct
- usu u/l and painless
- excessive tearing and mucoid material secretion
- treated with nasolacrimal massage until age 1 year
- nasal duct probing done after age 1
What is mononeuritis multiplex?
painful asymmetric asynchronous sensory and motor peripheral neuropathy involving isolated damage to >/- 2 separate nerves. Mononeuritis multiplex is commonly seen in pts with systemic vasculitidies like SLE, Churg-strauss, cryoglobulinemia, etc.
Neurocysticercosis is the most common cause of seizures in the developing world. How does one get it? Symptoms? Imaging results?
via fecal oral contaminated with larvae of taenia solium, pork tapeworm. Most neurocystiercal infections are asymp. Symptoms occur due to mass effect or an inflammatory response. Imaging will show ring-enhancing lesions signifying edema. Pt may or may not have eosinophilia.
Progressive multifocal leukoencephalopathy is caused by JC virus and is a disease found in immunosuppressed individuals. What are symptoms? What do you see on imaging?
- neurologic findings like hemiparesis, visual field deficits, ataxia, aphasia, decreased cognition, destruction of myelin (central demyelination)
- imaging will show patchy, focal, NONenhancing lesions of white matter
acute angle closure glaucoma is a medical emergency. what’s happening?
-iris is pushed or pulled up against the trabecular meshwork at the angle of the anterior chamber of the eye, causing a block in the normal outflow of aqueous humor, increasing intraocular pressure.
What is iridocyclitis?
a type of uveitis caused by inflammation (most likely autoimmune) involved the anterior structures of eye (iris, anterior chamber, ciliary body) presenting as u/l painful red eye, blurred vision, photophobia, and tearing. On PE, ipsilateral and consensual photophobia, 360 degree perilimbal injection, hypopyon (inflammatory cells floating in the aqueous humor), miosis.
opthalmology consult asap
how to differentiate simple vs complex partial seizures
simple -no LOC
complex -LOC
both can have focal motor signs and/or sensory symptoms
Meniere disease is characterized by?
- sensorineural haring loss, tinnitus and episodic vertigo lasting 20 min -24 hrs
- assoc with endolymphatic hydrops
- treatment is symptomatic with meclizine or scopolamine
What are symptoms of acoustic neuroma? How to differentiate from menierres
- vertigo, tinnitus and sensor hearing loss
- with CN deficits most typically CNVIII palsy
- MRI can usually show acoustic neuroma
Symptoms of food-borne botulism
- symmetric descending paralysis
- prominent bulbar palsies
- normal sensory
- absence of fever
The middle cerebral artery supplies blood to the trunk, arm, face area of motor and sensory cortices, and Broca and Wernicke speech areas. What are symp assoc with infarction of left vs right middle cerebral
left middle cerebral perfuses the left hemisphere which is more commonly the dominant hemisphere will lead to aphasia (wernicke or brocas). Infarction of nondominant side will cause contralateral neglect.
Basilar artery occlusion affects?
brainstem leading to paralysis of all extremities, heavy sensation deficits, difficulty in swallowing and difficulty in respiration. Most common cause of “locked in syndrome” presenting as quadriplegia and inability to speak in an otherwise cognitive individual with preserved eye function
Von hippel lindau is an inherited AD syndrome characterized by?
- hemanigoblastomas (blood vessel tumors of brain, eyes, spine)
- retinal angiomas
- increased risk for RCC and pheochromocytoma
NF type 2
“central form” of neurofibromatosis with multiple brain tumors, including schwannomas of the eight nerve, meningiomas, and gliomas
Medulloblastomas
arise in cerebellar vermis and grow down into 4th ventricle
- commonly presents with signs of increased ICP (nocturnal or morning headaches, N/V, AMS), truncal ataxia, head titubation (bobbing), nystagmus
- second most common brain tumor in children
Meningiomas
extrinsic tumors that arise from arachnoid cap cell, can arise from dura at any site most commonly the skull vault.
-common in adults; rare in children
Brainstem gliomas
-long history of minor complaints
-produce some neck stiffness or discomfort, proceeding to torticollis
-localized isolated CN findings
-
Nontraumatic intracerebral hemorrhage in majority of cases are due to which 4 possibilites
- hypertension
- amyloid angiopathy
- ruptured saccular aneurysm
- vascular malformation
Cerebral amyloid angiopathy hemorrhage
CAA is due to deposition of Ab amyloid in the small arteries and arterioles of leptomeninges and brain. Intracerebral bleeding related to CAA occurs in neocortex in a LOBAR distribution.
HTN and brain bleed -usu involves which sites?
primarily affects intraparenchymal arterioles and small arteries leading to intraparencyhmal bleed. Basal ganglia, cerebellum and pons are most commonly affected sites. CORTICAL bleed is usually not related to HTN.
pt with eye pain, blurry vision, red eye with signs of increasd IOP in the setting of juvenile RA points toward?
secondary acute angle closure glaucoma due to anterior uveitis
Arnold chiari malformation type 1 vs type 2
arnold chiari is a condition in which the inferior poles of the cerebellar hemispheres and the medulla protrude thru the foramen magnum into the spinal canal without displacing the lower brain stem due to developmental deformity of the occipital bone and upper end of the cervical spine.
Type 1 -wihtout meningomyelocele
Type 2-with meningomyelocele, also more assoc with hydrocephalus
Syringomyelia
can be assoc with chiari malformation
- cavitation of the spinal cord usually cervical
- b/l loss of pain and temp at the level of the lesion
- as the disease progresses, there is muscle weakness, –> flaccid paralysis and atrophy of the upper limb muscles due to destruction of ventral horn cells.
Pts with MS with flaccid, hyporeflexic bladder, urinary retention with leakage. Try on?
bethanechol to help with bladder contractility in addition to timed voids, fluid restriction, and intermittent self-cath, applying pressure on the lower abdomen while voiding
What’s ketotifen?
a histamine H1-receptor antagonist and a mast cell stabilizer for allergic conjunctivitis
What is acute diffuse encephalomyelitis?
a diffuse autoimmune demyelination process that typically follows a viral infection or antiviral vaccination, producing mutliple hyperdense inflammatory lesions in the brain and spinal cord in the white matter. There will be signs and symptoms of demyelination w/ encephalopathy, acute hemiparesis, cerebellar ataxia, cranial neuropathies and spinal cord dysfunction
What is transverse myelitis?
caused by an inflammatory process of the grey and white matter of the spinal cord. It can lead to axonal demyelination and manifests with weakness and various sensory disturbances below the level of the lesion
Low CSF volume headache -symptoms? How to diagnose?
headache that worsens with being upright and improves with laying supine. It can be assoc with neck pain, stiffness, hearing changes, visual changes, vertigo/dizziness, and N/V.
Test for CSF-specific beta-2-transferrin
Then brain imaging to identify the etiology of CSF leak with MRI better for looking at low CSF and CT for bony defect or past trauma
symptoms of anterior cerebral infarct
contralateral weakness and cortical sensory loss in the contralateral leg with or without weakness in arm. Urinary incontinence, confusion and behavioral disturbances
symptoms of middle cerebral artery occlusion
contralateral hemiplegia, hemisensory loss, aphasias, homonynous hemianopsia
symptoms of posterior cerebral artery occlusion
contralateral homonymous hemianopia, visual hallucinations, agnosias.
symptoms of vertebral artery occlusion
may not cause clinically noticable symptoms b/c of good collateral circulation.
Pure motor lacunar infarct affects?
genu/posterior limb of internal capsule
infarction of anterior limb of internal capsule causes mainly
ataxic hemiparesis
Drug induced myopathy:compare findings of statin vs steroids
corticosteroids: LE weakness and proximal muscle atrophy, CK enzymes within normal limits, EMG is normal
statins: significant increase in CK levels in addition to muscle weakness
Petechial rash in the setting of long bone fracture is pathognomonic for
fat embolism
Worry about spinal epidural abscess as causing spinal cord compression when someone with sudden back pain with bowel incontinence, motor and sensory changes also has symptoms of infection. What’s the most common pathogen assoc with spinal abscess?
staph aureus
Optic neuritis presents with several days of loss of vision, eye pain with movement, central scotoma, loss of color, afferent pupillary defect and inflammation of optic nerve with flame hemorrhages. (most pts have normal fundoscopic exam; next common finding is optic disk swelling/papillitis) how to treat and what test to order?
can treat with corticosteroids
order MRI to rule out MS since optic neuritis is commonly found in MS
TIAs involving vertebrobasilar artery aka vertebrobasilar insufficiency are usu much shorter than carotid TIAs. What’s the hallmark symptom of vertebrobasilar insuff? What are other symptoms?
hallmark: vertigo
other symptoms: diplopia, drowsiness, dysphagia, dysarthria, hemisensory loss, facial numbness or paresthesia, or syncope (drop attack)
Thalamic stroke presents as
pure sensory defects
PICA ischemia can result in Wallenberg syndrome aka PICA syndrome. symptoms?
- ipsilateral facial sensory loss
- contralateral body sensory loss
- vertigo
- ataxia
- dysarthria
- dysphagia
- horner syndrome
winging of the scapula is most likely caused by damage to which nerve? What can damage this nerve?
injury to long thoracic nerve which innervates the serratus anterior muscle
direct trauma to lateral sides of chest wall, activity that stretches the nerve like weight lifting and complications of surgery like modified radical mastectomy.
Radial nerve innervates brachioradialis, extensors of wrist, supinator of forearm, triceps. Damage causes?
wrist drop
benign recurrent vertigo aka vestibular neuronitis is?
syndrome characterized by sudden onset of vertigo, nausea, and vomiting. There is no change in hearing.
Explain complex regional pain syndrome
severe hyperesthesia (pain with innocuous stimuli) unilaterally, usually in an arm, weeks to months following trauma
initial symptoms include swelling, edema and redness
later in the course, signs and symptoms include atrophy, cyanosis, or pallor and contracture
left-sided neglect, left-sided weakness, left-sided homonymous hemianopsia indicate damage to which lobe?
right parietal lobe damage
**visuospatial neglect is specific to right parietal lobe lesions
Homonymous hemianospia with central sparing indicates lesion in which cortex?
occipital cortex lesion
If you suspect pseudotumor cerebri, what imaging study should you order?
MRI
Symptoms of anterior spinal artery occlusion
flaccid paralysis, loss of bowel and bladder function, loss of pain and temperature sensation since anterior spinal artery supplies the anterior horn cells and part of the spinothalamic pathways
normal opening pressure of LP
70-180 mmHg
Head bobbing is characteristic of which cause of tremor?
essential tremor
ALS is a progressive neurodegenerative disorder that affects motor neurons. Therefore symptoms are UMN and LMN signs. What’s the prognosis?
incurable
death w/in 3-5 years after diagnosis b/c of involvement of respiratory muscles
In someone with NPH, how much CSF will you be drawing
Usu large volume LP (30-50 mL) which is both diagnostic and therapeutic
What are the “do” and “don’t” in advice we give to caretakers of ppl with seizures
Do:
- place pt on the side
- put a pillow or other soft object under the pt’s head
- loosen tight clothing around the neck
- remove sharp objects from the surroundings
Don’t:
- put any object into pts mouth
- call am ambulance unless seizing lasts more than 10 min
- try to restrain pts
SIADH can develop after a CNS disease like brain injury due to head trauma, cerebrovascular accident, tumor or infection. What do you expect in terms of serum Na+, intravascular volume, serum osmolarity, urine osmolarity, and urine sodium excretion?
- hyponatremia (100 mOsm/kg)
- excessive urine sodium excretion (to normalize intravascular volume) > 25 mEq/L
What are the 6 P’s found with acute limb ischemia?
- Pain
- Pallor
- Paralysis
- Paresthesias
- Pulse deficit
- Poikilothermia (cold)
- irreversible if there’s complete paresthesia and paralysis without audible pulses –> amputation to prevent sepsis
- if there’s sign of reversibility, do arteriography first before intervention
Tuberous sclerosis is a neurocutaneous syndrome with hallmark cutaneous lesion described as hypopigmented ash-leaf macule that can be seen with Woods (UV) lamp. In infants with tuberous sclerosis, they often have infantile spasms and flexion of body with crying. How to treat infantile spasms?
ACTH to negative feedback CRH which is increased causing the spasms
IV drug abuser, new onset back pain, and fever, suspect? What to do next?
spinal epidural abscess (most commonly staph)
MRI is good, CT w/ contrast is an alternative
NO LP because of high risk of soft tissue infection; do blood cultures or direct needle aspiration of abscess
Sudden loss of bladder or bowel control + weakness + low back pain with metastatic cancer of spine is suggestive of epidural compression of spinal cord. What are next steps?
-IV dexamethasone followed by MRI followed by neurosurgery consult
Third nerve palsy can be caused by intracranial aneurysm characterized by
dilated pupil
drooping eyelid
inability to adduct elevate or depress the eye
In diabetic microvascular infarcts, how will CNIII findings differ from an intracranial aneurysm compressing the CNIII?
in diabetic microinfarction, parasym fibers are usually spared so can no dilated eye
Lumbar spinal stenosis is characterized by neurogenic claudication. Explain.
- pain radiating beyond the back to the buttocks, thighs or legs often causing the patient to stop walking
- pain is worsened with extension of the lumbar spine
- pain improves with forward flexion of the hips, knees, or lumbar spine as occurs with sitting or stooping forward (“shopping cart sign”)
- numbness and tingling may occur
- positive romberg may occur
Ring enhancing lesion on CT scan in HIV patient with low CD4 count, which 2 are on top of your differential?
- toxoplasmosis
- CNS lymphoma
Subarachnoid hemorrhage will result in the worst/thunderclap headache, N/V, stiff neck, blurry vision. This worst headache can be preceded by sentinal headaches from small bleeds. How to work up?
- CT without contrast is first test of choice
- if neg, but highly suspicious of SAH, do LP
- LP will show xanthrochromia -yellow color and/or RBCs
intracerebral hemorrhages are managed supportively. How does that differ from cerebellar hemorrhages?
Cerebellar hemorrhages have abrupt onset characterized by inability to walk due to imbalance, vomiting, headache, neck stiffness, gaze palsy, facial weakness.
-Surgery is immediate and life-saving to prevent cerebellar tonsillar herniation and coma.
If you suspect shaken baby syndrome, what are the steps of management?
do fundoscopic exam b/c ccan detect increased ICP (papilledema), then CT
also don’t forget to call CPS
Define enthesopathy
inflammation with assoc pain and tenderness at the insertion sites of tendons and ligaments onto bone. It is commonly seen in seronegative spondylarthropathies, such as anklyosing spondylitis and reactive arthritis
How to manage postherpetic neuralgia, severe pain that occurs after resolution of herpetic rash?
TCAs like desipramine
anticonvulsants like gabapentin or pregabalin
Medulloblastoma is the most common malignant brain tumor of childhood. Where does it grow? What are symptom?
It grows from the cerebellar vermis and presents with cerebellar signs and symptoms along with signs and symptoms of hydrocephalus as it commonly obstructs 4th ventricle
Ependymoma is another brain tumor of children and young adults. How does it differ from the most common one that affects cildren, medulloblastoma?
Ependymoma is often a tumor near ventricle, particularly the 4th ventricle. Medulloblastoma is a tumor of cerebellar vermis that can obstruct the 4th ventricle
Imaging to detect primary brain lesions vs metastatic
primary -use MRI
metastatic -CT with contrast is sufficient
Pronator drift is a sign of?
UMN disease
Pt just put on anticoagulants and develops neuro findings, get?
CT to rule out hemorrhage b/c anticoagulants increase risk of bleeding since it thins blood
Juvenile nasopharyngeal angiofibroma is a benign tumor that is markedly vascular. Who does it primarily affect? What are symptoms?
It occurs primarily in adolescent males. The lesion’s blood supply is from internal maxillary artery. Signs and symptoms include frequent epistaxis, nasal discharge, rhinorrhea, nasal obstruction, recurrent otitis media, conductive hearing loss, diplopia, eye pain. It looks like grayish-red mass in the posterior nasal pharynx. Dx is confirmed with MRI or CT scan with contrast.
What’s the most accurate test to confirm CSF leakage?
beta-2-transferrin (protein)
What is otosclerosis
AD disease in which new immature bone with abundant vascular channels cause ankylosis of the stapedial foot plate causing progressive b/l conductive hearing loss that becomes clinically present in late teens to early 20s. Pregnancy can cause condition to occur earlier or with OCP use.
Cavernous sinus syndrome
progressive severe headache or facial pain, usu unilateral or localized to retro-orbital and frontal regions
when opthalmoplegia develops, first nerve is typically CN VI therefore problem with lateral gaze. Can see exopthalmos, and lid edema, pupillary dilation signifying CN III involvement
rarely presents with high fever
Symptoms of orbital cellulitis
fever, hot, tender, red, swollen eyelids, protruding eyeball with visual deficits (due to extra-ocular movement), dilated & fixed pupil when the eyelid is pried open.
Primary open angle glaucoma risk factors: elevated IOP, black race, family history, DM. How should these guide screening?
- every year after 40 in pts with risk factors present
- every 3-5 yrs for pts btw 40-60 with no risk factors
- every 1-2 years after 60 with no risk factors
