Boards facts Flashcards
Targets for DBS in Parkinsons
GPi and STN
Mechanism of dabigatran
Direct thrombin inhibitor
Mechanism of rivaroxiban, apixiban
Factor Xa inhibitors
Chronic progressive ophthalmoplegia with cardiac involvement
Kearns-sayres
PComm aneurysm CN effects
CN III lesions
sensory innervation to the shoulder as well as the teres minor and deltoid muscles
Axillary nerve (posterior cord, C5-C6)
responsible for stabilizing the scapula against the thorax
Serrates anterior (long thoracic nerve, C5,6,7 right off brachial plexus)
responsible for the first 15 degrees of shoulder abduction
Supraspinatus (suprascapular nerve, c5,6)
CSF analysis will show an elevated protein, low glucose, and an elevated lymphocytic-predominant white cell count
Cryptococcus, CD4<200!
Lasmiditan
Migraine abortive that targets 5HT-1F receptors , safe in CAD
Looks like Rathke’s cleft cyst, but calcified
Craniopharyngioma
acute denervation to the tibial (gastrocnemius) and deep peroneal (tibialis anterior) innervated muscles
Sciatic nerve proximal to knee
The only CMT with axonal (not demylenating) polyneuropathy
CMT 2
CIDP, Charcot-Marie-Tooth type 1A, and Refsum disease can have this finding appreciated on a sural nerve biopsy.
Onion bulbs
superior gluteal (gluteus medius) tibial (gastrocnemius), and tibialis anterior nerves
L5 radiculopathy, the most common radiculopathy
VPL
relay center for sensory information of the body
VPM
relay center for sensory information of the face as well as taste
superior colliculi, located in the midbrain, actually play a prominent role in
Visual system
Auditory pathway
Hair cells > cochlear nucleus > trapezoid body > ventral pons > superior olivary complex > inferior colliculi (midbrain) > thalamus
thalamic astasia
Unilateral thalami stroke imitating cerebellar stroke (full strength but can’t stand)
Thalamic aphasia
presents with a fluency-predominant deficit and less impressive difficulties with comprehension
inferior olivary nucleus in the medulla, the red nucleus in the tegmentum of the midbrain, and the contralateral dentate nucleus in the cerebellum
Mollart’s triangle, lesions cause palatial myoclonus
ipsilateral third nerve palsy with contralateral hemiparesis
Weber’s sydrome, medial midbrain
Lesion for Alexia without agraphia
Splenium of the corpus callous
change in heart rate, blood pressure, respiratory rate during a seizure
Increase > R insula, decrease > l insula
Forearm pronation
median or radial nerve
Wrist flexion
Median nerve
elbow flexion
Musculocutaneous nerve
Musculocutaneous nerve and C6-7 portion of median nerve
Lateral cord
Radial nerve and axillary nerve
Posterior cord C5-C8
Ulnar nerve and C8-T1 portion of median nerve
Medial cord
Deltoid abduction plus triceps and wrist extension
Axillary AND radial nerves = posterior cord
Scapular winging
long thoracic nerve (C5-C7) damage (serratus anterior)
SCM (contralateral head turn) and trapezius (assists deltoid in lifting arm >90 degrees)
Spinal accessory nerve
ascending tract that carries information about pain and temperature
Spinothalamic (C shaped)
descending tract that controls skeletal muscle motor movement of the contralateral limbs
Paternal corticospinal (oval shaped)
Rhomboids
Dorsal scapular nerve
pufferfish dishes. Tetrodotoxin mechanism
preferentially block sodium channels
Looks like radial neuropathy at the axilla, plus weak deltoid and latissimus dorsi
Posterior cord plexopathy
Tongue deviation from a stroke–vascular supply
middle medulla is primarily supplied by the anterior spinal artery (ASA)
lacunar infarct to the ventral posterior nuclei
Pure sensory stroke
developmental regression, failure to thrive, and ataxia; tiger without eyes
Leigh syndrome
Osteolytic lesion in a child
Langerhans cell histiocytosis
anti-Hu paraneoplastic syndrome, Sjogren syndrome, pyridoxine toxicity, and exposure to certain chemotherapeutic agents
Common causes of sensory neuron-patchy (like isolated loss of proprioception)
type-II topoisomerase inhibitor that disrupts DNA synthesis and repair
Mitoxantrone
selectively targets the CD20-positive B-cells causing antibody-dependent cellular cytolysis
Ocrelizomab
α4-integrin antagonist that inhibits the transmigration of T-cells across the blood-brain barrier
Natalizomab
inhibits pyrimidine de novo synthesis thus inhibiting rapidly dividing cells, including t-cells
Teriflunomide
prolonged exposure to colchicine, amiodarone, chloroquine, or hydroxychloroquine
Myopathy and neuropathy!
The myelencephalon is responsible for the formation of the
Medulla
The telencephalon develops into
cerebral hemispheres, the basal ganglia, the hippocampus, and the amygdala
The diencephalon develops into the
thalamus, hypothalamus, and retina
The mesencephalon develops into the
Midbrain
The metencephalon develops into
the pons and cerebellum.
posterior predominant demyelination
X-linked luekodystrophy
Diffuse demyelination sparing U fibers
Krabbe
Frontal predominant demyelination
Alexander’s disease
Diffuse demyelination including U fibers
Canavan (Krabbe and metachromatic spare U)
Accumulates in Krabbe
galactosylceramide (space crab, U sparing)
Sphingomyelin accumulates in
Neimann-Pick disease
Glucosylceramide accumulates in
Gaucher disease
Globotriasylceramide accumulates in
Fabry disease (global fabric)
café-au-lait spots, Lisch nodules, and axillary freckling
NF1
Schwannomas
NF2
Ash-leaf spots and facial angiofibromas
Tuberous sclerosis
Hemangioblastomas
Von-Hippel Landau disease
can cause first-dose bradycardia, atrioventricular block, and QT interval prolongation
fingolimod
Looks like ALS but with conduction block
multifocal motor neuron (MMN) disease! Treatment is IVIG
deletion, mutation, or inappropriate methylation of the paternal copy of the q11-q13 segment of chromosome 15
Prader-Willi
deletion, mutation, or inappropriate methylation of the maternal copy of the q11-q13 segment of chromosome 15
Angelman
Infantile spasms and ash leaf spots, dx and tx
Tuberous sclerosis, vigabatrin (hipsarthymia, but NOT treated with ACTH)
Lacosamide mechanism
Selective enhancement of slow inactivation of SODIUM channels
Topiramate mechanism
SODIUM channels, GABA, glutamate and carbonic anhydrase inhibition
Entacapone
COMT inhibitor, reduces dopa breakdown
Exacerbates myoclonic seizures (5)
Gabapentin, pregabalin, vigabatrin, lamotrigine, carbamazepine
Enzyme inducing AEDs
phenytoin, phenobarb, oxcarb, carb, topiramate >200mg
Riluzole mechanism
inhibition of glutamine release (ALS)
irresistible attacks of sleep
ropinirole, pramipexole
Alemtuzumab
monoclonal antibody that targets the CD-52 receptor on activated T- and B-lymphocytes
Targets VEGF receptors
Avastin
Galactosyloceramidase deficiency
Krabbe
Alpha-galactosidase A deficiency
Fabry
Alpha-glucosidase deficiency
Pompe
myophosphorylase deficiency.
McArdle
Eteplirsen
Treats Duchenne’s with out-of-frame mutations on exon 51 of the dystrophin gene
Most cases present in children under the age of 10 and are located in the 4th ventricle
Ependymoma if (perivascular rosettes)
adenosine diphosphate (ADP) receptor inhibitor that must be activated by p450
Clopidogrel
Cardiomegaly, hepatomegaly, progressive muscle weakness, macroglossia, and hypotonia, and vacuolar myopathy can be seen on a muscle biopsy.
pompe, tx is alglucosidase alfa
Typical MR spectroscopy findings associated with glioma (choline, lactate, choline creatinine ratio)
levations in choline and lactate, reduction in NAA, and relatively unchanged creatine. The higher the ratio of choline to creatine, the high probability the lesion is a high-grade glioma
the only FDA-approved medication for primary progressive multiple sclerosis (PPMS
Ocrelizumab
NF1 stuff besides neurofibromas
optic nerve/chiasm glioma, pilocytic astrocytoma; cafe au lait spots, lisch nodules and axillary freckling
Assymetric finger weakness
Inclusion body myositis
SCLC and myotonia
Isaac’s syndrome, potassium channel antibodies
Perimysial inflammation
Dermatomyocytis
dysmorphic facies and elevated very-long-chain fatty acids
Zellwegger syndrome, peroxisome disfunction
Bilateral thalamic strokes
Artery of percheron
wrist extension
Radial nerver
Radial nerve injury: axilla, spiral groove or posterior interosseous
Wrist drop only at spiral groove, posterior interosseous is motor only
TX syddeham chorea
steroids
PD degeneration leading to tremor
substantia nigra pars compacta
lead poisoning leads to
wrist and finger peripheral neuropathy
from paint, causes CNS effects: AMS, cerebellar, basal ganglia
toulene poisoning
warfarin reversal
PCC is faster than vit K or FFP
Phenelezine
MAO-i, causes wt gain, sedation, constipation, urinary retention
ICP A waves
increased ICP, 30-50mmHg, >20min
ICp B waves
normal, respiratory cycle
ICP C waves
normal, cardiac cycle (c=cardiac)
forearm supination
biceps brachii (musculocutaneous) and brachioradialis (radial)
familial ALS inheritance
AD
manganese vs PD
manganese causes palladial degeneration (spares SN) and motor symptoms are symmetric at onset
Neomycin streptomycin gentamycin
Neo is cochleo-toxic, others are vestibulotoxic (so can treat meneire’s)
critical illness neuropathy: SNAP, CMAP, conduction velocity
decreased, decreased, does not change (Axonal not demyleinating)
Low vs High NMDA antagonism
Low: Memantine, High: Ketamine
Tx for lithium tremor
propranolol
Amitriptyline contraidication
Cardiac, but also BPH! (use nortriptyline, despramine)
endocrine abnormality not associated with proximal myopathy
HYPOparathyroidism
Tx for chorea gravidum
PLEX (if anti-phospholipid); haldol or chlorpromazine
latex allergy
spina bifida
brain areas involved in smell
frontal, temporal and limbic
DHE vs prochlorperazine in status migrainousis
No DHE if triptan within 4 hours
FHM and episodic ataxia genetic overlap (and 2 other conditions)
FHM1 and episodic ataxia 2 are both CACNA1A, calcium channel (same as SMA6 and childhood absence epilepsy)
motor function // origin of tibial nerve
tibialis posterior and plantar interossei // sciatic, L4-S3
motor function // origin of common peroneal nerve
short head of biceps femoris // sciatic, L4-S2
motor function // origin of sciatic nerve
long head of biceps femoris, semitendinous, semimembranous (“hamstrings”) // L4-S3
motor function // origin of saphenous nerve
no motor! sensory to medial lower leg // femoral, L3-L4
motor function // origin of sural nerve
no motor! sensory to lateral ankle and foot // L4-S1
optic neuritis refractory to steroids
PLEX
Are NF1 fibromas pre-malignant?
No, but patients do get malignant nerve sheath tumors and soft tissue sarcomas
Does optic neuritis count toward McDonald criteria?
No, at least 2 lesions in brain or spine separated by time
Vercay bodies
schwannoma (NF2)
Negri bodies
in pyramidal cells in rabies
Cowdry bodies
HSV, VZV, CMV
Pick bodies
FTD (tau fibrils, ubiquitin and tubulin)
psammoma bodies
balls of calcium seen in meningioma
weakness of hamstrings only
sciatic nerve (L5-S2)
weakness of hamstrings and dorsiflexors
S1-S2
weakness of hip flexors
femoral (L2-L4)
weakness of quadriceps (and numbness of medial calf)
L4
calcium release channel of sarcoplastic reticulum
RYR! mech of malignant hyperthermia and central core myopathy
paroxismal fast activity
infantile spasms
frontal intermittent rhythmic delta
toxic/metabolic encephalopathy
continuous spike-wave in slow sleep
Landau-Kleffner
Slow spike and wave
Lennox Gastaut
IV nimodipine
kills you? (calcium channel blocker for vasospasm prevention in SAH, give enterally)
Impairment in only one domain
Can still be major cognitive disorder! Alzheimers requires 2 domains, one being memory
strokes in Fabry’s
ischemic due to fatty deposits
intention tremor more likely in stroke or MS?
MS
lithium and endocrine
hyperparathyroidism
clozapine side effects
eosinophilic colitis, neutropenia (rare), drooling (common)
depakote and pancreatitis
yes! rarely
child who snores
has OSA
cell bodies of CNV arise from
semilunar ganglion
vasculitis with antibodies for PR3 (proteinase 3) or myeloperoxidase (MPO)
ANCA! GPA, EGPA and microscopic polyamgiitis
vasculitis and small vessel predominant in a child
Kawasaki
vasculitis with HBV
polyarteritis nodosa
vasculitis with HCV
cryoglobenemia
polyarteritis nodosa usually causes
mononeuritis multiplex, not strokes!
Whipple disease tx
Ceftriaxone
Stage 3 sleep
deep/delta, alernates with REM, first half of night
mania in pregnancy tx
Haldol
chorea, hemiballism, tongue biting
neuroacanthocytosis (not Leish-Nyhan–normal uric acid and tongue biting is accidental)
palpable vertebrae
C7
ocular bobbing
pontine lesion