Systemic disease/general/pregnancy Flashcards
Autonomic dysreflexia
At or above T6 SC injury level
Development 5 primary vesicles of brain
Telencephalon- cortex
Diencephalon - thalamus, BG, hippocampus, amygdala
Mesencephalon - Midbrain
Metencephalon - pons and cerebellum
Myelencehalon - medulla, SC
Cavernous malformation
CCM1 - genetic mutation
Pilocytic vs anaplastic astrocytoma
Pilocytic - cystic mass in cerebellum, supratentorium
Lateral temporal vs medial temporal seizures
Lateral - shorter, auditory hallucinations, vertigo
Vs medial
Most susceptible to hypoxia
CA1 hippocampus (where you get Burnina bodies)
Ach
Preganglionic, parasympatheic postganglionic is ACh
Sympathetic sweat glands - ACh
Cystathione beta synthase deficiency
Homocystinuria
Clots
Aphemia
Amusia
Aphemia - Pure word mutisms, anarthria but comprehensive reading and writing intact
Amusia - nondom temporal - can’t process music
NF
NF 1 - cafe au lait, Lisce nodules, optic glioma
NF 2 - BL schwanommas/hearing loss; adulthood
Fatal familial insomnia
N2 loss sleep architecture
Inherited prion disease
Reduction in total sleep
Kluver busy
T2 hyperintensity BL anterior temp lobe HSV encephalitis association
osmotic demyelination
trident/snout sign
insomnia drugs MOA
Ramelteon - melatonin R agonist
Zolpidem - GABAergic
Trazodone - 5-HT2 antagonist
Suvorexant - inhibits Orexin R - suppresses wakefulness promoted by Orexin -
Orexin,( hypocretin)-narcolepsy type 1 + cataplexy- no orexin, low orexin in CSF (autoimmune attacking orexin neurons)
(Modafinil - blocks DA transport, neurostimulant)
infective endocarditis
ischemic stroke most common neuro complication
-mycotic aneurysms: distal arterial
-mechanical valves more complications vs bioprosthetic valves; equal complications with native valve
L sided endocarditis more neuro complications
-less common: secondary hemorrhagic, SAH, abscesses, meningoencephalitis
Sickle cell
Ischemic stroke more common than hemorrhagic; more common in kids than adults
Plasma cell dyscrasias
-Waldenstrom
-MGUS - M monoclonal spike - myelin associated glycoprotein MAG - even if no MM-demyelinating neuropathy
-MM
-Plasmacytomas
-Amyloidosis
Can get POEMS syndrome
-encephalopathy -hypercalcemia, hyperviscosity (IG)
-infection
-paraneoplastic +
-infiltrative neuropathy (amyloid) -sensorimotor axonal neuropathy
-cryoglobulinemia - neuropathy, rare mononeuritis; cerebral vasculitis-stroke
type I - IgM - MM, Waldenstrom
type II-polyclonal IgM, IgG - lymphoproliferative, autoimmune, Hep C
type III - IgM, IgG, infections, autoimmune disorders
ITP vs TTP vs DIC
Thrombotic thrombocytopenic purpura - seizure, microangiopathic hemolytic anemia, low plt, encephalopathy, renal dysfxn
-schistocytes, decreased plt NORMAL coags ;
Tx: plasma exchange
HUS - E coli, Shigella - ab pain, diarrhea in kids ->seizures, encephalopathy, CN palsy
DIC - fibrinogen reduced, D dimer elevated, abnormal PT/PTT
ITP - no neuro manifestations - no schistocytes
hepatic encephalopathy
serum ammonia can be normal
-low protein diet, reduced ammonia
- from colon via lactulose
-TIPS increases risks
path: Alzheimer type II astrocyte
Celiac disease/gluten sensitive enteropathy
neuro Sx can be only manifestations
-axonal peripheral neuropathy, inalmmatory myopathy
-cerebral calcifications risk seizures
-loss Purkinje cells cerebellum (not reversible)
-no increased malignancy
Inflammatory bowel disease
venous, arterial thrombosis
cranial neuropathies - CN VII (Melkersson Rosenthal syndrome, tongue fissure+angioedema)
peripheral neuropathy - demyelinating + axonal
malabsorp vit E + B12
electrolyte abnormalities
seizures - hyponatremia, hypoglycemia, hypomagnesemia
weakness - hypokalemia (spares bulbar) ; hypocalcemia-tetany, tingling in fingers; hypermagnesemia
encephalopathy - hypercalcemia
thyroid disease
waxing waning encephalopathy - SREAT-autoimmune thyroiditis - check anti-microsomal Ab
hyperthyroidism - always tremor; can also get parkinsonism, dyskinesia, chorea
thyroid eye disease - restricted upgaze most common
-eyelid retraction - overactivation Muller muscle
hypothyroidism - pseudomyotonia (delay muscle relaxation after reflex)
-entrapment neuropathy
-axonal/demyelinating neuropathy
diabetic neuropathy
CN - CN III + VI most common
- ipsilateral forehead pain 2/2 ischemia
diabetic amyotrophy -pain, sensory loss then weakness
neuropathy - accumulate sorbitol, fructose; oxidativestress
-early small fiber affected - tingling pain (small fiber neuropathy NCS NORMAL)
osteopetrosis vs Paget’s vs achondroplasia vs polychondritis
increased bone mass 2/2 impaired bone resorption by osteoclasts - AD and AR
Sx: fracture + facial weakness, hearing/vision loss - CN 2/2 skull thickening - compress nerves - CN I, II, VII, VIII
-alk phos elevated
vs Paget’s disease - can compress nerves via skull enlargement
-Alk phos elevated
vs achondroplasia - short stature, developmental delay
vs relapsing polychondritis - Cartilage - inflammation, destruction of cartilage - ears, upper airway
Sjogrens syndrome
sensory neuronopathy - can affect face/trunk before limbs
-Dx - Schirmer test (xerophthalmia), Ro/La
systemic vasculitides - small and medium vessel
Necrotizing vasculitis ischemic neuropathy - vasa vasorum
-Polyarteritis nodosa - Hep B
-painful neuropathy, abdominal pain
- mesenteric aneurysms
-P-ANCA positive
-livedo reticularis, skin ulcerations, nodules
-Kawasaki disease - kids - fever, conjunctivitis, coronary artery aneurysms, aseptic meningitis
-Granulomatosis with polyangiitis (Wegener) - renal disease, sinusitis, pulm
-C-ANCA, proteinase-3 Ab
-Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)- asthma, eosinophilia, pulm, sinus Sx
-p-ANCA
Tx: steroids, cyclophosphamide
large vessel vasculitis
Takayasu - subclavian, aorta, cerebral vessels
-constitutional Sx, peripheral claudication
Giant cell/temporal arteritis - HA, strokes rare
Bechet’s disease
-uveitis, lesions, thrombophlebitis, can get cognitive effects late; increase ICP
-RARE peripheral nervous system involved
Diagnosis: skin pathergy test
Hypercoagulability disorders
APLS - Risk CHOREA gravidarum, HA, seizures
(RLS-low iron)
factor V leiden, antithrombotic II deficiency, protein C/S deficiency, prothrombin polymorphism, hyperhomocysteinemia
neurosarcoidosis
-aseptic meningitis with just HA
-CN VII most common N affected
-low glucose in CSF;
increase serum ACE
-diffuse leptomeningeal enhancement - base of brain or splenium corpus callosum
-intra/extramedullary spine lesions
-granulomatous infiltration hypothalamus - > central DI (no ADH)
pheochromocytoma
can see in Sturge Weber, TSC, NF, VHL
Dx: plasma free metanephrines most sensitive
[vs carcinoid syndrome - episode flushing/HA - urinary 5-hydroxyindoleacetic acid]
Pituitary
vascular supply: ICA (superior, inferior hypophyseal arteries) - capeillary plexus in hypothalamic median emenence
-hypophyseal portal vein ->infundibulum/stalk to anterior pituitary
anterior - adenohypophysis- Rathke pouch-oropharyngeal membrane
- PRL (DA), GH; TSH; FSH, LH; ACTH
hypothalamus : GHRH, TRH, gonadotropin release hormone, CRH
posterior pituitary - neurohypophysis - neuroectoderm of diencephalon
-hypothalamus supraoptic + paraventricular magnocellular neurons - make ADH, oxytocin ->secrete from post pituitary
central DI
pituitary resection -> ADH deficiency
-hypernatremia -
polydipsia, polyuria
Dx: desmopressin (ADH analog) ->increase urine osmolality, decrease sodium (vs nephrogenic DI - urine osmolality does not increase in response to desmopressin - risk with lithium)
Tx: drinking water, giving desmopressin
SIADH vs cerebral salt wasting
CSW - SAH
-hypovolemic on exam
-high urinary osmolality
Tx: fluids (isotonic vs hypertonic), salt supplement
SIADH - euvolemic; SAH, head trauma, paraneoplastic
-fluid restrict
postpartum cerebral angiopathy
-similar to RCVS -> vasospasm
-HA
normal BP + kidneys
Dx: MRI, angio, Doppler US
Tx: CCB
