Neuro Flashcards
Labs on neural tube defects?
Increased AFP
Increased AChE
What maternal disease is anencephaly associated with?
Maternal type 1 diabetes
What mutation is holoprosencephaly associated with?
Sonic headgehog
What conditions is holoprosencephaly associatd with?
Patau syndrome
FAS
How does syringomyelia present?
“Cape-like” bilateral loss of pain and temp sensation in UE (C8-T1)
- Ciari I malformation
Neuron staining?
Nissl (RER) - only in dendrites
Astrocyte staining?
GFAP
Where is Ach produced?
Basal nucleus of Meynert (degernation in Alzheimers)
Where is DA produced?
Ventral tegmentum
SNpc
Where is GABA prodcued?
Nucleus accumbens
- Made from glutamate and vit B6
Where is NE produced?
Locus ceruleus
Where is serotonin produced?
Raphe nucleus
Function of supraoptic nucleus of hypothalamus?
Makes ADH
Function of paraventricular nucleus of hypothalamus?
Makes oxytocin
Function of lateral area of hypothalamus?
Hunger
- Damage –> anorexia
- Stimulated by: ghrelin
- Inhibited by: leptin
Function of ventromedial area of hypothalamus?
- Satiety
- Damage (craniopharyngioma) –> hyperphagia
- Stimulated by: leptin
Function of anterior hypothalamus?
- Cooling (PS)
Function of posterior hypothalamus?
- Heating (S)
Function of suprachiasmatic nucleus?
- Circadian rhythm
- SCN releases NE –> pineal gland –> melatonin
Extraocular movements during REM due to?
PPRF
Treatment for bedwedding?
Oral desmopressin (1st line) Imipramine (TCA) 2nd line
Treatment for night terrors and sleepwalking?
Benzos
Function of VPL of thalamus?
Sensory from body
Funciton of VML of thalamus?
Sensory from face
Function of lateral geniculate nucleus of thalamus?
Vision
Function of medial geniculate nucleus of thalamus?
Hearing
Function of ventral lateral nucleus of thalamus?
Motor
Mesocortical DA Pathway
Negative sx
Mesolimbic DA pathway
Positive sx
- Antipsychotics
Nigrostriatal DA pathway
EPS
- Movement disorders, antipsychotic drugs
Tuberoinfundibular DA Pathway
Prolactin –> galactorrhea, gynecomastia
Lateral Cerebellum Lesion
Fall toward injured (ipsilateral) side
Medial Cerebellum Lesion
Truncal ataxia (wide-based cerebellar gait), nystagmus, head tilting - Bilateral motor deficits
Athetosis (snake-like movement)
Basal ganglia (Huntington)
Chorea (dancing)
Basal ganglia (huntington)
Dystonia
Writer’s cramp, belpharospasm, spasmodic torticollis, EPS due to antipsychotic meds
Essential Tremor (rest + movement)
Familial, self-medicate with alcohol
- Rx: nonselective beta-blocker (propanolol), primidone
Hemiballismus
Contralateral STN (lacunar stroke)
Intention tremor (w/ movement)
Cerebellar dysfunction
Resting tremor (at rest)
Parkinson
Brain findings in Parkinsons?
- Lewy bodies (alpha-synuclein - IC eosinophilic inclusions)
- Loss of DA neurons of SNPC
Type of gait in Parkinsons?
Shuffling gait (festinating gait)
Huntington DZ Genetics
AD w/ 100% penetrance
Trinucleotide CAG repeat on Chr 4
Anticipation (spermatogenesis)
NT in Huntington
Increased DA
Decreased GABA and ACh
Pathogenesis of Huntington
NMDA-R binding –> glutamate excitotoxicity –> neuronal death
Brain findings of Huntingtons
Atrophy of caudate and putamen w/ hydrocephalus ex vacuo
Kluver-Bucy Syndrome
- Hyperphagia, hypersexuality, hyperorality
- AMYGDALA
- HSV-1 encephalitis
Nondominant Parietal Cortex (R) Lesion
Hemispatial neglect on L
Gerstmann Syndrome
- Dominant parietal cortex lesion
Agraphia, acalculia, fingeragnosia, left-right disorientation
Cerebral perfusion driven by/
PCO2
ACA Stroke
Motor and sensory loss of LOWER LIMB (contralateral)
MCA Stroke
Motor and sensory loss of FACE and UPPER LIMB (contralateral)
Aphasia (if L hemisphere)
Hemineglect (if R hemisphere)
Lenticulostriate Artery Stroke
- Lacunar infarcts
- Secondary to unmanaged HTN
Motor and sensory loss of FACE and BODY
Like MCA but not aphasia/hemineglect
PCA Stroke
Contralateral hemianopia with macular sparing
Anterior Spinal Artery Stroke = MEDIAL MEDULLARY SYNDROME
- Motor loss of UPPER and LOWER LIMBS (lateral CST)
- Decreased contralateral proprioception (ML)
- Tongue points ipsilateral (hypoglossal, CN XII) (caudal medulla)
PICA Stroke = LATERAL MEDULLARY (WALLENBERG) SYNDROME
- Vomiting, vertigo, nystagmus
- Decreased pain and temp sensation from IPSILATERAL FACE (CN V) and CONTRALATERAL BODY (STT)
- ***DYSPHAGIA, HOARSENESS (CN X - nucleus ambiguus)
- Decreased gag reflex
- Ipsilateral Horner Syndrome
- Ataxia, dysmetria
AICA Stroke = LATERAL PONTINE SYNDROME
- Vomiting, vertigo, nystagmus
- ***PARALYSIS OF FACE, DECREASED LACRIMATION, SALIVATION, and TASTE from anterior 2/3 of tongue (CN VII - facial nucleus)
- Ipsilateral decreased pain and temp of face, contralateral decreased pain and temp fo body
- Ataxia, dysmetria
Basilar Artery Stroke = LOCKED IN SYNDROME
- Preserved consciousness, vertical eye movement, blinking
- QUADRIPLEGIA
- LOSS OF VOLUNTARY FACE/MOUTH/TONGUE MOVEMENT
Where do saccular aneurysms most commonly occur?
ACA
Rupture of saccular aneurysmleads to?
Subarachnoid hemorrhage
Epidural hematoma is rupture of which artery
Middle meningeal (branch of maxillary)
cause of epidural hematoma?
Skull fracture (temporal)
CT of epidural hematoma?
BICONVEX (lens shaped), doesn’t cross suture lines
Subdural hematoma is rupture of which vessels?
Bridging veins
What patients are subdural hematomas seen in?
- Trauma
- elderly, alcoholism
- SHAKEN BABIES
Cause of subarachnoid hemorrhage?
Rupture of berry aneurysm of AV malformation
Spinal tap of subarachnoid hemorrahge?
Bloody or yellow xanthochromic
What can occur after subarachnoid hemorrhage?
VASOSPASM
Prevent with nimlodipine (Ca2+ channel blocker)
Intraparenchymal hemorrhage caused by?
systemic HTN
Amyloid angiopathy (recurrent lobar hemorrhagic stroke in elderly)
Vasculitis
Neoplasm
CT of subdural hematoma?
CRESCENT SHAPED, crosses suture lines
Where does intraparenchymal hemorrahge usually occur?
Basal ganglia and internal capsule
Can be lobar
Charcot-Bouchard aneurysm implicated in which type of bleed?
Intraparenchymal hemorrhage in internal capsule
Flow through ventricles
Lateral ventricle –> foramina of Monro –> 3rd ventricle –> cerebral aqueduct of Sylvius –> 4th ventricle –> foramina of Luschka (lateral) or Magendie (medial) –> subarachnoid space
Pseduotumor Cerebri (Idiopathic Intracranial HTN) RF
- Woman of childbearing age, Vit A excess, danazol, tetracycline
Pseduotumor Cerebri (Idiopathic Intracranial HTN) Rx
- LP provides HA relief
- Weight loss
- Acetazolamide (decrease CSF production)
- Topiramate
- Repeat LP, CSF shunt, optic nerve fenestration
Communicating Hydrocephalus
Decreased CSF absorption by arachnoid granulations (post-meningitis)
Normal Pressure Hydrocephalus
- ELDERLY
- Expansion of entricles –> distorts fibers of corona radiata –> triad of urinary incontinence, ataxia, and cognitive dysfunction
- Magnetic gait (feet stuck to floor)
Noncommunicating Hydrocephalus
Structural blockage (stenosis, colloid cyst, tumors)
Ex vacuo ventriculomegaly
- Looks like increased CSF on imaging but it’s actually decreased brain
- Alzheimers, HIV, Pick DZ
What levels is intermediate horn (sympathetic) seen?
T1-L2/3
Dorsal Column
- P, vibration, fine touch, proprioception
- Decussates in medulla
- ROMBERG
Sinothalamic tract
- Lateral: pain, temp
- Anterior: crude touch, P
- Decussates at anterior white commissure
Lateral Corticospinal Tract
- Voluntary movement of contralateral limb
- Decussates at caudal medulla (pyramidal decussation)
Atrophy and fasciculations - UMN or LMN?
LMN lesion
Babinski - UMN or LMN?
UMN lesion
Polio - presentation and spinal cord?
- ANTERIOR HORN - LMN LESION ONLY
- Plus nonspecific sx
- CSF: increased WBCs and protein, no change in glucose
- ASSYMETRIC WEAKNESS
- Fecal to oral transmission –> replicates in oropharyns and small intestine –> spreads via bloodstream into CNS
Werdnig-Hoffman
- ANTERIOR HORN - LMN LESION ONLY
- “Floppy baby” + hypotonia + tongue fasciculations
- AR
- SYMMETRIC WEAKNESS
ALS
- UMN (LATERAL CORTICOSPINAL) AND LMN (ANTERIOR MOTOR HORN NEURON)
- NO SENSORY OR BOWEL/BLADDER DEFICITS
- Defect in superoxide dismutase 1 –> free radical injury
- Rx: riluzole
ALS Enzyme Defect
Superoxide dismutase 1 –> free radical injury
ALS Rx
Riluzole extends survival (decreases glutamate)
Tabes Dorsalis
- DORSAL COLUMN
- Tertiary syphilis
- PROGRESSIVE SENSORY ATAXIA
- Charcot joints, shooting pain, Argyll Robertson Pupils
- Absence of DTRS, + Romberg
Syringomyelia
- ANTERIOR WHITE COMMISSURE OF SPINOTHALAMIC TRACT –> bilateral loss of pain and temp in “cape-like” distribution
- Chiari I
Vit B12 Def
- SPINOCEREBELLAR TRACTS
- LATERAL CORTICOSPINAL TRACTS (UMN signs)
- DORSAL COLUMNS (bilateral loss of position and vibration, gait abnormalities)
Friedrich Ataxia Genetics
AR trinucleotide repeat disorder (GAA) on Chr 9
- FRATAXIN (iron binding protein) –> impaired mt functioning
- Staggering gait, frequent falling, nystagmus, pes cavus, hammer toes, DM, HYPERTROPHIC CARDIOMYOPATHY
- Kyphoscoliosis in kids
Cremaster Reflex
L1, 2
Anal Wink Reflex
S3, S4
Exits skull - CN I
Cribiform plate
Exits skull - CN II, ophthalmic artery
Optic canal
Exits skull - CN 3, 4, 6, V1
Superior orbital fissure
Exits skull - CN V2
Foramen rotundum
Exits skull - CN V3
Foramen ovale
Exits skull - middle meningeal artery
Foramen spinosum
Exits skull - CN 7, 8
Internal auditory meatus
Exits skull - CN 9, 10, 11
Jugular foramen
Exits skull - CN 12
Hypoglossal canal
Exits skull - brainstem
Foramen magnum
Which CN constricts pupil?
- CN III (oculomotor) - sphincter pupillae [Edinger-Westphal nucleus, muscarinic R]
Which CN is in charge of accommodation?
CN III
Which CN in charge of eyelid opening (levator palpebra)e?
CN III
Which CN in charge of lacrimation?
CN VII
Which CN in charge of salivation?
- CN VII *submandibular, sublingual
- CN IX *parotid
Which CN in charge of closing eylid?
CN VII *orbicularis oculi
Which CN involved with hearing?
- CN VIII (hearing)
- CN VII (auditory volume modulation via stapedius)
Which CN involved with monitoring carotid body chemo/baroreceptors?
CN IX
Which CN monirots aortic arch chemo/baroreceptors?
CN X
Corneal Reflex
- Afferent: V1
- Efferent: VII
Lacrimation Reflex
- Afferent: V1
- Efferent: VII
Jaw Jerk Reflex
- Afferent: V3
- Efferent: V3
Pupillary Refelx
- Afferent: II
- Efferent: III
Gag Reflex
- Afferent: IX
- Efferent: X
CN V Lesion
Jaw TOWARDS lesion (unopposed pterygoid)
CN X Lesion
Uvula AWAY (weak side collapses)
CN XI Lesion
Can’t turn head to opposite side (SCM)
Shoulder droop on side of lesion (trap)
CN XII Lesion
LMN Lesion
Tongue deviates TOWARD lesion (“lick your wounds”)
CN VII UMN Lesion (Motor Cortex)
CONTRALATERAL paralysis of lower facial muscles
FOREHEAD SPARED
CN VII LMN Lesion (Nucleus/Nerve)
IPSILATERAL paralysis of upper and lower facial muscles, hyperacusis (stapedius), loss of taste sensation to anterior tongue
Rx of Bells Palsy
Corticosteroids, acyclovir (HSV)
Conductive Hearing Loss
- Weber (top of head) –> AFFECTED EAR
- Rinne –> abnormal (bone > air) on side that Weber localized to
Sensorineural Hearing Loss
- Weber (top of head) –> UNAFFECTED EAR
- Rinne - normal
- Hearing loss is on side opposite of Weber
Noise-Induced Hearing Loss
ORGAN OF CORTI
Cholesteatoma
Desquamated keratin debris within middle ear
- Conductive hearing loss
- Pearly mass
- Painless otorrhea
What do you NOT give to someone with closed/narrow angle glaucoma?
Epinephrine (mydriatic)
Most common viral cause of conjuntivitis?
Adenovirus
What is uveitis associated with?
Sarcoid, RA, JIA, HLA-B27 related conditions
Dry Macular Degeneration
DRUSEN
Prevent with multivitamin and antioxidant
Wet Macular Degeneration
Choroidal neovascularization
Rx: anti-VEGF and smoking cessation
Nonproliferative Diabetic Retinopathy
Hemorrhages and macular edema
Rx: blood sugar control
Proliferative Diabetic Retinopathy
Chronic hypoxia –> new blood vessel formation
Rx: peripheral retinal photocoagulation, surgery, anti-VEGF
Retinal Vein Occlusion
Arterial atherosclerosis
- Retinal hemorrahge, venous engorgement, edema
Central Retinal ARtery Occlusion
- Acute, painless
CHERRY RED SPOT
Retinitis pigmentosa
Night blindness
Bone spicule-shaped deposits
Marcus Gunn pupil
CN II defect
CN III Damage
- Motor output (vascular DZ, DM): ptosis, “down and out” pupil
- PS output (compression): absent pupillary light reflex, “blown pupil”
CN IV Damage
- Upward gaze
- HEAD TILT TOWARD SIDE OF LESION
- Problems going down stairs –> compensatory head tilt in opposite direction
CN VI Damage
- Medially directed eye
- Horizontal diplopia
Function of MLF?
Coordinate VI and III –> eyes move in same horizontal direction
Internuclear Ophthalmoplegia
- Lesion in R MLF –> when you look to the L the L eye gets nystagmus (abducting eye) and the R eye looks straight ahead (impaired adduction)
Who has an increased risk of developing Alzheimers?
- Down patients (APP is on Chr 21)
- ApoE4 (sporadic) [ApoE2 has decreased risk]
- App, presenilin-1/2 (familial)
Brain Findings in Alzheimers
- Senile plaques (EC beta-amyloid from APP) [may cause amyloid angiopathy]
- Neurofibrillary tangles (IC hyperphosphorylated tau)
Frontotemporal Dementia Presentation
Personality/behavior probs + Aphasia
FT Dementia Brain Findings
- Hyperphosphorylated tau (round Pick bodies)
- Ubiquinated TDP-43
Lewy Body Dementia Presentation
Dementia + visual hallucinations –> parkinsonian features
Lewy Body Dementia Brain Findings
Lewy bodies (insoluble aggregates of alpha-synuclein)
Vascular Dementia causes
HTN, atherosclerosis, vasculitis
- Elderly
Creutzfeldt-Jakob Brain Findings:
Spongiform cortex
PRPc –> PRPsc (beta-pleated, resistant to proteases)
HLA associated with MS?
HLA-DR2
Presentation of MS?
- Optic neuritis (sudden loss of vision, Marcus Gunn pupils)
- INO (MLF)
- Hemiparesis
- Hemisensory sx
- Bladder/bowel dysfunction
- Women in 20-30s, relapsing/remitting, whites living further from equator
Charcot triad of MS?
Scanning speech, intention tremor, nystagmus
CSF in MS
Increased IgG and myelin basic protein
Oligoclonal bands
Gold standard for MS dx
MRI
- Periventricular plaque (oligodendrocyte loss + reactive gliosis)
MS Rx
- Disease-modifying: beta-interferon, glatiramer, natalizumab
- Acute flares: IV steroids
- Neurogenic bladder: muscarinic antagonists
- Spacticity: baclofen, GABA R antagonists
- Pain: opioids
Guillain Barre Syndrome cells affected?
Schwann cells –> PNS (endoneural)
GBS CSF findings??
Increased CSF protein with normal cell count (albuminocytologic dissociation)
Infections associated with GBS?
Campylobacter, viral
Molecular mimicry
Charcot-Marie-Tooth DZ
AD Foot deformities (pes cavus), LE weakness, sensory deficits
Krabbe DZ
AR lysosomal storage DZ - deficiency fo galactocerebrosidase –> buildup of galactocerebroside in macs and psychosine –> destroys myelin sheath
Metachromatic Leukodystrophy
- AR lysosomal storage DZ due to arylsulfatase A deficiency –> buildup of sulfatides –> impaired production and destruction of myelin sheath
Progressive Multifocal Leukoencephalopathy
Destruction of oligodendrocytes
AIDS patients
Due to reactivation of JC virus
Increased risk with natalizumab, rituximab
Adrenoleukodystrophy
X-linked
- Disrupted metabolism of very-long-chain FA –> build up in nervous system, adrenal glands, testes
Rx for cluster HA
Acute: sumatriptan, 100% O2
Prophylaxis: verapamil
Rx for tension HA
Analgesics, NSAIDs, acetaminophen, amitriptyline
Rx for migraine
Acute: NSAIDs, triptans, dihyroergotamine
Prophylaxis: lifestyle changes, beta-blockers, calcium channel blockers, amitryptiline, topiramate, valproate
Meniere DZ Presentation
- Vertigo, unilateral hearing loss, tinnitus
BPPV caused by?
Otoliths
Sturge-Weber Syndrome
- Activating mutation of GNAQ –> neural crest probs
- Port-wine stain of face
- Ipsilateral leptomeningeal angioma (seizures)
- Intellecutal disability
- Episcleral hemangioma (early onset glaucoma)
- Tram track calcification
Tuberous Sclerosis
- Hamartomas
- Angiofibromas
- Mitral regurg
- Ash-leaf spots
- Cardiac rhabdomyoma
- AD
- Intellectual disability
- Renal angiolypoma
- Seizure
- Shagreen patches
NF 1
- Cafe au lait
- Lisch nodules (iris hamartomas)
- Cutaneous neurofibromas
- Optic gliomas
- Pheochromocytomas
- Mutated NF1 tumor suppression gene (RAS) on Chr 17
- NEURAL CREST
Von Hippel Lindau DZ
- Hemangioblastomas in retina, brain stem, cerebellum, spin
- Angiomatosis in skin, mucosa, organs
- Bilateral RCC
- Pheochromocytoma
Glioblastoma Multiforme
- Astrocytes, GFAP +
- Pseudopalisading necrosis
Meningioma
- Arachnoid cells
- Whorled pattern, psammoma bodies
Hemangioblastoma
- Von-Hippel Lindau (+ retinal angiomas)
- Secondary polycythemia (ectopic EPO)
Schwannoma
- NF-2 = bilateral vestibular schwannomas
- S-100+ (neural crest)
Oligodendroglioma
Calcified, fried-egg, capillary chicken wire pattern
Pilocytic Astrocytoma
- GFAP +
- Rosenthal fibers, cystic + solid
Medulloblastoma
Primitive neuroectoderm tumor
- NOncommunicating hydrocephaus
- Drop mets to spinal cord
- Homer-Wright rosettes, small blue cells
Ependyoma
- Hydrocephalus (4th ventricle)
- Pervascular rosettes
- Rod-shaped belpharoplasts
Craniopharyngioma
- Bitemporal hemianopia
- Supratentorial tumor
- Rathke pouch
- Calcifications
- Cholesterol crystals in “motor oil” like fluid
Pinealoma
- Parinaud syndrome (vertical gaze palsy)
- Obstructive hydrocephalus (cerebral aqueduct)
- Precocious puberty in males (beta-hCG)