P-neuro Flashcards
young child w ET and RAPD
-description
-ddx
-eval
anomalous (small) optic disc w double ring sign
-optic nerve hypoplasia:
–VA nml to LP depending on if macular fibers are involved -> VF defects, strab, amblyopia, nystagmus
–can be associated w CNS abnormalities (if absent septum pellucidum, then DeMorsier syndrome -> severe endocrine abnormalities)
–can be idiopathic or caused by maternal DM1 or ingestion of phenytoin/quinine/LSD/EtOH
- optic nerve atrophy (genetic or tumor)
- optic nerve coloboma
hx: onset, progression (?prior exams), sxs (vision, strab, nystagmus, FTT, stunted growth, developmental delays, seizures) and RFs (maternal DM1 or drug ingestion during pregnancy)
exam: may need EUA
-ocular VS (alignment, nystagmus)
-nerve exam
-foveal hypoplasia
testing: OCT nerve (thin), neuroimaging of brain and orbit to look for a myriad of cortical malformations, including absent septum pellucidum (picture)
Young child w optic nerve hypoplasia and this MRI
- dx?
- management
- counsel
optic nerve hypoplasia + absent septum pellucidum = DeMorsier Syndrome
maximize vision: CRx, patching, strab
Endocrine eval and pituitary hormone replacement (growth hormone, TSH, ACTH)
VA can range from 20/20 to NLP
-If hypoplasia alone, usually can lead productive lives
-If CNS abnormalities, more likely intellectual and other disabilities. Need close f/u. Can stop worrying about hormonal derangements after puberty.
description
ddx
eval
blurring of disc margins without vessel obscuration or disc heme
-pseudopapilledema (optic disc drusen): examine fundus of parents
-papilledema: TVO x sec, pulsatile tinnitus, positional headaches, weight gain, drugs (steroids, OCP, vit A, Li, tetracycline / cipro / cyclo)
-papillitis: pain w movement
-exam: ocular VS, nerve exam (color vision, RAPD)
-testing: VF (arcuate defect), Bscan on low gain (hyperfluorescence at nerve head). Can also consider CT to look for calcifications, optic disc autofluorescence (picture-hyperfluorescent nodules in normally dark disc)
dx
mgmt
optic disc drusen
-normal VA but VF defect and at rare risk for NAION / vein occlusion
-follow annually w VF and disc photos
description
ddx
eval
abnormal disc vessels that appear to be optociliary shunts
ddx = causes of optociliary shunts = chronic poor drainage of CRV
- optic nerve sheath meningiomas (> sphenoid wing meningioma, optic nerve glioma though gliomas are more common in children): chronic progressive loss of vision, decreased color vision, RAPD, VF defects, proptosis, decreased motility, pain, lower lid edema and disc edema
- chronic papilledema
- prior CRVO: acute vision loss
- glaucoma
History: onset, progression (acute vs. chronic), sxs (pain, lower lid edema, vision loss, color vision, VF defects, proptosis, decreased EOM), RFs (FHx, NF, h/o IIH / CRVO / glaucoma)
exam:
-ocular VS
-nerve exam (color, hertel / resistance to retropulsion, VF)
-NF exam: CALBORN (2/6 for dx)
-?glaucoma/CRVO
testing: MRI (tram track = ONSM, a/w NF2; fusiform / kinked optic nerve = optic nerve glioma, a/w NF1)
middle aged woman with optociliary shunt + MRI
- can worsen w what condition?
- dx?
- mgmt
- counsel
pregnancy
optic nerve sheath meningioma
- no biopsy as it can compromise vision
- observe if vision is stable and proptosis is not disfiguring
- Otherwise, fractionated radiation to improve vision and slow tumor progression / improve disc edema
- If tumor is extending intracranially / encroaching the chiasm -> neurosurgery
- counsel: natural hx is slowly progressive vision loss and proptosis over yrs / decades, VA 20/20-LP, usually observe w visual fields and neuroimaging
description
ddx
eval
corkscrew conjunctival vessels
-carotid-cavernous fistula (no pain or discharge): whooshing sound, diplopia
-low flow: older patient w HTN
-high flow: old head trauma
-cavernous sinus thrombosis
-TED
-conjunctivitis: glaucoma gtts? discharge?
-episcleritis / scleritis: pain
-IOIS
hx: onset, progression, sxs (above), RFs (trauma, HTN)
exam:
-ocular VS
-exposure keratopathy due to proptosis
-nerve exam (color, Hertel, disc edema, tortuous vessels)
-auscultate orbit for bruit
-blood in Schlemm’s canal
test: CTA / MRA to look for enlarged superior ophthalmic vein. If negative, refer for angiography.
corkscrew vessels + MRI (description)
dx
mgmt
enlarged superior ophthalmic vein
carotid-cavernous fistula
refer to neuro-radiology for closure of CCF
if HTN: mmanage
if exposure keratopathy from proptosis: lubrication
if elevated IOP: glaucoma gtts
potentially life threatening but good prognosis if managed
DDX for various CN palsies:
1. CN3
2. CN4
3. CN6
4. CN7
Common:
- Ischemic: DM, cardiovascular, stroke, GCA
- Infectious: syphilis, lyme, TB
- Inflammatory / Infiltrative: IOIS, sarcoid, collagen vascular diseases (lupus, RA, Sjogren, scleroderma), mets
- Compressive: tumor
- Traumatic
- Congenital
CN3/4/6
-cavernous sinus syndrome (2/2 tumor, thrombosis, vascular), mucor
CN3
- pcomm aneurysm
CN6
- intracranial pressure
CN7
- temporal bone fracture, FN block, parotid surgery
- otitis, Ramsay-Hunt (VZV reactivation that affects facial nerve), Guillain-Barre
- cerebellopontine angle mass (tinnitus, vertigo, hearing loss, nystagmus)
- Bell’s
Parks 3 step test
e.g.1 RHT, worse on right head turn and tilt, excyclotorsion
e.g.2 LHT, worse on left head turn and tilt, incyclotorsion
- which eye is hyper
- worse on left or right head turn
- worse on left or right head tilt
e.g. 1: right SO
1. RHT = problem w right depressors or left elevators
2. worse on right head turn = problem with left gaze muscles
3. worse on right head tile = problem w OD intortors or OS extorters; SINRAD: superior intort, rectus adducts
e.g. 1: would be left SO except incyclotorsion so actually skew (vertical misalignment 2/2 asymmetrical otolithic input to ocular motor nucleus)
binocular diplopia
- ddx
- eval
- nerve palsy: use parks 3 step to determine which nerve. Etiology congenital, ischemic or traumatic.
- skew deviation: vertical misalignment 2/2 problems w inner ear and its afferent projections, most commonly MS or brainstem lacunar infarct
- myasthenia gravis: variable strabismus.
- TED
-hx: onset (old photos), progression, sxs (monocular vs. binocular, horizontal vs. vertical, intermittent vs. variable vs. constant; head tilt), RFs (trauma / stroke / MS / cardiovascular disease / DM / HTN / thyroid / MG)
-exam:
–ocular VS, hertel
–Parks 3 step + double maddox to meausre cyclotorsion (5 if unilateral CN4 palsy, 10 if bilateral; record if the eye is in / ex: read straight off the device so that if patient rotated 10 out from vertical, that’s 10 ex), vertical fusion (10PD if congenital, 2PD if acquired),
–other CN: lid function
–macular dragging
-testing:
-if new onset in <45yo w/o trauma and vasculopathy or if multiple CNs involved -> neuroimaging
-T3, free T4, TSH, TSI
-anti-Musk, anti-AchR, ice/rest/ten
CN4 palsy: mgmt
when to image?
Congenital
- decompensates in 4th or 5th decade. Same strategies as traumatic
Traumatic
- unlikely to self improve; offer strab surgery (SO tuck, harada ito to correct torsional component) once measurements stabilize for >6mo
- symptomatic mgmt: patch, fogging, prism
- if cause of CN palsy is unknown or presumed to be vasculopathic, follow every month. If no improvement within 3mo / new problems, then image.
sudden onset Down and out eye + ptosis
ddx
eval
mgmt
CN3 palsy: ischemic vs. compressive (pcomm, cavernous sinus syndrome [CN3-6], mucor)
- hx: onset, progression, sxs (fever, headache), RFs (trauma, cardiovascular disease, recent illnesses, immunocompromise)
- exam: ocular VS (motility spares abduction due to CN6), anisocoria (if enlarged pupil, then likely compressive such as PCOM aneurysm because the parasympathetic fibers are externally located), neuro exam
- testing: unless complete (ophthalmoplegia + ptosis) + pupil sparing + clear vasculopathic, emergent CTA and MRI brain and orbit
-mgmt:
– if mucor (answer): ENT, ID, NSGY
– if pcomm (prompt): IR, NSGY
TED: never ptosis
- exam: ?RAPD, hertel, lid lag
MG: never pupil involvement
- hx: variable; worst at night
- exam: cogan lid twitch, lid weakness, upgaze fatigue, variable diplopia
- testing: ice, rest, tensilon (have atropine ready for side effects), anti-Musk, anti-AchR
Horner: small pupil w ptosis, not large
sudden onset painful CN3 palsy with anisocoria - dx and mgmt
PCOMM aneursym
- stat CTA/MRA
- neuro consult for immediate intervention to prevent massive subarachnoid hemorrhage
60M w DM and HTN p/w diplopia
ddx and eval
imaging?
-CN6 palsy: congenital or acquired (ischemic, traumatic, increased intracranial pressure, cavernous sinus)
-MG
-TED
-orbital fracture of medial wall causing restriction
-hx:
–onset, progression, recurrence, constant vs. variable vs. intermittent
–Sxs: diplopia - monocular, binocular, horizontal, vertical, constant, intermittent, variable with fatigue or throughout day; scalp tenderness, jaw claudication, fever, weight loss; pain w motility
–RFs (trauma, cardiovascular disease, diabetes, HTN, trauma, thyroid disease, recent illness, immunocompromise)
-exam: ocular VS, hertel, lid, saccade + smooth pursuit, upgaze fatigue, VF, disc swelling or pallor, neuro exam, blood pressure
-labs: acetylcholine receptor antibody, thyroid lab, ESR/CRP +/- temporal artery biopsy
-testing: if bilateral CN6 palsy or multiple CNs involved, h/o cancer or optic disc edema, then order imaging
mgmt of CN6 palsy
-medicine referral for management of underlying disease
-symptom mgmt: patching, fogging, prisms
-90% of ischemic CN6 resolve in 90 days so follow monthly to resolution. If no improvement within 90 days or if progression of symptoms (involvement of new nerves), then image
-if symptomatic and negative workup, then strab surgery after stable measurements for >6mo
ddx
eval
testing if:
-infectious
-inflammatory
-stroke
-trauma
-when to image?
- bell’s palsy (dx of exclusion)
- infection (lyme, syphillis, otitis, Ramsay Hunt, Guilain-Barre)
- infiltrative (sarcoid, mets, collagen vascular disease like lupus, RA, AS)
- ischemic (HTN, DM, stroke)
- cerebellopontine angle mass: tinnitus, vertigo, hearing loss, nystagmus - trauma (temporal bone fracture)
- iatrogenic (FN block, parotid surgery)
eval: follow algorithm
–image if lower face only or bilateral
- hx: onset/progression/recurrence, sxs (pain, hearing loss, tinnitus, vertigo, diplopia), RFs (trauma/surgery/disease
- exam: ocular VS, lid position / lag / orbic strength, K health, upper vs. lower face, unilateral vs. bilateral, complete neuro exam including 5, 6, 7, 8, motor weakness, ataxia, balance, lymph nodes, skin rashes
- test:
–if infection: lyme, EBV, HIV, HZV, RPR, VDRL
–if infiltrative: sarcoid (ACE, lysozyme, chest CT), collagen vascular disease (ESR, ANA, ANCA)
–if stroke: MRI, carotid dopplers, ECHO, neuro consult
–if trauma: CT
–if cancer or duration >3mo = MRI
mgmt of bell’s palsy
acyclovir + pred x 14 days if within 3 days of symptoms onset (MUST ENSURE TB NEGATIVE PRIOR TO STEROIDS)
K: lubricate, gold weight / tarrsorrhaphy
most regain use over 3-6mo. Follow monthly until then. If no improvement or new symptoms after 3mo: image + neuro consult
Anisocoria algorithm and eval
mgmt for Horner’s
hx: onset, progression, sxs, RFs
Horner:
-hx: congenital vs. acquired, trauma, chiropractic, pain, neuro deficits, SOB / cough
-exam: ptosis with small pupil, dilation lag, heterochromia suggests congenital
-ddx: hydroxyamphetamine: dilates 1/2, not 3
–1st order (hypothalamus -> C8/T2): stroke (vertebrobasilar insufficiency), MS, brain tumor
–2nd order (exit spinal chord -> lung apex -> superior cervical ganglion): lung tumor (pancoast), mets
–3rd order: migraine, carotid dissection, herpes, trauma / forceps birth, Tolosa-Hunt (idiopathic inflammatory -> headaches and ophthalmoplegia),
-testing: emergent CTA/MRA, include lung (if non-revealing, gold std for dx carotid dissection is angiography)
-mgmt: if carotid dissection -> neurosg or IR for intravascular stenting w anticoag vs. observation
-prognosis: horner’s may or may not resolve upon correction of carotid dissection
CN3:
-hx: diplopia
-exam: ptosis, ophthalmoplegia
For Adie’s:
-exam: parasympathetic denervation -> vermiform movement, LND
-mgmt: observe
iris tear/iritis
-exam: AC cell, posterior synechiae
DDX for disc edema
Disc edema
- Ischemic: NAION, GCA, diabetic papillitis, malignant HTN, CRVO
- Infectious: syphilis, lyme, TB, bartonella
- Inflammatory / Infiltrative: IOIS, sarcoid, collagen vascular diseases (lupus, RA, Sjogren, scleroderma), mets
- Idiopathic: IIH
- Sinus venous thrombosis
- Compressive: glioma, meningioma, other tumors
- MS
description
ddx and eval
disc edema
-idiopathic intracranial hypertension (IIH)
–RFs: weight gain, vit A, steroid (use AND withdrawal), OCP, Li, tetracycline / cipro / cyclo
–s&s: positional headache, TVO (x secs), diplopia (2/2 CN6 palsy), pulsatile tinnitus, no spontaneous venous pulsation
–modified dandy criteria for dx: S&S + papilledema + normal MRI + elevated opening pressure
-intracranial mass
-venous sinus thrombosis
-malignant HTN: check BP
-papillitis (optic neuritis, diabetic, uveitis, leukemic infiltration): pain w eye movement, other intraocular inflammation
exam: ocular VS, nerve exam (color plates, edema, SVP)
-testing:
–RNFL and HVF 30-2
–MRI/MRV, if wnl, then LP for opening pressure (>25 in adults, >28 in children) and cytology
–B-scan to r/o drusen
pallid disc edema
ddx
eval
AAION = GCA
-hx: onset, progression, sxs (fever, weight loss, malaise, arthralgia/myalgia, proximal muscle weakness, temporal pain, scalp tenderness, jaw claudication, contralateral episode)
-exam: ocular VS, temporal cords, VF (GCA is a big deal - altitudinal defect), nerve palsies, CRAO / cherry red spot
-testing: elevated ESR/CRP/platelet though normal labs do not exclude diagnosis (ESR cutoff age/2 for M, (age+10)/2 for F); FA with patchy and delayed choroidal filling
NAION: must exclude GCA first in this age group.
optic neuritis: rare in the elderly
retinal vein occlusion
infectious (syphillis, TB, lyme) / infiltrative (ONSM)
painful red left eye lid: dx and mgmt
orbital cellulitis (fat stranding, proptosis) likely due to sinusitis (no periosteal abscess)
- IV unasyn, monitor daily, once improving - > PO augmentin
- ENT consult for sinusitis
- if periosteal abscess, consider draining
- good prognosis
description
ddx
eval
bilateral proptosis + conj injection + scleral show + lid retraction w lateral flare
TED
- hx: onset, progression, sxs (VA, pain, DES, hyperthyroid [heat intolerance, palpitations, weight loss]), RFs (smoking, level of control of thyroid disease)
- exam:
–ocular VS
–nerve exam (color vision, disc swelling / pallor)
–orbital exam (hertel, resistance to retropulsion, prism)
–K exam (lid lag, exposure)
–CAS (baseline orbital pain, motility orbital pain, swelling/redness of eyelid / conj / caruncle; change in VA / proptosis / motility)
- tests: TSH, T3, free T4, thyroid stimulating immunoglobulin, CT to look for muscle enlargement with tendon sparing, RNFL and HVF to monitor nerve
orbital tumors/ mets
Parinaud dorsal midbrain syndrome = sylvian aqueduct syndrome
- exam: upward gaze palsy, convergence retraction nystagmus, light-near dissociation, and bilateral lid retraction
- tx the underlying cause (pinealoma, congenital aqueductal stenosis, demyelination, thalamic stroke)
Idiopathic orbital inflammatory syndrome
young boy w upper eyelid fullness that enlarged over 14 days
ddx
- rhabdomyosarcoma (violet appearing): sudden onset painless proptosis
- chalazion
- lymphangioma (pic): fever, changing proptosis w valsalva or URIs
- metastatic neuroblastoma
- cellulitis
- plexiform neurofibroma
hx: onset, duration, progression, associated symptoms (fever, pain, lid swelling, discharge, vision change), triggering events (URI, sinusitis, trauma, h/o cancer)
exam:
-ocular VS
-lid exam (position, lag, painful or warm to touch)
-nerve exam (color vision, VF, nerve swelling or pallor)
-orbital exam (hertel, cranial nerve including K and trigeminal hypoestehsia 2/2 concern for orbital tumor, chorioretinal folds 2/2 compression of globe)
-systemic (temperature, lymph nodes in head and neck)
testing: CBC (cellulitis), CT for mass/infection
young boy w sudden proptosis
-image description
-dx
-mgmt
CT scan showing homogenous nasal mass without cyst, globe displacement and possible bony erosion
rhabdomyosarcoma (2/2 hx + location on CT)
-excisional biopsy (necessary for dx)
-mets workup (liver, bone and lungs)
-refer to onc for radiation and/or chemo
-prognosis dependent on type but usually good (95% cure rate) if no extraorbital involvement. Emphasize need for collaborative care
Localization of visual fields
A. central scotoma
B. Monocular vision loss
C. Bitemporal hemianopia
D. Homonymous hemianopia
E. superior quadranopia
F. inferior quadranopia
G. central sparing homonymous hemianopia
A. ipsilateral retina
B. ipsilateral ON
C. optic chiasm (posterior lesions are more central)
D. contralateral optic tract and beyond (bowtie atrophy)
E. contralateral inferior radiation and bank
F. contralateral superior radiation and bank
G. contralateral occipital lobe (posterior cerebral artery is occluded; if no central sparing then tract is also involved)
top left VF defect
description
ddx
eval
mgmt
Junctional scotoma
- chiasmal tumor (pituitary - pushes nerve from below, craniopharyngioma - pushes nerve from above and posterior, meningioma, glioma)
- vascular: aneurysm or stroke
hx: onset, progression (?prior VFs), sxs (), RFs (endocrine abnormalities [galactorrhea, weight gain/loss, buffalo hump change in menstrual cycle], NF1 CALBORN, neuro deficits [speech, mental status, sensory, motor functions], cancer)
exam:
-ocular VS
-nerve exam: color vision, nerve pallor/edema
testing:
-pregnancy test (tumor can enlarge)
-MRI to look for mass / vascular abnormalities
-pituitary labs (GH, PRL, ACTH, FSH, LH, TSH)
mgmt:
- nsgy for excision
- onc for adjunct radiation / chemo
- endo for pituitary abnl
- f/u 6mo
- counsel: benign tumors w good prognosis but risk of recurrence
NF1 dx criteria
2+ of CALBORN
cafe au lait
axillary freckling / astrocytic hamartoma (mulberry like - prompt)
lisch nodules
bony deformity (no greater sphenoid wing)
optic glioma
relative affected
neurofibromas
description
ddx
eval
s-shaped deformity of right upper eye lid causing mechanical ptosis
plexiform neurofibroma
rhabdomyosarcoma / sarcoma
neuroblastoma (proptosis, ecchymotic eyelid, Horner’s pupil, image)
hx: onset, duration, progression, associated sx (pain, vision change)
exam: ocular VS, color vision, VF, refraction because of mechanical astigmatism, CALBORN, optic nerve pallor/edema
testing: urine/blood catecholamines and metabolites HVA/VMA (r/o neuroblastoma), CT (sphenoid wing dysplasia, optic glioma)
description
ddx
eval
left eye hypoglobus, proptosis, no eyelid erythema
mucocele (above medial tendon)
encephalocele (above medial tendon, image)
dacryocystocele (below medial tendon)
orbital mass (e.g. dermoid)
sinus-originating tumor (e.g.SCC)
-hx: onset, duration, progression, associated sx (pain, diplopia, decreased vision), triggering events (trauma, sinusitis, dacryocystitis)
-exam: ocular VS, hertel, strab measurement, optic pallor/edema, choroidal folds (globe compression)
-testing: CT/MRI
description
dx
mgmt
circumscribed cystic mass that originates from the sinus with bony erosion
mucocele
-refer to ENT for endoscopic evacuation: urgent if RAPD/HVF.
-If pyocele: intraoperative culture and sensitivities
-Counsel risk of recurrence, residual strab needing surgery and need for sinus management
MVA, unconscious w OS sluggish pupil
description
ddx
eval
axial CT showing tenting of the posterior globe = “tear drop” sign
-retrobulbar hemorrhage
-orbital mass/tumor
hx: mode of injury
exam: ocular VS (demars for accurate IOP if eyelids swollen), forced ductions if no suspicion for open globe, lid injury, K injury, iris injury, uveitis, hyphema, lens dislocation, VH, RD/commotio, nerve pallor/edema
testing; CT for foreign body (metallic, organic) and fractures
7yo M s/p trauma OS p/w diplopia
description
ddx
eval
mgmt
CT coronal scan showing fracture of inferior orbital wall with hemo-sinus and likely muscle entrapment
orbital fracture w entrapment
orbital fracture w/o entrapment
-hx: mode of injury, onset, duration, progression of sx, associated sx (VA, pain, diplopia, flashes), last meal
-exam: ocular VS, enophthalmos, forced ductions if restricted EOM (looking for restriction or activation of oculo-cardiac reflexes - decreased HR, nausea), full exam for other signs of trauma
-NPO -> urgent repair in kids (24-48hrs) 2/2 risk of greenstick fractures that impinge muscle; 10-14 days for adults to wait for swelling to go down (repair indicated if bothersome diplopia, >50% of floor, enophthalmos >2mm) -> forced ductions + abx in OR -> plate if large fracture
-f/u 1wk to ensure healing and check EOM
-counsel risk of infection due to surgery/plate, diplopia, strab, enophthalmos, need for additional surgeries
description
ddx
eval
dx and mgmt
-coronal MRI showing hyperintense region in ST left orbit with extension into the bone
- primary tumor (lymphoma, lacrimal gland tumor)
- met
- vascular anomaly
- inflammatory (IOIS, collagen vascular)
-hx: onset, duration, progression, associated sx (VA, pain, diplopia), triggers/risk factors (h/o cancer, smoking)
- exam: ocular VS, color vision, hertel, VF, systemic symptoms (night sweats, weight loss)
- testing: CBC (r/o lymphoma), c-ANCA (r/o wegener’s), full body scan / PET after consultation w heme/onc given age
-primary tumor based on age and slow progression; lymphoma most common in 60+yo.
- biopsy via lateral orbitotomy -> path w special stains for lymphoproliferative malignancies
-if lymphoma: local external beam radiation usually curative for lesions confined to the orbit; onc referral for systemic workup (CBC, pet/CT, MRI, bone marrow biopsy) +/- chemo
-good prognosis if local
-if systemic, ok prognosis if responsive to chemo
description
eval
ddx
mgmt
(Segmental or diffuse) disc edema, contralateral eye disc at risk
hx: onset, progression, sxs (sudden painless loss of vision; r/o GCA w scalp tenderness, jaw claudication, malaise, weight loss), RFs (cardiovascular disease, DM, OSA, amiodarone, viagra)
exam:
-ocular VS
-nerve exam (colors, VF, CDR, heme, swelling, pallor)
testing:
-RNFL, HVF (altitudinal), FA (normal choroidal filling, mildly delayed disc filling)
-CBC, ESR/CRP
DDx:
- NAION
- AAION
- Optic neuritis
- CRVO
- Papillitis (DM)
Management
- Avoid antihypertensive at sleep
- Treat underlying dz
Education
- 40% can show improvement, 40% stable, 20% worse.
- Fellow eye at risk 15% at 5 years
- f/u 2 months, if worse, then MRI
