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Neuro-Ophtho
Dr. Chungs outline
| Question | Answer |
|---|---|
| Papilledema is a swelling of what? | The nerve fiber layer of the retina is swollen |
| What is the eponym of the characteristic retinal finding in papilledema? What does it represent? | Paton's Line, peripapillar concentric folds |
| What is the earliest field of vision to be lost in chronic papilledema? | Inferionasal quadrant is first lost in this condition |
| What is wrong if you have a bilateral sixth nerve palsy and occasional transient visual obscurations but normal vision? | Likely papilledema |
| What are the etiologies of pseudotumor cerebri? | Vitamin A, Tetracyclines, naladixic acid, obesity, pregnancy, steroid withdrawal, sulfa drugs, ear infections, carotid dissection |
| What are the treatments for pseudotumor cerebri? | diamox, lasiks, topamax, optic nerve decompression, shunts/LPs |
| How does NAION present? | Acute, painless, altitudinal (70%) vision loss, disk edema |
| What are the treatments for NAION? | Aspirin sorta, no good ones, NOT optic nerve decompression, check ESR |
| What % of ANION pts develop it in the contralateral eye within 2 years? | 15% |
| What % of ANION pts recover full visual function spontaneously? | 30% |
| What % of ANION pts will get ipsilateral recurrence? | 6% |
| What is the most common cause of pseudo Foster Kennedy Syndrome? | ANION |
| What is the treatment and duration of treatment for Arteritic ION? | IV Methylprednisolone x 3 days then oral for at least 6 months, usually 2 years |
| What does a temporal artery biopsy entail? What is the false negative rate? | taking 2-3 cm of the artery looking for granulomatous inflammation with giant cells in the elastic lamina, 3-9% false neg rate |
| Without treatment, what % of GCA patients get contralateral involvement? What other major site is involved in these patients and how many % of pts have this concurrently? | 75% of pts will have contralateral involvement w/o treatment, concurrent aortitis is seen in 15% of pts |
| What are the signs of Posterior ION? | NORMAL fundus, painless sudden loss of vision often altitudinal, dense APD, often due to GCA, PAN, SLE, HZV, hypovolemia, hypotension, surgery, anemia |
| How does Optic Neuritis present? | Acute painful (92%) loss of vision, with 20/20 to NLP, color vision issues, only 33% have fundus changes, positive APD, optic nerve VF defects |
| What field defects would you expect in a young female patient with painful loss of vision? | Optic nerve related ones such as central or arcuate scotomas or altitudinal loss |
| How predictive are MRIs for detecting MS in patients with optic neuritis? | If abnormal (>1 lesion) its 56% chance at 10 years, if normal its 22% at 10 years |
| What is the treatment for Optic Neuritis? | NOT Oral prednisone (doubles risk of recurrence) only IV methyl pred and it only delays acute phase, does not effect long term outcomes |
| What is the prognosis for visual outcomes in optic neuritis associated with MS? What are the recurrence rates with and without MS? | 70% recover to 20/20, 92% to at least 20/40, worse loss = worse outcome, recurrence is 35% without MS, 48% with |
| What is the standard of treatment for MS? | Beta-interferon, can do glatiramer or mitoxantrone but its cardiotoxic |
| What is the presentation and likely cause of optic neuritis in children? | Sudden, bilateral, simultaneous, significant vision loss, often due to post-viral state. Good prog with IV methyl pred |
| What is Uthoff's phenomenon? Who gets it? | Transient blurring or greying of vision with increase in body temperature (MS) |
| What is this called: Transient blurring or greying of vision with increase in body temperature? Who gets it? | Uthoff's phenomenon, in MS pts |
| What is neuroretinitis most often due to? How does it present? | Cat scratch fever (1-2% of all CS pts). Presents as acute painless vision loss with disc edema and serous detachment at peripapilla, will form macular star in 4-7 days (exudates in Henle's layer) |
| Where is a macular star seen? What causes it? | Exudates in Henle's layer seen in neuroretinitis |
| What else can cause neuroretinitis? | Other than cat scratch, syphilis, lyme disease, TB, viral illness |
| What is neuromyelitis optica? What is the diagnostic test and treatment of choice? | aka Devic's disease, it is a optic neuritis with a transverse myelitis. Test NMO IgG and treat with high dose steroids |
| What is the NMO IgG test used for? What is the other name? | Neuromyelitis Optica, aka Devic's disease |
| How does Leber's Hereditary Optic Neuropathy (LHON) present? | acute painless profound loss of vision with contralateral involvement rapidly. Mostly men 10-30 years old. Fundus appears mostly normal, pseudo edema, peripapillar telangiectasias |
| How is LHON acquired? | Mitochondrial inheritance, deletion 11778 most common and worst, 14484 is best, 3460 middle, 50% sons, 10% daughters affected (85% carriers). Issues with retinal ganglion cells degenerating |
| What is the treatment of choice for LHON? | Nothing great, just CoQ, succinate and anti-oxidants |
| What is the likely cause of a slowly developing progressive loss of vision with loss of color vision and a RAPD. What is the visual field likely to look like? | Compressive Optic neuropathy presents this way, likely to have a central scotoma that breaks out temporally |
| What is the most common cause of compressive optic neuropathy? What kind of people do they occur in? | Optic nerve glioma, 75% <10y/o, 90%<20y/o, 10%-70% have NF1 |
| What type of compressive optic neuropathy would make you think of a genetic underlying cause? | Bilateral or chaismal optic nerve gliomas would make you think of NF type 1 |
| How do optic nerve gliomas present? | Progressive visual loss, proptosis, optic nerve pallor (60%), spasmus nutans (see saw nystagmus), can have Russell's syndrome |
| What are the treatment options of optic nerve gliomas? | Observation mostly, surgery if disfiguring, if very poor vision, or if chiasm is threatened, can do chemo |
| How to optic nerve gliomas look on MRI? Path? | fusiform enlargements, juvenile pilocytic astrocytomas or malignant glioblastomas if adult (painful vision loss) |
| How does an optic nerve glioma present in adults? | It is a PAINFUL loss of vision, likely due to glioblastoma, loss of vision in months, loss of life shortly thereafter |
| How does an optic nerve meningioma present? Who gets them? | These present as progressive vision loss, some proptosis, disc edema, in females bimodal. |
| How do optic nerve meningiomas look on CT? On path? | They have the characteristic railroad track "tram track signal". They look like benign meningioepithelial cells with psammoma bodies |
| What are the treatment options for optic nerve meningiomas? | observation, radiation, surgery (vision is sacrificed) most people suggest conservative |
| What are optociliary shunt vessels? What is the differential? | Ciliary blood flow dilation to accomodate for chronic central retinal vein obstruction, due most often to CRVO but also meningioma, glioma, arachnoid cysts, chornic papilledema, COAG. |
| What is the course of toxic/nutritional optic neuropathies? | Slow, progressive, bilateral and symmetric loss of vision with cecocentral scotomas (maculopapillary bodies effected) |
| What are the etiologies of toxic optic neuropathies? | MEDICAL: Methanol, Ethambutol, Digitalis, Isoniazid, Chloramphenocol, Alcohol, Lead. Also vincristine, nutritional, cisplatin, plaqunil, penicillamine etc |
| What can Amiodarone do to the optic nerve? | Causes bilateral, diffuse VF loss with slow resolution of disc edema follow d/c |
| What is probably happening in someone who comes in to the office in their 20s with red-green color issues, stable vision loss and central/bitemporal VF loss and temporal pallor of the nerve | Dominant Optic Neuropathy, affects Chromosome 3 GTPase in mitochondrial membrane which causes retinal ganglion cells to degenerate |
| What are the likely primary issues that lead to radiation induce optic neuropathy? When and What does it present as? | Acute painless LOV occuring usually 1 year after radiation for pituitary tumors, meningiomas or invasive sinus adenoCA. MRI w/ gadolinium shows optic nerve enhancement |
| How does Trauma affect the optic nerve? Prognosis and Treatment? | Avulsions of nerve are possible, 1-5% of head injuries result in optic neuropathy (25% direct, 75% indirect) No real good treatment, maybe High dose steroids, 1/3 improve |
| What is papillophlebitis? | Diagnosis of exclusion with unilateral disc edema in young healthy pt with normal vision/pupils, self limited, ?CRVO |
| Which type is more likely to get diabetic papillopathy? What does it look like? | Type 2 more likely, looks like hyperemic disc with radial dilation of vessels around nerve, spontaneously regresses, some pallor, maybe due to ischemia |
| What is optic nerve head drusen? Who does it occur in, how does it present? | Calcification of hyaline from degenerated RGCs. Occurs in caucasians, 75% bilaterally, AD incomplete penetration, small C:D, anomalous branching of vasculature, enlarged BS 60%, arcuate 60%, or transient obscurations 10% |
| What are some associations with optic nerve hypoplasia? | maternal diabetes, LSD, young age, quinine, anti-epileptics, ETOH, basal encephalocele, septo-optic dysplasia, |
| What is deMorsier syndrome? | Septo-optic dysplasia: optic nerve hypoplasia, absence of septum pellucidum, agenesis of the corpus collosum, endocrine dysfunction (30% panhypopit) |
| What are the etiologies of optic chiasm pathologies? | Pituitary tumors, meningioma, aneurysm, craniopharyngioma, gliomas, mets, abscess, ventriculomegally, demylenation, inflammation, trauma, ischemia, apoplexy |
| What might be the cause of a sudden headache and development of bitemporal hemianopia? | Pituitary apoplexy, ischemic necrosis of pituitary gland/tumor, treat with steroids before surgery |
| What is Benedikt's syndrome? | Injury to ipsilateral 3rd nerve palsy and contralateral cerebellar tremor and paresis (intention) due to damage of the red nucleus (Webers + Red nucleus) paralysis of upgaze |
| What is Weber's syndrome? | paremedian branches of the basilar artery occlude and cause cerebral peduncle ischemia and third nerve palsy including contralateral hemiplegia |
| What is Parinaud's syndrome? | Paralysis of upgaze, dilated pupil, loss of convergence and light reflex, lid retraction and nystagmus retractorious |
| What is Foster Kennedy Syndrome? | Ipsilateral optic atrophy, ipsilateral aniosmia, contralateral papilledema all due to a tumor |
| What happens if you lesion the central caudate nucleus (subnuclei of 3rd nerve) | bilateral ptosis, contralateral SR weakness and bilateral pupil involvement |
| What are some causes of pupil sparing 3rd nerve palsies? what needs to be done | vaso-oclusive, demyelinating, mysasthenia, GCA, migraines, often painful. NO imaging unless <50 or incomplete |
| What are some causes of pupil involving third? What needs to be done? | Aneurysm, compressive, tumor. MUST image if pupil involving, incomplete 3rd or <50 years of age |
| Who needs imaged when it comes to 3rd nerve palsies? | Pupil involving (unless diabetes), <50 y/o or incomplete 3rd, divisional third, aberrant regeneration |
| How does diabetic third nerve palsy present? | Third nerve symptoms with pain and 1-2 mm dilation, usually resolves in 3-6 months |
| What are the issues that can occur in the cavernous sinus? | Carotid cavernous fistula, aneurysm, pituitary tumors, infections, inflammation, usually involves multiple CN palsies |
| What is the likely cause of lid retraction on eye depression with segmental pupillary contraction when adducting or vertically moving the eye? What was the likely cause? | Aberrant regeneration of CN III following tumor or aneurysm, pseudo Von Graefe (NEVER after HTN or DM) |
| What type of ocular motility pattern does trauma to the head most commonly cause? Why? | V-pattern esotropia due to bilateral 4th nerve palsies with excyclotorsion <10degrees |
| What are the causes of 4th nerve palsies? | Trauma, congenital, ischemic, elevated IOP, MG, demyelination, tumor (schwannomas) |
| What is Brown's syndrome? | Shortening of the Superior oblique tendon such that patients are unable to fully elevate their eyes in adduction. They compensate with upward head tilt. Can be congenital or acquired with trauma/inflammation. Dx with forced ductions |
| What is S.O. myokymia | arrhythmic twitching of the SO muscle in its field of action, can be treated with Neurontin, tegretol, baclofen etc |
| How do you evaluate a suspected 4th nerve palsy? | Three step test, see which is higher, head turn then head tilt. Check double maddox rod for torsion. check fusional amplitude if congenital, tensilon testing? scan if young or persistent |
| What is the cause of a sixth nerve palsy combined with a horner's syndrome? | Cavernous sinus issue, sympathetics and CN6 travel together in there for a bit |
| What is Gradenigo's syndrome? | Pediatric pseudo-vestibular schwanomma, 6,7,8 are paretic, 5 is painful ipsilaterally. Complicated Otitis media |
| What is Moebius syndrome? | Bilateral 6th and 7th nerve palsies, aplasia of lower CNs, palatine and pharyngeal issues, deafness etc. CANNOT MOVE EYES laterally |
| What is Duane's syndrome? | abduction deficit with narrowing of fissure/retraction, can have both ab and ad issues or just ad but rare. Due to hypoplasia of abducens with co-innervation by CNIII |
| What is the differential for sixth nerve palsies? | thyroid eye, MG, spasm of near reflex, medial wall orbital fracture, decompensated esophoria (comitant), |
| What sits in the Cavernous sinus, which components are unlike the others? | CN III, IV, V1/2 and VI. VI is not encased. V3 never enters CS |
| What is a CCF, who does it happen to and how does it present? | Carotid Cavernous fistula, occurs in males mostly, post traumatic, with ophthalmoplegia and proptosis |
| How do a CCF and a Dural Sinus Fistula differ? | CCF is high flow, post traumatic and severe. Sinus fistulas are low flow, moderate in symptoms and often spontaneous with red eye |
| What are some primary tumors of the cavernous sinus? | Craniopharyngioma, chordoma, pituitary, meningioma, ganglioma, chondrosarcoma, nasopharyngeal carcinoma |
| Who does nasopharygneal carcinoma occur in? What eye manifestations does it have? | Asian men mostly, causes 6th nerve palsy, associated with EBV |
| What are some infectious causes of cavernous sinus disease? | Mucormycosis (DM), aspergillosis, bacterial sinusitis extension, zoster, TB. But also think Sarcoidosis and Wegners |
| What is Tolosa Hunt syndrome? | recurring attacks of severe pain and cavernous sinus syndrome that is treated effectively by high dose steroids. Diagnosis of exclusion |
| What are saccades? What is a normal latency? What is a normal velocity? | Rapid eye movements for refixation. Latency is 150-200msec, velocity is 400-500msec |
| What cells are involved in horizontal saccades? What nucleus is involved? | Burst cells in PPRF initiate saccades by projecting to VI nerve nucleus, MLF goes from here to contralateral MR subnucleus, pause cells are constantly firing except when saccades are going, |
| What controls the neural integration during saccades? | Nucleus prepositus hypoglossi (NPH) and media vestibular nucleus (MVN) near CN IX nucleus |
| What is the name for acquired oculomotor apraxia? How is congenital different? | Balint's syndrome, normal pursuit but bilateral lesions of supranucleus area and frontoparietal cortices leads to this issue. In congenital you have abnormal fast phase on OKN and head thrust with horizontal saccades |
| What is a frontal gaze palsy? How is it manifest and what is the likely outcome? | Gaze preference to ipsilateral side of frontal lobe lesion. Usually resolves in weeks, OKN is normal |
| What are square wave jerks? What causes them? | Macrosaccades that are jerk like, fixation instability, likely due to cerebellar disease, PSP, MS or parkinsons |
| What are flutters? What are some common etiologies? | Horizontal to-and-fro saccades. Due to: postviral encephalopathy, neuroblastoma (anti-hu), paraneoplastic (anti-hu/yo), MS, toxins, hyperosmolar coma |
| What is opsoclonus? What are some common etiologies? | chaotic, multidirectional nystagmus, due to same things as flutters: post-viral encephalopathy, neuroblastoma, paraneo (anti-hu/jo), MS, toxins, hyperosmolar coma |
| What is the pathway for smooth pursuit? | Primary visual cortex to middle temporal to middle superior temporal, posterior parietal, dorsolateral pontine, medial vestibular to optic motor nuclei |
| What is the latency and velocity of smooth pursuit? | Latency = 125-135 msec, velocity = 30-60 degrees per second |
| What are some causes of issues with smooth pursuit? What happens with a lesion in the pathway? | fatigue, altered mentation, CNS disease, parkinsons, PSP, alzheimers, schizophrenia. A lesion results in ipsilateral saccadic movements if any with contralateral homonymous VF loss |
| What is hemidecortication? | Slow pursuit ipsilaterally, abnormally fast contralaterally, saccadic in both directions. Pursuit paretic nystagmus forms with ipsilateral fast phase |
| What are the basics of PSP effects on the eyes? | neurodegenerative disease with progressive loss of voluntary eye movements, downgaze, then upgaze, then lateral. intact dolls eyes and bells. eyelid apraxia, square wave jerks |
| What are the basics of PSP non-ocular effects? | Axial rigidity, dementia, death from aspiration pneumo in 5 years, no treatment, midbrain atrophy on MRI |
| What are the ocular manifestations of parkinsons | infrequent blinking, hypometric saccades, cogwheeling pursuits, convergence paresis |
| What are the ocular manifestations of olivopontocerebellar atrophy? | pigmentary retinopathy, optic atrophy, progressive external ophthalmoplegia (also ataxia and slurred speech) |
| What are some neuro causes of issues with smooth pursuit? | Huntingtons, wilsons, ataxia telangiectasia, whipples, lipid storage diseases, PML |
| What is fischer's syndrome? | One and a half disease. PPRF or Sixth nerve nucleus with MLF also injured |
| What is internuclear ophthalmoplegia? | Damage to the MLF resulting in ipsilateral adduction deficit and abducting nystagmus on contralateral side. Bilateral can have upbeat nystagmus, in young its MS, in older pts its vascular |
| What is WEBINO? | Wall-eyed bilateral INO, damage to the rostral midbrain affecting the convergence center and MLF bilaterally |
| What is the vertical gaze center? | Rostral interstital MLF, (riMLF) in the midbrain. Receives input from FEF and Sup colliculus and is involved in the interstitial nucleus of cajal (INC) |
| What is dorsal midbrain syndrome? | Aka parinauds, it is paralysis of upgaze w/ light-near dissociation, convergence retraction nystagmus, lid retraction (collier's sign) and skew deviation +/- papilledema |
| What is the etiology of Parinaud's by age group? | 10 y/o = pinealoma, 20 y/o = head trauma, 30 y/o = brainstem vascular lesion, 40 y/o = MS, 50 y/o = CVA |
| What is skew deviation? | non-localizing VERTICAL deviation of the eyes, hypotropic is ipsilateral to lesion usually, alternating is due to INC issues, otolith disruption |
| What is the major difference between congenital and acquired nystagmus? When is congenital nystagmus relieved/aggravated? | No oscillopsia with congenital, remains horizontal in vertical gaze, better when accomodating, worse when fixation and anxiety, abolished in sleep, inverted OKN response |
| What is spasmus nutans? | Head tilting/nodding, onset by 1 y/o, gone by 4 y/o, fine, rapid horizontal nystagmus, otherwise normal, must neuroimage though as it is identical to monocular nystagmus of childhood |
| What is monocular nystagmus of childhood associated with? | appears identical to spasmus nutans but associated with chiasmal gliomas. |
| What is Heimann-Bielchowsky phenomenon? | Slow, coarse verticle pendular nystagmus seen unilaterally in young patients with profound visual loss in same eye |
| What are some etiologies of gaze-evoked nystagmus? | drugs, cerebellar disease, gaze paretic nystagmus |
| What is periodic alternating nystagmus? | Jerk nystagmus in 1 direction for 90 seconds followed by opposite direction for 90 seconds, cycles every 4 minutes. Etiology: congenital, MS, stroke, blind, drugs, CJD |
| What is the differential for up-beat nystagmus based on the direction of gaze? | Primary gaze: anterior vermis disease (Wernieckes) Up gaze: drugs. can also be cerebellar degen, MS, tumor, stroke, encephalitis etc |
| What are some causes of downbeat nystagmus? | Arnold-Chiari, tumors, demyelination, stroke, trauma, alcohol, lithium, anticonvulsants, Mg deficiency, syrinx, paraneo, B12 def, spinocerebellar degen, basilar invagination, idiopathic |
| What is see-saw nystagmus? | one eye rises and intorts while the other falls and extorts then they switch. This indicates chiasmal lesion, check VF. Can also be brainstem CVA, MS, trauma or arnold chiari |
| What is oculopalatal myoclonus? | vertical nystagmus with syncronous movement of whole face, even body, occuring months to years after brainstem stroke, see hypertrophy of inferior olivary nucleus |
| What is oculomasticatory myorhythmia | pendular vergence oscillations coordinated with contraction of masticatory muscles, associated with neuro whipples disease (fever, diarrhea, weight loss) |
| What is the pathway for volitional facial movements? | Cortex, internal capsule, cerebral peduncle, facial nucleus (lower face = contralateral, upper face = both), internal auditory meatus in petrous bone |
| How are emotional facial movements controlled? | blinking controlled by extraparamidal system with connections to amygdala, BG, thalamus etc |
| What does the motor branch of the facial nerve supply? | stapedius, stylohyoid, muscles of facial expression (buccinator, platysma, occipitalis) |
| What does the visceral motor branch of the facial nerve do? | Lacrimal, submandibular, sublingual glands and mucous membranes of nose/palates. Greater petrosal nerve (lacrimal/nasal), chorda tympani (submandibular/sublingual) |
| What are the sensory components of the facial nerve? | small area behind ear, skin of choncha of auricle, taste of anterior 2/3 of tongue |
| What causes crocodile tears? What is it? | Aberrant connection of fibers destined for the submandibular/sublingual glands that instead go to lacrimal gland, often after bells or injury to facial nerve. You cry when eating or smelling food |
| What is the symptomatology of bells palsy? What is the treatment? | Pain preceeds or concurrent with paralysis, can have facial numbness, decreased tearing and taste. Steroids at 3 and 9 months have been proven beneficial |
| What is Ramsey Hunt syndrome? | Reactivation of HZV in the geniculate ganglion causing bells palsy |
| What is the prognosis for Bell's palsy? What is the most common adverse outcome? | 85% return to function by 3 weeks and complete by 2-3 months, 25% have incomplete or aberrant regeneration (more likely with dysacusis, complete palsy, old age or impaired lacrimation) |
| What cranial nerves do sarcoid affect first? What other location in the head? | CN 7 then CN 2, parotid infiltration is common |
| What is Melkerson-Rosenthal syndrome? | Recurrent facial paralysis and tongue swelling/furrowing in childhood or adolescence |
| How does blepharospasm present? | Bilateral, age 40-60, can be associated with BG dysfunction or in Meige's syndrome with lower face involvement, rule out dry eyes, inflammation, meningeal irritation etc |
| What are the treatments for blepharospasm? | Medical: baclofen, haldol, clonopin, Botox!, myectomy |
| What is Meige's syndrome? | Blepharospasm and oromandibular dystonia combined, you get upper and lower face spasms |
| What is the cause of hemifacial spasm most often? What is the treatment? | an anomalous blood vessel that compressed nerve at nerve root entry. Rx = Janetta procedure = microvasular decompression. Should image as 0.5% are due to tumors |
| What is facial myokymia? | Undulating, vermiform facial movements, usually pontine glioma in kids, demyelinating in adults, begins in orbicularis and spreads usually. Rx = dilantin |
| What symptoms occur from a pontocerebellar angle tumor? | Abduction palsy, facial nerve weakness, hyperacusis, loss of taste, decreased tearing, horners, ataxia/clumsiness, 8th nerve sometimes |
| What is the efferent pathway for the pupillary constrictor nerves? | EW to third nerve, inferior branch of third nerve, synapse in ciliary ganglion, spinchter and body via short ciliary nerves |
| What is the efferent pathway for the pupillary dilator nerves? | first order = hypothalamus to ciliospinal center of budge (T1), 2nd = lung apex to synapse at sup cervical ganglion. 3rd = ICA into CS then to orbitl 6th nerve, then V1 then ciliary body (NO synapse) then via long ciliary nerves |
| Where do the sudomotor nerves innervating the face travel? | In the external carotid artery for facial sweating |
| What conditions can cause APD? | Optic neuropathy, chaismal lesion, large retinal issue. CONTRALATERAL causes: optic tract, dense cataract, midbrain lesions |
| What are some causes of light/near dissociation? | Argyll Robertson, Parinauds, pretectal lesion, diabetes, alcholism, MS, Sarcoid, amyloid, encephalitis, tonic pupils |
| What is an Argyll Robertson pupil? | Usually due to syphilis, its a small pupil <2mm, that accomodates but doesn't react to light, poor dilate with drops, iris atrophy is common |
| How do you make the diagnosis of a pharmacologically dilated pupil? | 1% pilocarpine fails to constrict |
| How do you diagnose Adie's tonic pupil? Who does it occur in? | 0.125% pilocarpine constricts (or 2.5% methacholine), DTRs are diminished in 90% of patients (Holmes-Adies), knee>ankle>arm. Mostly young women, unilateral, due to issues with ciliary body? |
| What does Adie's look like? | Dilated pupil, usually unilateral, vermiform constrictions, thinning of pupillary ruff, accomodation is slow but recovers in months, zoster, GCA, trauma can cause |
| What are some non-adies causes of bilateral tonic pupils | syphilis, diabetes, alcoholism, dysautonomia |
| What is the triad of horners? Be specific about dimensions. What are some other signs you can get? | Ptosis (2mm due to muellers), miosis, anhydrosis (unless third order), dilator lag, can have reverse ptosis (inferior tarsal droop) |
| What tests are used for horner's patients? | 4% cocaine should dilate normally, Paredrine (hydroxyamphetamine) works if 1st or 2nd order, apraclonidine works regardless (denervation hypersens) |
| What are some causes of Horner's based on the order of nerve level? | 1st order = CNS lesions, neck trauma, syrinx. 2nd order = lung lesion, thoracic aortic aneurysm, brachial plexus trauma. 3rd = benign, ICA dissection, CCF or cavernous disease, nasopharyngael CA |
| What are some signs of congenital horners? | Lighter iris ipsilateral to horners, birth trauma to brachial plexus, r/o neuroblastoma, drop tests aren't accurate |
| What is the cause of painful horners until proven otherwise? | dissection of ICA, cluster headaches |
| What type of patients get benign episodic pupillary dilation? | Migraneurs, lasts minutes to hours |
| What are the disadvantages of angiography, MRA and CTA? | Angiography = 2.5% complication risk, MRA can miss 2-3mm aneusysms, CTA still uses contrast |
| What is the best MRI setting for the orbit? What about for demyelination? Tissue water content? | STIR, FLAIR, T2 |
| What are the strong epidemiologic associations regarding MS? | moderate climates, increases with latitude, 2:1 females 20-35 y/o, family history (20x), HLA-DR15/B1 |
| What proportion of pts with MS present with relapsing remitting course? What % become secondary progressive at 10 years, what % are primary progressive, what % have benign course? | 90% r/r course, 50% secondary at 10 yrs, 10-20% primary, 30% have benign |
| Which is better? Early or late presentation? Short or long period between first and second attacks? | Worse to get it later in life and worse if there is a short gap between attacks |
| What does a spinal tap look for in pts with suspected MS? | Oligoclonal bands, IgG, IgG/albumin index |
| What 3 things usually make the diagnosis of MS? | MRI abnormality, Spinal tap, 2 episodes separated by time with characteristic symptoms |
| What are the non-ocular characteristic symptoms of MS that can lead to diagnosis? | Vertigo, ataxia, scanning speech, intention tremor, weakness, parasthesias, L'hermettes sign, emotional instability, urinary incontinence |
| What are the ocular manifestations of MS? | 6th nerve, INO, skew, nystagmus, gaze palsy's, third nerve, venous sheathing in retina, posterior uveitis, vasculitis, optic nerve disease |
| Describe the optic nerve disease in MS | 100% demyelination, 75% acute optic neuritis, Uhthoffs, despite good VA, contrast, VA, color and steropsis are poor |
| What is the % of men and women individually who will go on to have MS after an episode of optic neuritis? | Men = 34% at 15 years, 45% at 20 years. Women = 74% at 15 years and 91% at 20 years |
| What are the treatments for MS? | Avonex, Betaseron, Copaxone, Rebif. Beta interferons reduce exacerbations by 1/3, can cause flu like symptoms, costs >10k per year |
| What are the symptoms of NF1 | Neurofibromas, cafe-au-lait spots, axillary freckling, AD 17Q11.2, lisch nodules, plexiform neurofibromas (S-shaped lid), absense of sphenoid (pulsing exoph), optic nerve glioma 15%, other tumors/bone defects |
| What are the symptoms of NF2 | Bilateral Acoustic neuromas, PSCs, AD 22q11, no lisch nodules |
| What are the symptoms of Tuberous Sclerosis? | adenoma sebaceum, mental deficiency, epilepsy, periungal fibromas, cafe-au-lait or ashleaf spots, brainstones, cardiac RMS, renal cysts, angiomyolipomas, astrocytic hamartomas of retina, AD on chrom 9 |
| What are the symptoms of Sturge-Weber? | Nevus flameus, glaucoma, seizures, MR, ipsilateral leptomenigial hemangioma, cavernous hemangiomas of choroid |
| What are the symptoms of Klippel-Trenaunay-Weber syndrome? | Nevus flammeus, hemangiomas, varicosity, hemihypertropy of limbs, CNS angiomas |
| What are the symptoms of Von Hippel Lindau syndrome? | Retinal angioblastoma, feeder vessel and drainer vessel, multiple and bilateral usually, cerebellar hemangioblastomas, medulla or spinal cord, cysts of organs, pheos, RCCA, AD or sporadic |
| What are the symptoms of ataxia telangiectasia syndrome? | aka louis-bar syndrome, cerebellar ataxia with Teles, thymic hypoplasia (IgA issues), conj teles, ocular motor apraxia (saccades then pursuits), fixation nystagmus and strabismus, poor convergence, OKN fine, Lymphoma/leukemia common |
| What are the symptoms of Wyburn-Mason syndrome | racemose hemangiomas (direct AV connections), no leakage on FA, AVMs in midbrain, posterior fossa, basofrontal, maxilla, pterygoid, mandible, retina doesn't usually bleed |
| What is Miller-Fisher syndrome? | A form of Gulliain-Barre with ataxia, ophthalmoplegia and areflexia. Post viral or vaccine, sensory symptoms with facial diplegia, albuminocytologic dissociation, GQ1b antibodies |
| What are the symptoms of myotonic dystrophy? What is its inheritance? | AD, Chrom 19 type 1.. or 3 type 2, CTG expansion, ophthalmoplegia, ptosis, orbicularis weakness, lid lag, low IOP, christmas tree cataracts, retinal pigment changes, EMG gives diagnosis |
| What are the cardinal manifestations of CPEO? | Progressive ptosis, ophthalmoplegia and occasionally orbicularis weakness leading to exposure keratopathy |
| What are some conditions that CPEO can coincide with? | Kearns Sayers, Oculopharyngeal dystrophy, Bassen-Kornzweig |
| What is Kearns-Sayers disease? | Mitochondrial dx. CPEO, pigmentary retinopathy (<20yo), plus heartblock, elevated CSF protein or cerebellar dysfunction. Can also have deafness, short stature, MR, puberty issues, vestibular dysfunction, endocrine issues, BG calcification |
| What is oculopharyngeal dystrophy? | AD disease of french-canadian ancestory, bulbar involvement (dysphagia) at age 50-60 with CPEO presenting first. can get late stage proximal muscle weakness, not mitochondrial. |
| What is Bassen-Kornzweig syndrome? | Abetalipoproteinemia, acanthocytosis, retinal pigmentary changes |
| What % of all patients with MG will present with ocular signs? What signs are those? | 50% show up with: ptosis, cogan's lid twitch, diplopia, orbicularis weakness |
| What % of all patients with ocular MG will progress to normal MG? How long do you wait? | 80% will progress within 2 years |
| What disease complicates MG frequently? At what rate does it do so? | Graves disease 5-15% of the time coincides |
| How do you diagnose MG? | tensilon test or (prostigmin), EMG, CT for thymus enlargement (10-15% have thymomas), ACh receptor Ab testing. ANA, ESR, TFT |
| What are the treatments for MG? | Mestinon (pyridostigmine), corticosteroids, thymectomy, Imuran/Cyclosporin/Cellcept for immunosuppresion |
| What are the ACh receptor Ab tests you get for MG what % are found usually? | Binding is + in 95% if generalized, 50% if ocular, Blocking Abs can be found 1% of time if binding is negative, modulating should be ordered when binding is negative, also look for muscle specific kinase (Musk) |
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