Neuro ophthalmological diagnoses you can’t afford to miss

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  • Stiff, looking straight ahead.Felt that upbeat nystagmus may represent somnolent gaze
  • No titubation, trunkal ataxia or limb ataxia.Neurologist also noted the vertical nystagmus
  • Korsakoff amnestic syndrome is a late neuropsychiatric manifestation of Wernicke encephalopathy with memory loss and confabulation; sometimes, the condition is referred to as Wernicke-Korsakoff syndrome or psychosis.
  • In the Western world, thiamine deficiency is characteristically associated with chronic alcoholism, because it affects thiamine uptake and utilization. However, Wernicke encephalopathy may develop in nonalcoholic conditions, as in prolonged starvation, hyperemesis gravidarum, bariatric surgery, HIV-AIDS, and even in healthy infants given the wrong formulas.Frequently unrecognized, Wernicke encephalopathy is more prevalent than commonly supposed.
  • The oculomotor signs are nystagmus, bilateral lateral rectus palsies, and conjugate gaze palsies reflecting cranial nerve involvement of the oculomotor, abducens, and vestibular nuclei. Less frequently noted are pupillary abnormalities such as sluggishly reactive pupils, ptosis, scotomata, and anisocoria.
  • Neuro ophthalmological diagnoses you can’t afford to miss

    1. 1. David M Katz, MD Bethesda Neurology, LLC7830 Old Georgetown Rd, C-20 Bethesda, MD 20814 301.540.2700
    2. 2. Patient #1 73-year-old healthy female seen 2/20/13 complaining of sinus pressure around her eyes and blurred vision OD for several weeks  Internist prescribed two rounds of antibiotics for a presumed sinus infection  Vision blurred OD 3 weeks ago  Transient blurred vision OS 10 days ago  CT orbits ordered by PMD normal
    3. 3. Patient #1 Sees optometrist and ophthalmologist:  Vacc 20/400 OD, 20/20 OS  No relative afferent pupillary defect (APD)  Confrontation visual fields full OU  Funduscopic exam: normal, no optic disc edema or atrophy
    4. 4. Patient #1 Differential Diagnosis?  Infection? (no pain, proptosis, injection, normal CT sinuses)  Bilateral retrobulbar optic neuritis? (too old)  Anterior ischemic optic neuropathy/AION? (no disc swelling or heme)  Functional visual loss? (no secondary gain)  Chiasmal lesion? (no bitemporalhemianopsia)  Retrogeniculate lesion? (no homonymous hemianopsia)  Toxic optic neuropathy? (not symmetrical, no atrophy)  Leber’s Hereditary Optic Neuropathy? (too old, wrong sex, normal appearing nerves)  Posterior ischemic optic neuropathy/PION? (no blood loss, no recent surgery or severe trauma) Why no RAPD? Because both optic nerves must be affected or it’s retinal or functional Work up? (hint, a $10 test)
    5. 5. Patient #1 ESR=95 (nl<40 for age) CRP=108 (nl<8) Diagnosis: Giant Cell Arteritis Work up: unilateral 2.5 cm temporal artery biopsy positive (done 2/22/13) Treatment: IV methylprednisolone (Solumedrol) 1 g/d x 5 days, then oral prednisone 100 mg/d Bi-weekly ESR/CRP and taper prednisone according the lab results and residual symptoms
    6. 6. Temporal Artery Biopsy: H&E stain
    7. 7. Temporal artery biopsy Thickening of the intima, necrosis of the internal elastic lamina and media and lymphocytic infiltration of the vessel wall with giant cells are seen acutely. Biopsies of patients on steroids >2 weeks show disruption of the internal elastic lamina but few or no lymphocytes or giant cells, termed “healed arteritis”. A temporal artery biopsy specimen > 2 cm confirms the diagnosis in 95% of cases. If negative, a contralateral biopsy has a yield of only 3% (Boyev 1999).
    8. 8. Arteriticischemic optic neuropathy Vision loss from GCA is most often due to ischemic optic neuropathy (ION), either A(nterior)ION (with disc swelling and heme) or P(osterior)ION (without) as in this patient GCA can also cause CRAO or choroidalinfarctions GCA patients tend to be older (mean age=75 years) than non-arteritic ION patients (mean age=57 years)
    9. 9. GCA diagnosis Symptoms: headache, scalp tenderness, jaw claudication, swollen and pulseless superficial temporal arteries, PMR, fatigue, weight loss, fever, malaise, transient monocular blindness, diplopia Elevated ESR present in >90% of patients
    10. 10. GCA treatment High dose steroids must be initiated as soon as the diagnosis is considered, don’t wait for TABx results Return of vision after steroid initiation is <15% There is anecdotal evidence that high dose IV steroids (methylprednisolone IV 1 g x 5 days) may be more effective than high dose oral steroids (ex. 100mg prednisone) at preventing further vision loss . Patients with recent and/or bilateral visual loss are better candidates for IV steroids until a well designed study proves otherwise Delayed diagnosis of GCA is the #1 cause of medical malpractice in ophthalmology (and I assume optometry)
    11. 11. Arteritic AION NA-AION
    12. 12. NA-AIONArteritic ION  Mean age=57  Mean age=75  Va>20/60 in 50%  Va<20/200 in 70%  Hyperemic disc swelling  Pallid disc swelling  Small or absent cup  Cups of any size  Second eye involvement in 15% at 5 years  Second eye involvement in 50%: 1/3 within 1 day, 1/3 within 1 week and1/3 within 1 month  Cup enlarges once edema  Cup unchanged once edema resolves resolves  FA shows late disc leakage  FA shows late disc leakage and  Cotton wool spots and retinal patch choroidal non-perfusion infarcts are very rare  CWS very common
    13. 13. Patient #2 65 yo hypertensive female presents with painless, progressive unilateral ptosis and binocular diplopia and generalized headache for two weeks
    14. 14. What’s the problem and what’s the cause?
    15. 15. Diagnosis? Pupil involved, partial right III nerve palsy Cause?  Microvascular “diabetic” III (not a complete, pupil sparing III n palsy)  Midbrain stroke (no hemiparesis or ataxia, III nerve progressive, not acute in onset)  Myasthenia gravis (pupil involved)  Restrictive (no proptosis, pain or eyelid retraction)  Compressive lesion (tumor or aneurysm) must be excluded immediately
    16. 16. A right third nervepalsy caused by aruptured right posteriorcommunicating artery(Pcom) aneurysm. TheCT shows subarachnoidblood in thequadrigeminal cisternand the arteriogramshows a PcomaneurysmEndovascular coilingsuccessfulIII nerve palsy resolvedover several months
    17. 17. III nerve palsy evaluation Pupil-involved III nerve palsy or progressive III nerve palsy: requires immediate attention. Start with an MRI + gad and MRA brain. If normal and suspicion is still high, proceed with CT angiogram (CTA). Use LP if imaging studies are non-diagnostic and suspicion still high for SAH. Cerebral arteriogram with endovascular coiling treatment of choice for aneurysms with small necks Acute partial III nerve palsy without headache: start with MRI and MRA. If pupil spared, check pupils daily and if remains normal, CTA or LP not needed. If pupil dilates, proceed with CTA or arteriogram Pupil-sparing complete III nerve palsy: if isolated in a >40-year-old check for diabetes and HTN. Do not need to proceed with emergency imaging. If doesn’t improve by 2 months, proceed with MRI and MRA. If >60, especially with new onset headache, check ESR and CRP for giant cell arteritis Aberrant regeneration of III nerve: eyelid retraction in adduction and/or infraduction, miosis in adduction. Only caused by compression (aneurysm, tumor, prior trauma) and requires imaging study if no trauma history
    18. 18. Case #3 22-year-old previously healthy female was admitted to her local hospital in Portsmouth, VA with progressively worsening confusion, lethargy, gait instability which, according to her family began several weeks prior (patient unable to give history) An inpatient ophthalmology consult was requested to evaluate abnormal eye movements noted by the family, ICU staff and neurology consultant
    19. 19. History of Present Illness Two weeks prior to admission, patient presented to her local ER with weakness, fatigue, blurred vision, gait instability, memory loss and dizziness.  59 lb weight loss since bariatric surgery three months prior  BP 100/70, P=114  Diagnosis: “non-specific vertigo”. Discharged on meclizine
    20. 20. Medical History Past medical history  Morbid obesity, BMI=44 (obese>30)  Obstructive sleep apnea, not using CPAP  Hypercholesterolemia Past surgical history  Laproscopic Roux-en-Y gastric bypass three months prior Past ocular history  Strabismus surgery age 5 for congenital exotropia. Baseline visual acuity (VA) 20/20 OD, 20/50 OS Medications  Multi-vitamin, iron, oral B12 Allergies  None known Family history  Diabetes, obesity, glaucoma, coronary artery disease Social history  Denied alcohol, tobacco, illicit drugs. Full-time college student
    21. 21. Neuro-Ophthalmological Exam on Admission Visual acuity uncorrected: 20/100 OU at near Confrontation visual fields: full OU Ocular motility:  Intermittent upbeat nystagmus in primary position OU  Impaired supraduction OU  Normal convergence  Alternating exotropia Pupils: No RAPD, 2.5 mm in darkess, 1+ reactive to light and near OU Intraocular pressure: 15 OD, 17 OD via Tonopen Biomicroscopic exam: unremarkable Fundus: unremarkable optic discs, vessels and retinae
    22. 22. Neurologic Exam Drowsy but arousable. Gave short answers, oriented to person and “hospital”, not time. Poor short term memory, no aphasia Cranial nerves: V, VII-XII intact Motor: 3/5 strength throughout but “poor effort” noted Sensory: patient reacted to pin bilaterally Reflexes: 1/4 and symmetrical with flexor plantar responses Coordination: finger-to-nose dysmetric, no tremor Gait: severe ataxia, could not ambulate without assistance  T=100.7, P=123, BP=133/95, RR=19
    23. 23. Additional Testing Cranial CT unremarkable Electroencephalogram unremarkable Lumbar puncture:  RBC 7  WBC 4  Protein 64 (nl<46)  Glucose 85  Gram’s stain, culture, acid-fast stain, cryptococcal antigen, Lyme titer all negative  Vitamin B12 1750 (nl>250), folate 11 (nl>6), copper 133 (nl 80-163) looking for Wilson’s Disease
    24. 24. Patient’s Axial FLAIR MRI
    25. 25. Cranial MRI Interpretation“Bilateral symmetric, nonenhancing T2 signal abnormalitiesin the thalami, periaqueductal gray matter andhypothalamus. Diffusion weighted MRI unremarkable”Diagnosis: highly suggestive of variant Creutzfeldt-JakobDisease (vCJD). Most likely human case of Mad CowDisease.CDC contacted. No other cases reported in US. The threeknown US cases had all lived in England prior to moving toUS.
    26. 26. Final trip to hospital Diagnosed with variant Creutzfeldt-Jakob/Mad Cow Disease (vCJD/MCD) and discharged home to die. Life expectancy several months Two weeks later returned to ER with hypotension, tachycardia, respiratory distress, unresponsive  Pronounced dead from cardiopulmonary arrest in ICU 12 hours later at age 22
    27. 27. Politics of Mad Cow Disease South Korea banned importation of US beef in 2003 because of fears of human transmission of the prion that causes mad cow disease Several days before this patient was diagnosed with vCJD the South Korean prime minister signed an agreement with the US government to end the ban, without input from the general public Once this case went public, 10,000 South Koreans held protests for 40 days, leading to the resignation of 9 ministers The agreement was upheld but with an amendment that the US would randomly test 1% of cows in perpetuity because of this case
    28. 28. Autopsy Post mortem brain biopsy specimen sent to National Prion Disease Pathology Surveillance Center at Case Western University  Western blot and histopathology did not show prion disease  Focal petechial hemorrhages and focal inflammation of small vessels and increased cellularity and disorganization of the vessel walls in the pontineperiventricular region and mamillary bodies  Acute petechial hemorrhages, severe edema and relative neuronal preservation  Acute Wernicke’s encephalopathy due to thiamine deficiency
    29. 29. Wernicke’s Encephalopathy Due to thiamine (vitamin B-1) deficiency Carl Wernicke, a Prussian neuro-pathologist, first reported a case in 1881 as a triad of acute mental confusion, ataxia, and ophthalmoplegia Korsakoffamnestic syndrome: memory loss and confabulation in survivors of Wernicke’s
    30. 30. Wernicke’s Encephalopathy Associations  chronic alcoholism  prolonged starvation  hyperemesisgravidarum  bariatric surgery  HIV-AIDS  healthy infants given the wrong formula  Carbohydrate exposure a common trigger
    31. 31. Wernicke’s Encephalopathy: Ocular Abnormalities Signs:  Nystagmus  Bilateral abduction palsies  Conjugate gaze palsies Less frequently noted are pupillary abnormalities such as sluggishly reactive pupils, ptosis, scotomata, and anisocoria
    32. 32. Summary AION, PION, CRAO or diplopia with headache >50 years of age, get stat ESR and non-cardiac CRP even if optic disc normal (PION). Start steroids as soon as GCA suspected, “shoot first and ask questions later”. “Rule of the III nerve”: pupil sparing complete III in vasculopath, OK to watch pupil. Progressive III or incomplete III or pupil involved III, get stat MRI and MRA, then perhaps CTA and LP. Abnormal eye movements, ataxia and confusion= Wernicke’s until proven otherwise. Bariatric surgery #1 cause, severe alcoholism is now #2.
    33. 33. Thank YouDavid M Katz, MD New Yorker 5/16/12

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