Download outline25103 - American Academy of Optometry

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Case History
a. Patient Demographics: 55 y.o. Mexican female
b. Chief Complaint: Gradual onset constant, throbbing, retrobulbar pain w/
eye movement associated with a sudden decrease in central vision of the
left eye following diagnosis of cutaneous Herpes Zoster.
c. OHx: presbyopia OU; MHx: HTN, hypothyroidism, Herpes Zoster (1 wk
duration), s/p partial hysterectomy, s/p partial left colectomy sec to
diverticulitis.
d. Medication: acyclovir, prednisone, endocet, levoxyl, self-d/c antihypertensive med
e. Other salient information: Neurologic examination reveals right upper
extremity tremor and tandem gait difficulty. Symptoms progress to
spastic quadriparesis and right eye involvement.
Pertinent Findings
a. Clinical: Visual acuity at presentation OD: 20/20, OS: 10/600; 3+ left
APD, pain w/ extraocular muscle movement OS, dyschromatopsia OS,
central scotoma on Goldmann visual field, normal slit lamp and fundus
exam.
b. Physical: Right upper extremity tremor and tandem gait difficulty;
negative for Herpes Zoster rash.
c. Laboratory studies: CBC w/ diff, ESR, CRP, PT, INR, Serum chemistries,
ACE, ANA, c-ANCA, p-ANCA, Rf, Lyme titer, thyroid functions, HIV,
HbA1c, RPR, WNV, HTLV-1, NMO-IgG Ab, VZV Ab
d. Radiology studies: MRI brain & orbits w/ & w/o contrast, MRI spine,
CXR, MRA
e. Others: LP w/ opening pressure & screening for glucose, protein,
cytology, RBC, IgG, PCR & infectious screen for viral (HSV-1, HSV-2,
VZV, CMV, EBV), bacterial (including cryptococcus), fungal etiology,
MBP, OCB; Goldman visual fields
Differential diagnosis
a. Primary / leading: Disseminated VZV (causing b/l retrobulbar optic
neuritis & quadriparesis)
b. Others: Neuromyelitis Optica, GCA, neoplastic Dz, vasculitic Dz,
neurosarcoidosis, Wegener’s granulomatosis, carotid artery dissection,
MS, meningitis, lupus, HBV, HSV, HIV, Syphilis, Lyme Dz,
Cryptococcosis, Coccidiomycosis, Mycoplasma pneumonia, Aspergillosis,
Cerebral vasculitis, WNV myelopathy, disseminated CNS viral Dz
Diagnosis & Discussion
a. Elaborate on the condition: Neuromyelitis optica (Devic’s syndrome) is
defined as a severe transverse myelitis associated with an acute unilateral
or bilateral optic neuritis without clinical involvement beyond the spinal
cord or optic nerves. The syndrome is characterized by a monophasic
occurrence or multiphasic episodes.
b. Expound on unique features: This case resulted in a high titer serum
NMO-Ab w/ evidence of b/l optic neuritis and myelitis on neuroimaging.
V.
The patient presented with an antecedent cutaneous herpes zoster reaction
which has been documented in the literature in many NMO patients; she
also manifested a previously diagnosed autoimmune disorder
(hypothyroidism). During her clinical course, she presented to the ER w/
hiccups, a clinical sign now recognized in many NMO patients.
Treatment, management
a. Treatment & response to treatment: This patient was initially treated w/ IV
acyclovir and IV methylprednisolone given clinical suspicion for
disseminated VZV. Following positive NMO-Ab results, however, the
patient underwent serum plasmapheresis where she regained upper
extremity mobility w/ remaining paraplegia. Visual acuity remained hand
motion at six inches in the inferior-temporal visual field OD and returned
to 20/40 OS.
b. Research: A serum autoantibody marker of neuromyelitis optica:
distinction from multiple sclerosis was published in the Lancet by Lennon
VA, Wingerchuck DM, Kryzer TJ et al. in 2004 and has proven to be one
of the leading discoveries in NMO diagnosis and was particularly useful in
this case.
c. Bibliography:
Wingerchuk DM. Devic’s disease. 2006 rare neuroimmunologic disorder
symposium. Online: http://www.myelitis.org/devics_disease.htm.
Wingerchuk DM, Hogancamp WF, O’Brien P, et al. The clinical course of
neuromyelitis optic (Devic’s syndrome). Neurology. 1999;53:1107.
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Anchard C, Guinon L. Sur un cas de myelite aigue diffuse avec double
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Ortiz de Zarate JC, Tamaroff C, Sica RE, et al. Neuromyelitis optica
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changes. Arch Neurol. 1984;41:1103-1105.
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Neurol. 1999;46:878-886.
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VI.
Conclusion
a. Clinical pearls: The previously diagnosed cutaneous Herpes Zoster was a
red herring in this case & persistence in re-evaluating DDx was inherently
important when the laboratory results did not support the clinical picture.
Knowledge of current neurologic research was highly important as well.