Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
I. II. III. IV. 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. Kuroiwa Y. Neuromyelitis optica (Devic’s disease, Devic’s syndrome). In: Koetsier JC (ed.): Clinical Neurology. New York: Elsevier Science, 1985:397-408. Poser CM. Neuromyelitis optica. In: Clinical Neurology. Hagerstown: Harper & Row, 1983:70. Shibasaki H, Kuroda Y., Kuroiwa Y. Clinical studies of multiple sclerosis in Japan: Classical multiple sclerosis and Devic’s disease. J Neurol Sci. 1974;23:215-222. Wingerchuk DM & Weinshenker BG. Neuromyelitis optica: Clinical predictors of a relapsing course and survival. Neurology. 2003;60:848853. Weinshenker BG. The natural history of multiple sclerosis. Neurol Clin. 1995;13:119-146. Mandler RN, Davis LE, Jeffery DR, et al. Devic’s neuromyelitis optica: A clinicopathological study of eight patients. Ann Neurol. 1993;34:162168. O’Roidan JI, Gallagher HL, Thompson AJ, et al. Clinical, cerebrospinal, and magnetic resonance imaging findings in Devic’s neuromyelitis optica. J Neurol Neurosurg Psychiatr. 1996;60:382-387. Fazekas F, Offenbacher H, Schmidt R, et al. MRI of neuromyelitis optica: Evidence for a distinct entity [letter]. J Neurol Neurosurg Psychiatr. 1994;57:1140-1142. Misu T, Fujihara K, Nakashima I, et al. Intractable hiccups and nausea with periaqueductal lesions in neuromyelitis optica. Neurol. 2005;65:1479-1482. Chalumeau-Lemoine L, Chretien F, Gaëlle Si Larbi A, et al. Devic disease with brainstem lesions. Arch Neurol. 2006;63:591-593. Tourtellotte WW, Staugaitis SM, Walsh MJ et al. The basis of intrablood-brain-barrier IgG synthesis. Ann Neurol. 1985;17:21-27. Lennon VA, Wingerchuk DM, Kryzer TJ et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004:11-17;364(9451):2106-2112. Blanche P, Diaz E, Gombert B et al. Devic’s neuromyelitis optica and HIV-1 infection. J Neurol Neurosurg Psychiatr. 2000;68:795-796. Merle H, Smadja D, Cordoba A. Neuromyélite optique et nécrose rétinienne aigue bilatérale d’origine zostérienne chez une patiente atteinte du SIDA. J Fr Ophthalmol. 1998;21:381-386. Lucchinetti CF, Mandler RN, McGavern D, et al. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain. 2002;125:1450-1461. Anchard C, Guinon L. Sur un cas de myelite aigue diffuse avec double nevrite optique. Arch de Med Exper er d’Anat Path. 1889;1:696-710. Stansbury FC. Neuromyelitis optica (Devic’s disease). Presentation of 5 cases with pathological study and review of literature. Arch Ophthalmol. 1949;42:292-335. Goulden C. Optic neuritis and myelitis. Trans Ophthal Soc UK. 1914;34:229-252. Beck GM. A case of diffuse myelitis associated with optic neuritis. Brain. 1927;50:687-703. Correale J, Fiol M. Activation of humoral immunity and eosinophils in neuromyelitis optica. Neurology. 2004;63:2363-2370. Ortiz de Zarate JC, Tamaroff C, Sica RE, et al. Neuromyelitis optica versus subacute necrotic myelitis. II. Anatomical study of two cases. J Neurol Neurosurg Psychiatr. 1968;31:641-645. Lefkowitz D, Angelo JN. Neuromyelitis optica with unusual vascular changes. Arch Neurol. 1984;41:1103-1105. Weinshenker BG, O’Brien PC, Petterson TM et al. A randomized trial of plasma exchange in acute CNS inflammatory demyelinating disease. Ann Neurol. 1999;46:878-886. Mandler RN, Ahmed W, Dencoff JE. Devic’s neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurol. 1998;51:1219-1220. Itoyama Y, Saida T, Tashiro K, et al. Japanese multi-center, randomized, double-blind trial of interferon beta-1b in relapsing-remitting multiple sclerosis: 2-year results. Ann Neurol. 2000;48:487. Gupta S, Jain A, Gardiner C, et al. A rare case of disseminated cutaneous zoster in an immunocompetent patient. BMC Fam Pract. 2005;6:50. 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.