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1/176. adult-onset MELAS presenting as herpes encephalitis.

    OBJECTIVE: To report an unusual presentation of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) manifested in late life with a clinical picture of herpes simplex encephalitis. DESIGN: Case report. SETTING: Clinical neurology department in a tertiary care hospital. CASE DESCRIPTION: A 55-year-old woman developed aphasia and delirium during ophthalmic herpes zoster infection treated with oral prednisone and ophthalmic steroids, which was followed by progressive cognitive decline without acute neurologic events for 5 years. At age 60, the patient presented with new onset of seizures, hemiparesis, and hemianopsia. Subsequently she developed cortical blindness, multiple traumatic soft tissue injuries from falls, acute psychosis, and severe dementia with periods of agitation. She died in a nursing home in March 1997, 6 years after initial presentation. RESULTS: magnetic resonance imaging scan of the brain showed hyperintensity on T2-weighted images involving temporal, parietal, and occipital lobes bilaterally as well as mild atrophy of brainstem and cerebellum. Single photon emission computed tomographic imaging showed hypoperfusion of temporal, parietal, and occipital lobes. Results of video electroencephalographic monitoring showed periodic lateralizing epileptiform discharges in temporal and occipital areas. The serum lactate level was normal in May 1996 and elevated in October 1996. The creatine kinase level was elevated with a 100% MM fraction in August 1991 and normal in March 1996. Results of repeated cerebrospinal fluid analyses indicated elevated protein levels. Analysis of dna was diagnostic of MELAS by mitochondrial dna point mutation at position 3243. The results of autopsy showed moderate cerebral, cerebellar, and brainstem atrophy with signs of infarction in temporal and parietal lobes bilaterally. CONCLUSIONS: The clinical presentation as well as age at onset of MELAS are highly variable. Onset of mitochondrial disorders can be provoked by febrile illness when there is mismatch between energy requirements and availability. In the differential diagnosis of herpes encephalitides, melas syndrome should be considered. ( info)

2/176. stroke after zoster ophthalmicus in a 12-year-old girl with protein c deficiency.

    A 12-year-old girl who had zoster ophthalmicus 10 months earlier presented with hemiparesis and corresponding basal ganglionic infarction related to middle cerebral artery branch thrombosis ipsilateral to the zoster. Hematologic evaluation disclosed protein c deficiency. This represents the first zoster-associated stroke reported in childhood associated with protein c deficiency, with extension of the latency period between zoster and infarction, previously reported to be 6 months. ( info)

3/176. Temporal artery biopsy in herpes zoster ophthalmicus with delayed arteritis.

    A 58-year-old man developed herpes zoster ophthalmicus with delayed hemiparesis. Temporal artery biopsy confirmed the presence of a vasculitis. Electron microscopy of the temporal artery failed to reveal viral particles. herpes zoster ophthalmicus with delayed arteritis appeared to be a contiguous spread of vasculitis to the carotid system and not a direct viral invasion. ( info)

4/176. Typical varicella zoster (ophthalmicus) in an hiv-infected person.

    A typical varicella zoster (ophthalmicus) in an incidentally hiv-infected person is reported in a young man. It was characterized by tense, grouped, vesiculobullous eruptions on a brick-red base. The diagnosis was substantiated by demonstration of swollen epidermal (balloon) cells with a nucleus/several nuclei containing inclusion bodies. Reticular degeneration was apparent. ( info)

5/176. zoster sine herpete with bilateral ocular involvement.

    PURPOSE: To report a case of zoster sine herpete with bilateral ocular involvement. METHOD: Case report. RESULTS: A 65-year-old man showed bilateral iridocyclitis with sectoral iris atrophy and elevated intraocular pressure unresponsive to steroid treatment. No cutaneous eruption was manifest on the forehead. A target region of varicella-zoster virus dna sequence was amplified from the aqueous sample from the left eye by polymerase chain reaction. Bilateral iridocyclitis resolved promptly after initiation of systemic and topical acyclovir treatment. Secondary glaucoma was well controlled by bilateral trabeculectomy. CONCLUSIONS: zoster sine herpete should be considered and polymerase chain reaction performed on an aqueous sample to detect varicella-zoster virus dna for rapid diagnosis whenever anterior uveitis accompanies the characteristic iris atrophy, even in the case of bilateral involvement. ( info)

6/176. Herpes zoster sine herpete presenting with hyphema.

    PURPOSE: To report a case of herpes zoster sine herpete presenting with hyphema. methods: A 69-year-old man was referred for traumatic hyphema and corneal edema in his left eye after a sandblast exposure three weeks previously. Slit-lamp examination demonstrated hyphema, anterior chamber inflammation, mid-dilated pupil, impaired corneal sensation, and high intraocular pressure, without any facial skin lesions. iris fluorescein angiography revealed tortuosity and extensive occlusion of iris vessels. The patient was treated with oral acyclovir and intensive topical steroids with a presumed diagnosis of severe herpes zoster uveitis. RESULTS: Clinical findings improved dramatically within several days. Typical sectorial iris atrophy with pupillary sphincter dysfunction and complete loss of corneal sensation developed after the resolution of intraocular inflammation. CONCLUSION: Herpes zoster should be considered in patients with uveitis and hyphema even in the absence of typical skin rash. ( info)

7/176. optic neuritis in herpes zoster ophthalmicus.

    BACKGROUND: optic neuritis in herpes zoster ophthalmicus (HZO) has been reported rarely. We report two cases of HZO optic neuritis with detailed magnetic resonance imaging study and treatment responses. CASES: One patient presented with anterior optic nerve involvement, and the second presented with retrobulbar optic neuritis. Contrast enhanced T(1)-weighted images were obtained in these 2 patients. Intravenous acyclovir and oral prednisolone were given simultaneously. OBSERVATIONS: magnetic resonance imaging revealed peripheral enhancement of the optic nerve sheath complex on T(1)-weighted scan. Both patients recovered their vision within 3 months following the start of treatment. CONCLUSIONS: magnetic resonance imaging is helpful for the diagnosis of HZO optic neuritis. Systemic acyclovir and steroid are effective in the treatment of HZO optic neuritis. ( info)

8/176. Acute retinal necrosis following contralateral herpes zoster ophthalmicus.

    BACKGROUND: A case report of contralateral acute retinal necrosis (ARN) following herpes zoster ophthalmicus. CASE: A 61-year-old male patient developed iridocyclitis and well-demarcated creamy-white retinal lesions at the nasal periphery in the right eye 1 month after herpes zoster ophthalmicus in the left eye. The patient had undergone surgery for primary lung cancer, and had subsequent intracranial metastasis of the tumor. OBSERVATIONS: The clinical diagnosis of ARN was supported by polymerase chain reaction investigation of the aqueous humor resulting in positive for varicella-zoster virus. Retinal lesions disappeared after systemic treatment with acyclovir, corticosteroids, and acetylsalicylate. No retinal detachment developed. CONCLUSIONS: We propose a careful ophthalmic follow-up for herpes zoster ophthalmicus patients because of the possibility of acute retinal necrosis developing in the contralateral eye. ( info)

9/176. Acute hemiplegia associated with herpes zoster infection in children: report of one case.

    Herpes zoster infection has been rarely reported to cause angiitis of the central nervous system in children. We describe a 4-year, 8-month-old female with acute hemiplegia and central facial palsy 6 weeks after she had had zoster ophthalmicus. The findings of magnetic resonance angiography, the clinical picture, and a preceding history of herpes zoster ophthalmicus suggested zoster vasculitis. Herpes zoster vasculitis is thus another consideration when examining a child with acute hemiplegia and a recent herpes zoster infection. ( info)

10/176. optic chiasm, optic nerve, and retinal involvement secondary to varicella-zoster virus.

    Immunocompromised patients are known to be at risk for varicella-zoster virus reactivation, often in atypical manners. We describe a 30-year-old man with simultaneous involvement of the retina, optic chiasm, and optic nerve with varicella-zoster virus who had a bitemporal visual field defect. ( info)
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