Fits and deafness

Abstract

3(65% monocytes) but no glucose decrease. CSF culture was negative. He was treated with multiple antiviral and antimicrobial drugs, including acyclovir, ampicillin, ceftriaxone, isoniazid, ethambutol, rifampicin, and pyrazinamide, without effect. Drug eruption occurred during therapy and was treated with oral prednisolone (30 mg/day). This improved the CSF abnormalities and stopped his seizures. Reduction of the steroid dose caused a recurrence of the seizures. He was transferred to our hospital for further evaluation. On admission, he was taking 15 mg prednisolone and did not have seizures. Neurological examination showed slight right abducens paresis, right peripheral facial paresis, bilateral papilloedema, right sensorineural deafness and complete loss of vestibular function on the right side. Right caloric response was absent, and an otoacoustic emission was absent unilaterally in the right ear, indicating that the outer hair cells of the cochlea were damaged. Papilloedema persisted despite normalised intracranial pressure. An ophthalmological examination found mild papillitis and serous retinal detachment, and leakage of dye during fluorescein angiography confirming posterior uveitis. He did not have apparent alopecia but he experienced a diffuse hair loss. A search for skin lesions under his drug-erupted, brownish skin detected small depigmented spots on his neck. Fontana-Masson staining of a biopsy of his colourless skin showed increased melanophages and decreased melanocytes, which indicated that the skin lesion was vitiligo rather than non-specific depigmentation. Marked inflammatory cell infiltration was seen around hair follicles in the biopsy. An electroencephalogram showed a left parieto-occipital sharp wave. Gadolinium-enhanced magnetic resonance imaging showed pronounced bilateral contrast enhancement of the meninges and an oedematous lesion in the left frontoparietal cortices (figure), compatible with meningoencephalitis. T2-weighted images showed a periventricular hyperintensity lesion. HLA-typing found A24, A2, B7, B67, Cw7, DR1, and DR4. HLA–DNA typing by the PCR–RFLP method found HLA