Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Unusual neurotoxicity associated with amiodarone therapy.

One hundred two patients with recurrent, drug-refractory tachyarrhythmias were treated with amiodarone for nine +/- eight months (mean +/- SD) (range, one to 50 months). Forty-five patients exhibited some form of neurotoxic reaction that was severe enough in nine patients to require discontinuation of treatment or reduction in dosage of the drug. The most frequent neurotoxic findings were tremor (44 patients), peripheral neuropathy (ten patients), and ataxia (seven patients). Five patients developed unusual neurotoxic manifestations: brainstem dysfunction characterized by downbeat nystagmus, hemisensory loss and ataxia, severe dyskinesia, jaw tremor, and proximal myopathy. Neurophysiologic studies revealed varying degrees of predominantly demyelinating peripheral neuropathy. Neurotoxic symptoms improved after discontinuing treatment or decreasing the dosage of the drug. Age of the patient and total cumulative dose did not seem to be risk factors for development of neurotoxicity. These neurotoxic findings suggest that amiodarone-induced neurotoxic reactions are not only confined to the peripheral nervous system, but also that parts of the central nervous system (eg, basal ganglia, brain stem, or their connections) may also be involved.

Dapsone-induced peripheral neuropathy.

A young man with dermatitis herpetiformis developed fatigue and neurologic complaints 4 years after he began oral dapsone therapy. Neurologic examination and nerve conduction studies confirmed the presence of a combined motor and sensory peripheral neuropathy. The symptomatic improvement reported by the patient was supported by improvement in the nerve conduction studies after cessation of dapsone therapy. Substitution of sulfapyridine did not adversely affect the resolution of his neuropathy.

Internuclear ophthalmoplegia after head trauma.

Internuclear ophthalmoplegia results from impairment of the medial longitudinal fasciculus. Multiple sclerosis is usually the cause in bilateral cases while a vascular lesion is commonly implicated in unilateral cases. Head trauma is a rare cause. We describe the case of a 52-year-old man who developed unilateral internuclear ophthalmoplegia following a head injury suffered in an automobile accident. Gradual improvement in the internuclear ophthalmoplegia occurred over a six-month follow-up period. Review of the literature reveals only ten previous reports of internuclear ophthalmoplegia secondary to head injury. In seven of these cases the internuclear ophthalmoplegia was the direct result of the trauma and in three it occurred only after subdural hematoma formation. These ten cases are summarized. Several theories for the pathophysiology of trauma-induced internuclear ophthalmoplegia are presented.

Consistent, effective technique for muscle and nerve biopsy.

Muscle and nerve biopsy provides information of increasing importance in the management of neuromuscular disorders. A simplified technique is presented that yields a specimen which equals and often surpasses in quality specimens obtained by more complex methods that require specially designed clamps and forceps.

Sign language aphasia in a non-deaf-mute.

A 19-year-old left-handed man, who was raised by deaf-mute parents and learned sign language concurrently with normal speech, sustained a traumatic cerebral contusion. He subsequently had no evidence of apraxic, visual-spatial, or sensorimotor deficits of the left limbs with which he was accustomed to use signs. Globally aphasic with a dense right hemiparesis, he initially recovered sign language to a greater degree than spoken language with a reversal on follow-up observations. Receptive skills improved to a greater degree than expressive skills with no marked difference between verbal and sign language, but with natural signs better preserved than finger spelling.

Sensorimotor stroke due to thalamocapsular ischemia.

A 61-year-old hypertensive diabetic man awoke with a numb, heavy right arm and leg; symptoms progressed within 30 hours to a dense right hemisensory syndrome involving head, face, trunk, arm, and leg, accompanied by a right hemiparesis, involving tongue, face, arm, and leg with extensor plantar response, leaving him barely able to move the arm and leg against gravity. No impairment in alertness, memory, language, praxic, or visual functions was evident at any time. Improvement in motor function began in 24 hours and progressed to walking status by discharge on day 23. Eight days passed before the sensory deficit showed improvement, and it was still prominent at discharge. Autopsy three months later showed a 4 X 2 X 4-mm lacune in the ventral posterior nucleus of the left thalamus, with a zone of pallor on stained microscopic sections extending into the immediately adjacent posterior limb of the internal capsule. This case appears to be unique in that a sensorimotor stroke has been produced by a confirmed thalamocapsular infarct.