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1/19. acetazolamide improves action myoclonus in Ramsay Hunt syndrome.

    The myoclonus of two patients with Ramsay Hunt syndrome was only partially controlled under treatment with clonazepam, sodium valproate, primidone, and piracetam. acetazolamide (200 mg daily) was added to these drugs, resulting in a dramatic improvement. Placebo substitution (one patient) and withdrawal of acetazolamide in the other patient resulted in marked aggravation of the myoclonus. The mechanism of action of acetazolamide in myoclonus is unknown. acetazolamide may be an additional therapeutic possibility for patients with severe action myoclonus. ( info)

2/19. acyclovir responsive brain stem disease after the Ramsay Hunt syndrome.

    We report an immunocompetent patient with the Ramsay Hunt syndrome (RHS) followed days later by brainstem disease. Extensive virological studies proved that varicella zoster virus (VZV) was the causative agent. Treatment with intravenous acyclovir resulted in prompt resolution of all neurological deficits except peripheral facial palsy. This case demonstrates that after geniculate zoster, brainstem disease may develop even in an immunocompetent individual and effective antiviral therapy can be curative. ( info)

3/19. myoclonus and sensorimotor integration in a patient with Ramsay Hunt syndrome.

    Clinical and neurophysiologic studies were done on a patient with action myoclonus secondary to Ramsay Hunt syndrome (dyssynergia cerebellaris myoclonica). Myoclonic jerks in the arms were much more common during movements directed to a target than in other movements. They appeared to be triggered primarily by external sensory inputs relevant to the movement rather than by the motor activity itself. Both somatosensory and visual inputs appeared able to trigger the myoclonic jerks. Myoclonic jerks in the deltoid muscle followed finger contact with a target by approximately 100 msec. Electrical stimuli delivered to the fingers during a reaching movement also triggered myoclonic jerks with a similar latency and also evoked giant cortical potentials which preceded the myoclonic jerks in deltoid by 15-20 msec. Our results suggest that during sensory guided movements, sensory inputs relevant to successful completion of the movement may have access to motor systems controlling the muscles involved. In our patient, who likely has lesions involving the cerebellar nuclei and/or cerebellar cortex, these sensory inputs appeared to result in an excessive motor response, possibly through mechanisms involving cerebellar-motor cortex connections. ( info)

4/19. Late-onset tacrolimus-associated cerebellar atrophia in a heart transplant recipient.

    tacrolimus is a macrolide immunosuppressant frequently used after solid-organ transplantation. Moderate and severe neurologic side effects have been reported in patients receiving tacrolimus. Cerebral neurotoxicity is a rare but fatal calcineurin inhibitor-related complication, especially in kidney and liver transplant recipients. Often a reduction or a change in immunosuppressive regimen is the only means of clinical management. Herein we report a case of a 31-year-old man who developed cerebellar atrophia while under immunosuppressive therapy 9 years after heart transplantation. His neurologic constitution ameliorated after an immunosuppressant switch from tacrolimus to sirolimus. ( info)

5/19. Ramsay Hunt syndrome with mental disorder.

    This is a case of Ramsay Hunt syndrome with mental disorder. The patient had action myoclonus, grand mal seizure and severe cerebellar ataxia. schizophrenia-like symptoms including delusion of persecution and self-reference, auditory hallucination and incoherence were characteristically observed before the neurological disturbance became manifest. Subsequently, euphoria, disinhibition, moria and mild dementia appeared with neurological symptoms. The possibility of Ramsay Hunt syndrome to accompany organic mental syndromes and the relationship between cerebellar dysfunction and psychiatric symptoms are discussed. ( info)

6/19. Cerebellorubral degeneration after resection of cerebellar dentate nucleus neoplasms: evaluation with MR imaging.

    The authors describe the magnetic resonance (MR) findings in seven patients who developed severe cerebellar symptoms and atrophy of the contralateral red nucleus following removal of unilateral neoplasms in the deep nuclei of the cerebellum. For most patients, pre- and postoperative spin-echo MR images were obtained with long repetition times (TRs) at 1.5 T. The long TR images obtained before surgery demonstrated unilateral masses involving the dentate nucleus. Long TR images obtained after surgical resection of the dentate nucleus showed increased signal intensity in all of seven contralateral red nuclei, three of seven ipsilateral superior cerebellar peduncles, and two of seven contralateral inferior olivary nuclei. Three other patients who underwent surgery for cerebellar neoplasms without resection of the dentate nuclei showed no postoperative brain stem changes on MR images. The authors speculate that the changes in the contralateral red nuclei are due to cerebellorubral degeneration (since well-described neural tracts interconnect the dentate nucleus and the contralateral red nucleus). Injury of the dentate nucleus may result in degeneration of distant neural connections. ( info)

7/19. Ramsay Hunt syndrome and coeliac disease: a new association?

    Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin e level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy. ( info)

8/19. Ramsay Hunt syndrome in dentatorubral-pallidoluysian atrophy.

    We report a case of Ramsay Hunt syndrome which was clinically characterized by myoclonus epilepsy, cerebellar ataxia, convulsions, and dementia. Major necropsy findings were dentatorubral-pallidoluysian atrophy. Dentatorubral-pallidoluysian atrophy may be associated with a variety of clinical symptoms, amongst which Ramsay Hunt syndrome can be included. ( info)

9/19. A neurologist's approach to a patient with hearing impairment.

    This article is a review of the central auditory pathways from an anatomic and clinical perspective. An approach to the assessment of patients with hearing impairment of central origin is provided. The review of the patient's history, physical examination, and laboratory assessment are dealt with in detail. ( info)

10/19. Preparalytic condition of the facial nerve detected by evoked electromyography.

    An abnormal condition of the facial nerve just before apparent paralysis was detected by evoked electromyography. We now report on 2 patients with herpes oticus and vertigo or tinnitus without apparent facial palsy. This abnormal condition is considered to be a subclinical paralysis (preparalytic condition), and early detection of the condition may prevent ensuing paralysis. ( info)
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