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In this paper we report on the application of dual-wavelength photodynamic therapy with a topical chlorin-based photosensitizer for treatment of Ramsay Hunt syndrome in a patient with HIV. Traditional treatment approach (combination of acyclovir and a glucocorticosteroid) failed to provide a significant outcome, while photodynamic therapy resulted in fast positive dynamics. No recurrence was observed in a 5-month-long follow-up.

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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.

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Acetazolamide treatment significantly improves action myoclonus in Ramsay Hunt Syndrome. A family with two brothers and a sister, and a sporadic case with Ramsay Hunt Syndrome and uncontrollable action myoclonus, are described where addition of oral acetazolamide lead to marked improvement in their action myoclonus.

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Varicella-zoster virus has been associated with a variety of neurological manifestations. We describe a patient with the Ramsay Hunt Syndrome who developed a contralateral cerebral infarction.

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Varicella-zoster virus has been associated with a variety of neurological manifestations. We describe a patient with the Ramsay Hunt Syndrome who developed a contralateral cerebral infarction. (AU)

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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.

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Herpes zoster oticus has a poor prognosis with permanent facial nerve dysfunction. We have studied 11 patients in a total group of 131 facial palsies seen in our service for a 20 month period. Four patients had no treatment. Two were treated with prednisone, and five were infused with acyclovir (10 mg/kg every 8 hours over a 10 day hospitalization period) and prednisone. These treatments were observed for 3 to 6 months. Patients follow up was, acyclovir group, 2 patients have achieved a House grade I, 1 patient grade II, 1 patient grade III and 1 left this treatment because side effects was observed. The other patients, 1 have achieved grade I, 2 patients grade II, 2 patients grade III and 1 patient grade V. The differences and final results are discussed.

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A six-month-old girl receiving erythromycin was given carbamazepine treatment. Two days later signs of carbamazepine intoxication developed and a serum carbamazepine concentration of 30 mg/l was found (4-9 mg/l being the desired therapeutical level). The interaction of erythromycin and carbamazepine by liver enzyme competition is clinically significant.

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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.

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We studied 28 patients with herpes zoster oticus prospectively over the six-year period between August 1974 and June 1980. We found that the results of measuring tear production, submandibular salivary flow, the response to maximal stimulation, and evoked electromyography gave us sufficient information to group these patients according to prognosis--either unfavorable or favorable--for spontaneous return of facial function. When the test results were 26% or more of normal, 100% of the patients had complete recovery without treatment; when the test results were 25% or less of normal, 69% had incomplete recovery: 19% had fair and 50% had poor recovery. Of the 31% with complete recovery, 4 were operated upon. The natural history of herpes zoster oticus in patients with a poor prognosis was improved if a transmastoid extralabyrinthine subtemporal decompression of the labyrinthine segment of the facial nerve was performed within 10 days of onset of the paralysis. The decision to perform this surgery was based upon the results of the prognostic tests mentioned above.

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Genetic, clinical, polyphysiographic and therapeutical aspects were investigated in a 37 year old patient suffering from: sporadic and localized segmentary myoclonia (present during rest and increasing slightly during movement), cerebellar syndrome, marked hypoacusia, hyper-reflexia of the lower limbs, spike and polyspike EEG discharges and massive myoclonia during photostimulation. A dominant autosomic inheritance of this syndrome was suggested by the occurrence of a more serious and rapid, yet similar, illness in the mother and by EEG alterations in an 8 year old son. The Authors suggest that, from the clinical point of view, they are dealing with an atypical Ramsay-Hunt syndrome (MCD) with signs of Pierre-Marie Heredoataxia. This supports the possibility, however rare, of intermediate forms between MCD and Heredoataxia. The patient underwent polyphysiographic investigations under the following conditions: i) without therapy; ii) with diphenylhydantoin; iii) with phenobarbital; iv) with sodium dipropyl-acetate; v) with clonazepam. The above investigations confirmed the previously described alterations, during wakefulness or sleep, observed in cases of Unverricht-Lundborg syndrome (PME) and Ramsay-Hunt syndrome (MCD). Clonazepam was found to be the most effective drug in attenuating myoclonia, in reducing the EEG spike activity and in reorganizing the phases and cycles of sleep.

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Ten patients affected by various myoclonic syndromes were tested with drugs acting on cerebral serotonin metabolism and with clonazepam (CZP). After L5HTP or serotonergic drugs administration a clear cut improvement was observed in the 2 patients affected by Ramsay-Hunt syndrome, while the patients with myoclonic epilepsy have shown no effect (3 cases) or negative response (1 case). Methysergide was active only in 1 patient affected by progressive erratic myoclonus who had a striking worsening of clinical picture. The main side effects observed were: gastrointestinal distress (L5HTP--4 patients, fenfluoramine--2, quipazine--1, methysergide--2) and cutaneous rash (quipazine--1 case). These results support the possible implication of the serotonergic system in the pathogenesis of myoclonus other than post-anoxic.

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