ABSTRACT
OBJECTIVE: To determine whether treatment with the levorotatory form of hydroxytryptophan (L-5-hydroxytryptophan), a controversial experimental drug, can improve the conditions of patients with ataxia. DESIGN: A double-blind crossover study with the levorotatory form of hydroxytryptophan was performed in 39 patients with degenerative cerebellar diseases. SETTING: Patients were selected from an ongoing prospective follow-up study at two university hospitals. PATIENTS: We studied 19 patients with Friedreich's ataxia, 13 with cerebellar atrophy, and seven with olivoponto-cerebellar atrophy. INTERVENTION: The levorotatory form of hydroxytryptophan was given orally in a dose of 1000 mg/d. Each treatment phase, with the levorotatory form of hydroxytryptophan or the placebo, lasted 10 months, after which the treatment of patients was crossed over to the other phase. MAIN OUTCOME MEASURES: Ataxia was documented and quantified by using a clinical score, posturography, and measurement of grip force and the rapid-syllable repetition rate. RESULT: The levorotatory form of hydroxytryptophan had no significant effect on cerebellar symptoms. CONCLUSION: Long-term treatment with a high dose of the levorotatory form of hydroxytryptophan does not improve the conditions of patients with ataxia.
Subject(s)
5-Hydroxytryptophan/therapeutic use , Cerebellar Ataxia/drug therapy , 5-Hydroxytryptophan/analogs & derivatives , Cerebellar Ataxia/physiopathology , Cross-Over Studies , Double-Blind Method , Hand Strength , Humans , Posture , Psychomotor Performance , SpeechABSTRACT
We report the case of a female patient with thrombocytopenic purpura Werlhof. Splenectomy was followed by relapsing pneumococcal meningitis. Particularly, repetitive infections were caused by pneumococcus capsula type 13. This type is not included in the common 23-polyvalent vaccine matter. The necessity of splenectomy in autoimmunological diseases should be considered carefully in the individual case. Patients should be well informed of the high risk of infection after splenectomy and should undergo medical treatment immediately.
Subject(s)
Meningitis, Pneumococcal/etiology , Postoperative Complications/etiology , Purpura, Thrombocytopenic, Idiopathic/surgery , Splenectomy , Adult , Diagnostic Imaging , Female , Humans , Meningitis, Pneumococcal/diagnosis , Postoperative Complications/diagnosis , Recurrence , Risk Factors , Sepsis/diagnosis , Sepsis/etiologyABSTRACT
Signs of atrophy on cranial CT were investigated in 35 patients diagnosed as suffering from autosomal dominant (n = 21) or idiopathic (n = 14) cerebellar ataxia. Thirteen patients with a pure cerebellar syndrome were examined after at least 4 years of disease (mean duration 10.5 years) and were classified as cerebellar atrophy (CA). Twenty-two patients with additional non-cerebellar signs were classified as olivo-ponto-cerebellar atrophy (OPCA). Four (30%) of the patients with CA had atrophy of the brain stem in addition. Of the 22 patients with OPCA, 9 (40%) had atrophy of the cerebellum only. In patients with CA or OPCA correlation of clinical signs with severity of atrophy on CT was poor. Atrophy on CT often fails to differentiate autosomal dominant or idiopathic cerebellar ataxias in CA or OPCA: patients with CA can also have atrophy of the brain stem and patients with OPCA do not necessarily show brain stem atrophy.
