Two patients with reinfection of Borrelia burgdorferi are presented. An 11-year-old girl developed recurrent acute peripheral facial palsy at an interval of five years. A 64-year-old woman showed paraesthesia in the leg and effusion in the knee. Three years later, an erythema migrans developed at the thigh. In both patients tick bites, corresponding clinical manifestations, and detection of specific antibodies proved the reinfections. The course of the humoral immune responses showed basic differences between the patients. At the interval between the first and second infection, the specific antibodies of the girl decreased beyond the cut-off level. On the other hand, the titer of specific IgG antibodies of the other patient remained at a constant level. Reasons for the failure of immune protection are discussed.

Acute Disease↗

PubMedDate not supplied

[Development of brain atrophy, therapy and therapy monitoring in glutaric aciduria type I (glutaryl-CoA dehydrogenase deficiency)].

Serial trans-fontanellar sonographic examination in a patient with glutaric aciduria type I (GA I) demonstrated that the typical frontotemporal cerebral atrophy developed postnatally within three months paralleling the onset of dystonic symptoms. Pathogenesis of the accompanying macrocephaly remains unclear and can form a diagnostic pitfall. Diet low in lysine and tryptophan led to a dramatic fall in urinary glutaric acid (GA) excretion but as in other patients with GA I did not substantially influence clinical symptoms and course. We determined unchanged levels of GA in plasma and cerebrospinal fluid resulting from variable renal tubular secretion and reabsorption of GA. Monitoring urinary excretion of GA appears inappropriate to control dietary treatment in GA I. Substitutive correction of secondary carnitine depletion seems to protect from deleterious metabolic crises. Treatment with valproic acid resulted in a rise of GABA-concentration in cerebrospinal fluid but did not ameliorate clinical symptoms. This finding is in contrast with the hypothesis that inhibition of cerebral GABA-synthesis by GA is responsible for the development of dystonia in GA 1. Although we observed impressing fluctuation of dystonic symptoms, levodopa did not show therapeutic effects. The extreme variability in the severity of neurologic disease in metabolically identical individuals leads to a "two-hit"-hypothesis.

Amino Acid Metabolism, Inborn Errors↗

PubMedDate not supplied

[Acute chorea following heart surgery with hypothermia and total bypass].

We report a 6 year old girl who developed acute chorea following cardiac surgery using hypothermia and total cardiopulmonary bypass. Rheumatic disease, cerebral embolism or hemorrhage were excluded. Cerebral hypoperfusion during surgery is the most probable cause for this neurologic event. Treatment with Tiaprid (Tiapridex) resulted in a rapid resolution of symptoms.

Brain Ischemia↗

PubMedDate not supplied

[Progressive ataxia and distal muscular atrophy--differential diagnostic considerations on Roussy-Lévy syndrome].

The Roussy-Lévy-Syndrome ist generally considered a pheno-typic variant of the hereditary motor and sensory neuropathy type I (HMSN I), i.e. the classic Charcot-Marie-Tooth-neuropathy, and is thus nosographically distinguishable from Friedreich's ataxia. While posterior column symptomatology, dementia and rapid progression are recognized as diagnostic criteria for Friedreich's ataxia and HMSN I is clinically defined by the typical features of a progressive polyneuropathy, the symptom triad "gait disturbance, areflexia and pes cavus" of the Roussy-Lévy-Syndrome has comparatively little diagnostic specifity. Because of the variability of clinical symptoms as shown by two case reports, the diagnosis of Roussy-Lévy-Syndrome in childhood should be made only with great caution. For early genetic counselling and prognostic evaluation, bioptic and neurophysiologic results are of decisive importance.

Adult↗