Partial Currarino syndrome in a non-pediatric patient. A rare cause of bacterial meningitis

Enterogenic meningitis is an infrequent cause of central nervous system infection. Among these causes the Currarino syndrome may be found presenting sacral agenesis, presacral mass and anorectal stenosis. This syndrome normally causes enterogenic meningitis in the early years of life. The case reported corresponds to a 24-year-old male presenting polymicrobial meningitis with fecal flora germs (anerobic enterococci, Bacteroides fragilis and Escherichia coli). These clinical findings led to radiologic lumbar study with the diagnosis of Currarino syndrome. The existence of neuroenteric fistulas justifies the development of fecal flora meningitis. The nosology of the syndrome as well as the therapeutic strategy are reviewed.     

Remote memory in Alzheimer's disease

The authors examined the severity and type of deficits in remote memory in patients with probable Alzheimer's disease (AD). In the first study, 40 AD patients showed significantly more severe deficits on both the free-recall and the recognition sections of the Remote Memory Scale (which measures memory for famous people and well-known events) compared with normal control subjects. In the second study, 25 AD patients showed significantly more deficits on the free-recall section of the Autobiographical Memory Scale compared with normal control subjects. Remote memory deficits in AD may be related to both retrieval deficits and damage to memory traces.     

Early onset cerebellar ataxia and preservation of tendon reflexes: clinical phenotypes associated with GAA trinucleotide repeat expanded and non-expanded genotypes

The aim of this study was to determine phenotypie characteristics of patients with early onset cerebellar ataxia (EOCA) with preserved tendon reflexes. The series comprises 25 patients, representing 10% of all ataxic patients who have been genetically studied in our laboratory since 1990. There were 11 males and 14 females. Fourteen patients were homozygous for the GAA expansion on chromosome 9q13 (group 1) and therefore a diagnosis of Friedreich's ataxia with retained reflexes (FARR) was given. The remaining 11 patients had two normal non-expanded alleles (group 2) and a working diagnosis of EOCA with retained reflexes (EOCARR) was established. Mean ages of onset were 13.7 +/- 5.9 years (3-25) for group 1 and 10.3 +/- 7.3 for group 2; the difference was not significant. Frequencies of symptoms and signs were also comparable for both groups the only significant differences being the higher frequency of nystagmus, cardiomyopathy and sensory neuropathy in group 1 patients. There was a tendency for FARR patients to have higher frequencies of hypopallesthesia in the lower limbs and skeletal deformities. In none of the cases diabetes mellitus was observed. We conclude that differentiation of FARR and EOCARR may be suspected by classical clinical and electrophysiological data and confirmed by analysis of the GAA repeat.