Splenic abscess secondary to a colosplenic fistula in Crohn's disease.

Colosplenic fistula associated to splenic abscesses is a rare condition in Crohn's disease (CD). Diagnosis is difficult and a computed tomography (CT) scan is helpful. Treatment includes surgical procedure. Atypical locations of fistulas and abscesses should not be overlooked in CD.

Signs of interhemispheric disconnection in marchiafava-bignami disease.

We report three cases of Marchiafava-Bignami disease. The diagnosis was made on clinical grounds. Evidence of interhemispheric disconnection was found in all three cases. This included left hand anomia, left-sided apraxia, left-hand agraphia, and left-sided pseudoextinction to both visual and tactile stimuli. In two cases, there was a left-ear extinction on a dichotic listening test.

Value of multiple sclerosis diagnostic criteria. 70 autopsy-confirmed cases.

We have evaluated the sensitivity of the most recent and most frequently used criteria for the diagnosis of definite multiple sclerosis by the retrospective study of the clinical files of 70 pathologically confirmed cases. For each case, the date of diagnosis was determined separately using different sets of criteria. The delay of diagnosis was then calculated. The diagnosis was made significantly earlier when certain criteria were used. This was more marked in the earlier years of the disease. We also found that cases of multiple sclerosis with progressive courses were diagnosed later than cases with other courses, whatever the criteria used. This was statistically significant only for a single criterion.

Serum crosslaps in comparison to serum osteocalcin and urinary bone resorption markers.

In this study we evaluated the routine practice and clinical application of serum crosslaps to urinary-crosslaps, -N-telopeptide-related fraction of type 1 collagen, -deoxpyridinoline, -totalpyridinoline and serum osteocalcin. The utility of both the serum and urine immunoassays for bone formation and resorption marker were tested in a cohort of 593 female and male patients from our outpatient clinic for osteology and rheumatology and compared to important osteoporosis risk factors like age, gender, E2 deficiency, bone density and chronic renal failure. The biochemical maker of bone formation, serum osteocalcin exhibit significant correlations to all five tested serum and urinary markers of bone resorption (p < 0.0001) crosswise to all different groups of patients. The group of chronic renal failure patients showed no significant correlation between the tested bone turnover parameters and the serum creatinine level except a significant increase and correlation for serum crosslaps and for the ratio of serum and urinary crosslaps. Associations between the age of the patients and the markers of bone turnover were rather poor. We found a significant, negative association between serum and urinary bone turnover markers and bone density and were interested, whether in patients with bone density < 2.5 SD an enhanced bone turnover could be detected in the same way as for E2 deficiency. Applying a discriminant analysis it was possible to discriminate between the patient with BD < 2.5 SD and those with BD > 1.0 SD with a sensitivity of 70% and a specificity of 65% using serum crosslaps. In case of urinary crosslaps the discriminatory power was slightly lower (sensitivity: 65.6%, specificity: 67.5%) and for serum osteocalcin the discriminatory power was negligible higher (sensitivity: 79%, specificity: 56%). The highly significant correlation between the urinary and serum crosslinked peptides by ELISA and serum osteocalcin supports the concept that these respective indices of bone formation and resorption both in urine and serum reflect a coupled process in vivo with sensitivity and specificity to pathological bone density, estrogen deficiency and chronic renal failure.

Adult adrenoleukodystrophy: a sporadic case?

This is a report of a case of the adult cerebral form of X-linked ALD. The 27-year-old patient presented with psychiatric disturbances. NMR was performed and compared to CT scan to define cerebral demyelination. The level of hexacosanoate was found to be increased in the patient's serum. Biochemical analysis of the patient's mother's serum and cultured fibroblasts and of serum samples from 10 other members of the family who could have been carriers of this X-linked disease, produced negative results. Hence, it is most likely that this case has occurred sporadically. HLA determination revealed the DR2 antigen which is often associated with multiple sclerosis.

[Recurrent acute dyskinesia as the sole manifestation of phenytoin poisoning]. / Dyskinésies aigues récidivantes seules manifestations d'un surdosage en phénytoine.

A 20 year-old epileptic female patient was admitted because of recurrent dyskinesias of 3 months duration. She was receiving phenobarbital, phenytoin (PHT) and clonazepam. PHT dosage had been increased 4 months earlier. Clinical examination and CT scan gave normal results. PHT plasmatic levels were 42 micrograms/ml on admission and there were no other symptoms of PHT toxicity. PHT-induced dyskinesias usually occur together with severe encephalopathy and/or clinical or paraclinical evidence of basal ganglia damage and/or other evidence of PHT toxicity, none of which were present here.

[Lateral inferior pontine infarction: 2 clinical aspects]. / Infarctus pontin inférolatéral: deux aspects cliniques.

Cases of lateral inferior pontine infarction are rare in the literature. We report two cases with MRI correlations. One involved the whole territory, the other the tegmental area only. Both also involved the middle cerebellar peduncle and one of them the adjacent lobules of the cerebellum. The first case emphasizes the classical alternate syndrome. The second case shows that acute vertigo mimicking a labyrinthine lesion can occur when involving the territory of the anterior inferior cerebellar artery.

[Treatment of progressive and severe forms of multiple sclerosis using a combination of antilymphocyte serum, azathioprine and prednisone. Clinical and biological results. Comparison with a control group treated with azathioprine and prednisone only. 4-year follow-up]. / Traitement des formes évolutives et graves de sclérose en plaques par l'association sérum antilymphocytaire, azathioprine, prednisone. Résultats cliniques et biologiques. Comparaison avec un groupe témoin traité par azathioprine et prednisone seuls. Surveillance pendant 4 ans.

45 patients were treated by combined application of antilymphocyte serum, azathioprine and prednisone: a control group of such 22 patients was besides treated by azathioprine and prednisone only. The treatment by antilymphocyte serum included a four weeks initial treatment, and one year continuous treatment one infusion per week. Azathioprine and prednisone were given every day during the same time. Azathioprine only was given during the three following year. The two groups were followed up during four years. This treatment was well accepted by patients under strict technical survey conditions. Serum sickness was noted in 11 p. cent of the cases. The equine antiglobulin antibody titre, increased during the initial treatment, was connected with serum sickness or, when the treatment was continued, with the inefficacy of the treatment. Rosettes titre decreased in ten cases, no clinical correlation was possible. The complement, immune complexes, were normal before treatment with most of the observed patients. The delayed hypersensitivity skin tests were negative after a month in 80 p. cent of the cases. The cerebrospinal fluid analysis, for the elements as well as for the rate of proteins and gammaglobulins, showed no significant variations before and after the treatment. The comparison with the control group showed a significant statistical difference as too the number of aggravated and improved patients after the first year. When grouping together stable and improved patients, the difference was statistically significant after the third year. The difference was not significant in years "two" or four. No difference in the frequency of the relapses could be noted, in the first year or the following three years. In spite of inconsistent results comparison showed favorable data after three years. The good results seemed due to the additional use of antilymphocyte serum. Such a treatment associating antilymphocyte serum to azathioprine and prednisone is beneficial in evolutive, remittent and recent multiple sclerosis. Through the length of the treatment remains difficult to determine, we conclude that it is really beneficial for such patients.

[Late syringomyelobulbia complicating spinal cord injury. Improvement after syringoperitoneal shunt]. / Syringomyélobulbie tardive compliquant un traumatisme médullaire. Amélioration après dérivation syringo-péritonéale.

A patient suffered from paraplegia of T-10 level at age 29, and he complained of new symptoms in the upper limbs at age 42. Gradual ascending worsening occurred, and the diagnosis of post-traumatic syringomyelobulbia was made 23 years after the initial accident. CT scan with metrizamide transit at the medulla level confirmed the diagnosis. Definite clinical improvement was seen after syringo-peritoneal shunting. Clinical, radiological and therapeutic aspects are briefly discussed.

[Myopathy with tubular aggregates. Clinical, biological and histological study of 2 cases]. / Myopathie avec agrégats tubulaires. Etude clinique, biologique et histologique de deux cas.

Two male patients, aged 46 and 48, presented with muscle pain on exercise, generalized or limited to the upper extremities. Physical signs were limited to mild muscle wasting in one case. There was no myoglobinuria. Electromyography was normal in one case, and showed some polyphasic units in the other. An ischaemic lactate test was normal in the one case in which it was performed. Serum creatine kinase activity was normal or only mildly elevated. Histochemical and ultrastructural study of muscle showed tubular aggregates in type II fibers in both cases, and otherwise gave normal results. Symptoms were unchanged with a follow-up of 2 and 16 years, respectively. Tubular aggregates are found in a number of unrelated clinical and experimental conditions, which are reviewed. In these cases and in 3 similar patients, previously reported, tubular aggregates seem to be the hallmark of hitherto unrecognized metabolic disorders.