Comparative study of indices of activity evaluation in rheumatoid arthritis

IntroductionChoosing between the different indices of activity evaluation in RA is often difficult considering the very heterogeneous clinical expression of the disease. The objective of our study was to evaluate the level of similarity between SDAI, CDAI, DAS28-_ESR and DAS28-_CRP indices in the evaluation of RA activity.Patients and methodsIn this transversal study, a total of 100 patients with RA responding to the ACR 87 criteria were followed up for a period of 20 months. The correlations between the four indices were studied through the Pearson's correlation coefficient (r). The similarity between these tools was evaluated through Kendall's (K) “tau” similarity coefficient.ResultsThe 87 female and 13 male patients (sex ratio: 6.7F/1M) were of a mean age of 52.9 ± 11.6 years (17–77) and have been living with RA for a mean of 8.3 ± 9 years (2 months–41 years). The DAS28-_ESR mean score was 5.53 ± 1.46 [1.25–8.05]. The DAS28-_CRP mean score was 5.01 ± 1.44 [1.68–7.81]. The CDAI mean score was 30.72 ± 15.04 [2–62] and that of SDAI was 28.86 ± 15.86 [2.3–71.3]. A positive, statistically significant correlation was noted between the four indices of RA activity. The level of similarity between the different indices was good (K variation between 0.758 and 0.943). DAS28-_ESR allowed classifying the patients in the same category of disease activity than DAS28-_CRP in a proportion of 85%. This proportion was 88% when comparing DAS28-_ESR to CDAI and SDAI, respectively. Regarding DAS28-_CRP and CDAI, these two indices classified the patients in the same category in a proportion of 80%, compared to 87% regarding DAS28-_CRP and SDAI. Finally, CDAI and SDAI classified the patients in the same category in a proportion of 92% with an excellent level of similarity.ConclusionDifferent evaluation indices of RA activity are currently available. DAS28 is the most used. CDAI and especially SDAI have a good level of similarity with DAS28. Their advantage is to be simple and quick, and seem therefore well adapted to the follow-up of outpatients.     

Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion-hemiplegia-epilepsy syndrome

A case of an acute life-threatening presentation of hemiconvulsion–hemiplegia–epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling right hemisphere with marked temporal herniation. The baby underwent a decompressive right hemicraniectomy with temporal cortical biopsies. The post-operative course was favourable. The histological findings were unspecific, showing a gliotic spongiosis with disseminated granular cells. The post-operative MRI depicted a right hemisphere atrophy. To our knowledge, a space-occupying presentation of HHE syndrome requiring surgical decompression has never been described before while only a few reports dealt with the neuropathological aspects of this syndrome.     

Pseudo-polymyositic aspect of amyotrophic lateral sclerosis

The causes of skeletal muscle weakness are multiple and their diagnosis is difficult, particularly in atypical myopathy. We report the observation of 58-old woman, who presents a muscle weakness of lower limbs with elevation of muscle's enzymes. These symptoms were bound initially to polymyosistis. However, the absence of inflammatory biologic syndrome and the results of muscular biopsy, make this diagnosis unlikely. The aggravation of the myopathy with extension to spinal muscles, the apparition of fasciculations and bulbar signs, the results of the electromyogram pose the diagnosis of amyotrophic lateral sclerosis.     

Lymphadenopathic form of alveolar rhabdomyosarcoma: A case report

We report the case of a special form of rhabdomyosarcoma called "lymphadenopathic" which was reported only two times in the literature. This lymphadenopathic alveolar RMS shows lymph nodes as clinical manifestation mimicking lymphoma with an unknown primary neoplasm. In the case we report, clinical examination, and radiological studies revealed superficial and deep located lymph nodes in association with bilateral pleural effusion. Conventional histological examination showed lymph node infiltration by a metastatic tumour having alveolar pattern. Some of tumour cells were round with multilobed off-centred nucleus and eosinophilic cytoplasm containing fibril material with longitudinal and transverse striation indicating a muscle nature of the tumour proliferation. The immunohistochemical study showed intense and diffuse immunoreactivity of tumour cells for vimentin and a discreet immunostaining by desmin. These findings were compatible with the diagnosis of lymph node metastasis by alveolar rhabdomyosarcoma.     

Testicular lymphoma: report of 4 cases

BACKGROUND: Primary testicular lymphoma is the most common testicular malignancy in the elderly, account for 1% of all non Hodgkin lymphoma (NHL). The aim was to report from new cases of testicular lymphoma CASES: We report the only four cases of testicular lymphoma observed in a period of 15 years; all these cases were primary diffuse large cell B NHL. Disease course is usually aggressive, with widespread organ involvement. Retrospective analyses show a poor prognosis for this presentation.     

Le médulloépithéliome du système nerveux central. À propos de trois cas

Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later.