Transient adult T-cell leukemia/lymphoma picture during varicella infection in an HTLV-1 carrier.

HTLV-1 (human T-lymphotropic virus type 1) is associated with tropical spastic paraparesis, adult T-cell lymphoma (ATL), and also with opportunistic infections. The risk for developing ATL in HTLV-1 healthy carriers is low, between 1 and 4%. Nothing is known about the events promoting the evolution from the healthy carrier state to symptomatic ATL. We describe the case of a 44-year-old French Caribbean man with a chronic and recurrent strongyloidiasis in which the occurrence of a hemorrhagic and necrotic varicella led to the discovery of an infection by HTLV-1 and an acute form of ATL. All hematological data were normal before the onset of varicella. ATL completely disappeared at the same time as the varicella healed. This leads us to hypothesize that acute infections such as the reactivation of varicella-zoster may act as a promoting factor for the development of ATL in healthy HTLV-1 carriers.

Short report: isolation and partial characterization of a Puumala virus from a human case of nephropathia epidemica in France.

Puumala (PUU) virus (Bunyaviridae: Hantavirus), the etiologic agent of nephropathia epidemica (NE), the mid form of hemorrhagic fever with renal syndrome, is enzootic in Europe and has been known to occur in France since 1983. We report the first isolation of PUU virus in France and western Europe from a case of NE acquired in France. The virus was isolated from a serum collected in the acute phase of the clinical course by successive blind passages in Vero E6 cells. Serologic typing using monoclonal antibodies confirmed the identity of the virus as PUU. The sequence of an 832-nucleotide fragment of the virus medium RNA segment obtained by the polymerase chain reaction (PCR) also classified it as a PUU virus. The sequence of this isolate from a human case in France is closely related to the sequence of a PUU virus obtained by the PCR from a German patient.

[Viral hepatitis in patients over 60 years of age. Clinical, etiologic and developmental aspects]. / Les hépatites virales après 60 ans. Aspects cliniques, étiologiques et évolutifs (78 cas).

The authors report a retrospective study of 78 cases of acute viral hepatitis observed from 1971 to 1985 in patients over 60. Viral hepatitis involved males as often as females. A, B, delta and non-A, non-B viruses were responsible for 11.5, 23.1, 5.1 and 60.3 p. cent of cases, respectively. From 1975 on, the frequency of non-A non-B viral hepatitis has reached 85 p. cent while blood transfusions appeared as the most important route of transmission. Outcome was severe in 10.3 p. cent of cases. Early in the course of disease, the risk of progression to cirrhosis was obvious and concerned 12 patients (1 out of 18 with B hepatitis, 3 out of 4 with D hepatitis, 8 out of 47 non-A non-B hepatitis). The long term prognosis (from three to five years) was not as poor as stated in previous studies.

[Hepatic amebiasis: study of 152 cases]. / L'amibiase hépatique: étude de 152 observations.

The purpose of this study was to describe and analyse retrospectively the clinical, serological, anatomical and evolutive features of 152 cases of hepatic amebiasis in young adults, treated and followed up in France from 1969 to 1983. The disease was revealed 3 times out of 4 by tender hepatomegaly with fever, but only in 6 cases by complications. Serological tests (immunofluorescence or hemagglutination) were always positive for amebiasis, whereas Entamoeba histolytica was absent from stools in 96.7 p. 100 of the cases. Hepatic amebiasis always caused a hepatic abscess: in these cases, the superiority of ultrasonography over all other diagnostic methods was confirmed, especially concerning the detection of multiple abscesses (47 p. 100). Complete recovery was obtained by medical treatment in 117 cases, either alone (98 cases), or combined with needle aspiration (19 cases). Nitro-imidazoles are the simplest treatment, but nevertheless in 5 cases they were not effective. These patients were then treated with dehydroemetine, associated in 2 cases with surgery. Four patients relapsed at mid or long-term after apparent recovery, in the absence of any obvious reinfection. A significant correlation between the course of the treated disease and the size and number of abscesses was demonstrated: it was possible to define a group characterized by a slow and/or complicated course (with single abscess of the right lobe whose diameter is equal to or greater than 10 cm, or multiple abscesses). The pathogenesis of hepatic amebiasis is not yet fully understood.

[Olfactory esthesioneuroma manifesting as Schwartz-Bartter syndrome]. / Esthésioneurome olfactif révélé par un syndrome de Schwartz-Bartter.

INTRODUCTION: Olfactory esthesioneuroblastoma is an uncommon neuroectodermal tumor originating from the olfactory epithelium, which is rarely associated with hormone excess syndrome. EXEGESIS: Asymptomatic olfactory esthesioneuroblastoma was diagnosed in a 22-year-old man who presented a syndrome of inappropriate antidiuretic hormone secretion. Following surgery, the immunohistochemical analysis demonstrated the existence of neurophysin hormone in tumoral cells. CONCLUSION: This case provides evidence that olfactory esthesioneuroblastoma can be uncovered by inappropriate antidiuretic hormone secretion.

[Hemophagocytic syndromes]. / Les syndromes hémophagocytaires.

Hemophagocytic syndromes are the clinicobiological translation of an unconnected macrophagic activity with hemophagocytosis. Their physiopathology is related with a deregulation of the T lymphocytes and an excessive production of cytokines. Acquired hemophagocytic syndromes are mostly associated with underlying pathology which they can reveal: immunodeficiency, infections (mostly of viral origin), hemopathies and cancers, auto-immune diseases. The main clinicobiological features are fever, hepatosplenomegaly and peripheric bicytopeny. In the majority of cases, the diagnosis is confirmed by a myelogram which shows the presence of benign histiocytes, actively phagocyting the hematopoietic cells. The pejorative prognosis of hemophagocytic syndromes (actual mortality rate 30 to 45%) requires an early therapy which associates etiological treatment of the underlying affection with pathogenic treatment (pulse of corticosteroids, immunoglobulins, immunosuppressors, or plasmapheresis).

[Yellow nail syndrome with chylous effusions]. / Syndrome des ongles jaunes avec épanchements chyleux.

Yellow nail syndrome is characterized by a yellow discolouration of the nails associated with idiopathic lymphoedema and pleuropulmonary manifestations. Pleural effusions are usually of the exudate type with a normal lipid content. The case reported here of a 74-year old man who had yellow nails together with chylothorax and chylous ascites provides an opportunity to discuss the origin of these rare effusions which, like the syndrome itself, were most probably due to primary abnormalities of the lymphatic system.

[Bacterial cerebral aneurysms without infectious endocarditis: analysis of a case and review of the literature]. / Les anévrismes bactériens cérébraux sans endocardite infectieuse: étude d'un cas et revue de la littérature.

INTRODUCTION: Bacterial intracerebral aneurysms, which are a common complication of infectious endocarditis, are unusual without Osler's disease. EXEGESIS: From the case report of a man with a bilateral bacterial intracranial aneurysm without endocarditis resulting in an hypopituitarism, we undertook a literature review of bacterial intracerebral aneurysms without endocarditis. CONCLUSION: Although this review has found few cases, this kind of aneurysms seems to have different features from those secondary to infective endocarditis: younger age of incidence, majority of Staphylococcus aureus, clinical presentation as a thrombophlebitis of the cavernous sinus, location of aneurysm on bigger cerebral artery.

[Mitochondrial cytopathies]. / Les cytopathies mitochondriales.

Mitochondrial cytopathies are diseases due to a defect of mitochondrial respiratory chain and are characterized by the presence of morphological abnormalities of mitochondria (ragged red fibers). The clinical manifestations can be polymorphic as various organs may be involved; but in general, these disorders affect either muscle alone (progressive external ophtalmoplegia, myopathy with weakness) or muscle and brain (encephalomyopathies). Some of these diseases may be due to alterations of the mitochondrial genome. Diagnosis can be achieved through the following steps: measurement of lactate, pyruvate and ketone bodies after fasting followed by a carbohydrate rich meal and/or exercise; histological examination and biochemical investigation of a skeletal muscle biopsy; study of possible mitochondrial genome alterations.

[Diagnosis of muscular intolerance during exercise caused by enzyme deficiency in adults]. / Diagnostic d'une intolérance musculaire à l'exercice par déficit enzymatique chez l'adulte.

The exertional muscle pain syndrome in adults is expressed by recurrent episodes of pain and/or episodes of acute rhabdomyolysis, sometimes with transient myoglobinuria or even acute renal failure. The enzymatic origin of this syndrome is suggested by a familial history, by its usual onset during childhood or adolescence, and by its recurrent attacks usually, but not exclusively, caused by exercise. The aetiological diagnosis, suggested by the clinical context and some dynamic tests, is almost always based on good histological and biochemical examinations of the muscle biopsy.

[Disulfiram (Esperal) toxicity. Apropos of 3 original cases]. / Toxicité du disulfirame (Esperal). A propos de trois observations originales.

Prescribed since 1948 to control chronic alcoholism, disulfiram may cause severe toxicity as report in three cases of acute motive axonal polyneuritis. Disulfiram toxicity may present different clinical aspects: 1) Cytolytic hepatitis with fatal evolution in 30% of cases (fulminant hepatitis), and full recovery for the other 70%. The onset of the symptoms usually occurs as early as 15 days to a maximum of 6 months (most within 2 months) after initiation of treatment. 2) Severe optic neuritis with full recovery in 2 months. 3) Peripheral neuropathy usually dose dependent, with different clinical presentations: polyneuritis with sensory, motor, or both deficits, and few cases of tetraplegia. 4) Encephalopathy frequently associated with one of the precedent symptoms, having a favorable outcome (probably resulting in inhibition of dopamine-beta-hydroxylase by disulfiram). The mechanism of toxicity (direct or idiosyncractic) remain unclear. Disulfiram has been used safely in millions of people since 1948, and we have only few cases reports of severe toxicity. From a practical point of view, treated patients should benefit by a neurological examination once a month, ophtalmological examination every 2 months, and hepatic enzymes monitored twice a month during the 2 first months. This is the price to prevent and to detect side effects of disulfiram therapy.

[Recurrent rhabdomyolysis revealing chronic exertional compartment syndrome of the biceps]. / Episodes récidivants de rhabdomyolyse révélant un syndrome de loge antérieure du bras provoqué par lexercice.

UNLABELLED: OBJECTIVE AND PATIENT: To report, an uncommon case of recurrent effort-related biceps compartment syndrome induced by strengthening exercises (body building) in a thirty-years-old white man. DISCUSSION-CONCLUSION: compartment syndrome is typically observed in the lower leg or in the forearm with pain, muscle tightness, cramp-like feeling during exertion. The involvement of the biceps was only described after traumatisms. Patient was initially thought to have metabolic myopathy because acute episodes of exercise induced myalgia and elevated serum CK. Tissue pressure monitoring prior and after a standard exercise test is helpful for diagnosing the recurrent form. The only alternative to fasciotomy is to stop sports activities.

[Subacute idiopathic eosinophilic pneumopathy with favorable outcome without corticotherapy]. / Pneumopathie éosinophilique subaiguë idiopathique d'évolution favorable sans corticothérapie.

The authors report a case of acute idiopathic eosinophilic pneumonia, a recently described entity of unknown etiology. The patients develop a rapidly progressive respiratory failure which is reversible following steroid therapy. The key to the diagnosis is an eosinophilia in the broncho-alveolar lavage or in the lung biopsy. Our observation of a favourable outcome in this case without steroid therapy is evidence perhaps of a less aggressive form of the disease.

[Incidence of nosocomial infections in a military hospital]. / Incidence des infections nosocomiales dans un hôpital des armées.

Over a 2-year period, 479 cases of nosocomial infections were identified in our hospital by a surveillance method based on the bacteriological laboratory results. The monthly incidence rate ranged from 1.8 to 4 percent of all in-patients. With this method, a 2 percent background noise and a 4 percent alarm threshold could be defined. The most frequent nosocomial infections were urinary tract infections (77 percent), purulent skin infections (12 percent) and septicaemias (10 percent). Compared during 15 days with the most sensitive surveillance method of the National Nosocomial Infections Surveillance System, our method proved insufficient to detect nosocomial lung infections and superficial surgical wound infections. On the other hand, it was highly satisfactory to watch for urinary tract infections and septicaemias. Improvements that would not put a heavy burden on the work of clinical departments are suggested.

[Disseminated Histoplasma duboisii histoplasmosis in a female inhabitant of the Ivory Coast]. / Histoplasmose disséminée à Histoplasma duboisii chez une Ivoirienne.

The authors describe a disseminated multivisceral form of african histoplasmosis due to H. duboisii in a 45 years old black woman. Clinical examination shows characteristic lesions of skin, bones and lymph glands. Lymphography reveals lesions of deep lymph glands and laparascopy lesions of liver and spleen. First untimely stopped, treatment was started again with ketoconazole. 20 months later, the course of the disease is, as for as we can appreciate it, favourable.

[Hepatic amebiasis in France. Apropos of 152 case reports]. / L'amibiase hépatique en France. A propos de 152 observations.

The authors report upon 152 cases of hepatic amebiasis (H.A.) observed in France between 1969 and 1983, among young european men (average age 29,2) who were hospitalised in the initial phase of their illness. H.A. was clinically revealed through a painful and febrile hepatomegaly in 3/4 cases and in 6 cases through complications. No chronic form was observed. The amebic serology (I.F.I. and/or H.A.P.) was always positive. The research of an intestinal portage of the parasite was generally negative. The hepatic functions were impaired in the third of the cases. Whichever technique was employed, the anatomic diagnosis has always been made in a phase of intra hepatic collection. The superiority of echotomography over other methods is confirmed (especially for diagnosis of multiple abscess: 47% of success). All patients recovered, most frequently through medical treatment (117 cases). The nitro-imidazoles (1,5 to 2 g per day during 7 to 14 days) remain the prefered treatment, but they are responsible for 5 failures which were overcome by emetine. On the other hand, 4 patients had, after being cured, showed middle and long term relapses without patient recontamination. The evolution of the treated H.A. is significantly correlated to the importance and/or the multiplicity of the liver collection: the voluminous abscess of the right lobe being the most potentially dangerous. The pathogenesis of the H.A. remains imperfectly known. The different clinical aspects which have been found, depend upon the preexistant immunity of the patient and could also be equally associated with a pathology of complex immunity systems.