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D Smadja

Publications and source records attributed to D Smadja.

At least 37 records · Page 2Linked to original sources
PubMed2000

[Thyrotoxic periodic paralysis: two new cases in Blacks].

INTRODUCTION: Thyreotoxic hypokalemic periodic paralysis (THPP) has mainly been described in Asian people. It is rare in Caucasians, and only 12 cases were reported in Blacks. EXEGESIS: We report two cases of THPP in black patients. Hypokalemia was important in case number 1, leading to severe flaccid tetraplegia with respiratory and cardiac complications, but was only mild in case number 2. Complete relief of paralysis was observed under potassic treatment in the first case and spontaneously in the second one. Hyperthyroidism was established only on the basis of biological tests in case number 1, and had been previously diagnosed but undertreated in case number 2. CONCLUSION: Whatever the patient's race, acute paralysis with hypokalemia requires testing for hyperthyroidism, even in the absence of suggestive clinical signs.

Adult↗
PubMed1999

Myelopathy due to calcification of the cervical ligamenta flava: a report of two cases in West Indian patients.

Two cases of cervical myelopathy due to calcification of the ligamenta flava (CLF) are described for the first time in black patients from the French West Indies. A pre-operative CT scan differentiated the diagnosis from one of ossification of the ligamenta flava. Microanalysis on the operatively excised specimen in one patient revealed a mixture of calcium pyrophosphate dihydrate crystals and hydroxypatite crystals. Poor outcome in one patient contrasting with excellent recovery in the other one, who had undergone posterior decompressive laminectomy, emphasizes the importance of surgery in the management of CLF.

Aged↗
PubMed1999

Human T cell leukemia virus type I expression in salivary glands of infected patients.

Human T cell leukemia virus type I (HTLV-I) sequences were sought in labial salivary glands of patients with HTLV-I-associated myelopathy or tropical spastic paraparesis and of seropositive neurologically healthy carriers. HTLV-I proviral DNA was found by polymerase chain reaction amplification in DNA extracted from lip biopsies of every patient. Viral RNA was found by in situ hybridization in the acini epithelium, as well as in lymphocytic infiltrates. This observation suggests that HTLV-I expression in labial salivary glands could participate in the inflammatory lesions observed in these patients. Some seronegative patients with Sjögren's syndrome or dryness syndrome were also positive for viral transactivator tax DNA (41% in Martinique and 16% in non-HTLV-I-endemic region). Despite histologic signs of lymphocytic infiltration, no viral expression was found in the labial salivary glands of these patients.

Carrier State↗
PubMed1999

EPIMART: prospective incidence study of epileptic seizures in newly referred patients in a French Carribean island (Martinique).

PURPOSE: To identify, in the population living in the island of Martinique, persons who had their first epileptic seizure or first came to medical attention because of an epileptic seizure. METHODS: Between May 1, 1994, and April 30, 1995, we collected all suspected cases of provoked and unprovoked epileptic seizures admitted to the hospitals or addressed to the private neurologists or pediatricians of the island. RESULTS: Three hundred nine cases were collected. Rate of initial diagnosis of provoked and nonprovoked seizures (standardized to the U.S. population): 77.7/100,000, with a bimodal distribution of the cases with age (86 in 0- to 10-year age group and 203 in patients older than 60 years). Sixty-three cases were classified as provoked seizures (incidence, 16.4/100,000). Alcohol consumption, stroke, and cranial trauma were the most frequent causes (30.1, 20.6, and 18.7%, respectively). Two hundred forty-six cases were classified as unprovoked seizures (incidence, 64.1): seizures with a stable condition, 74 cases (I, 19.3); seizures with an evolutive condition, 17 cases (I, 4.5); seizures of unknown etiology, 155 cases (I, 40.4). These figures must be considered as the minimal rate. CONCLUSIONS: The global incidence rate of newly referred persons with a diagnosis of epileptic seizures in this study is clearly higher than those observed in industrialized countries but lower than those in developing countries. The major risk factors are represented by alcohol consumption, followed by stroke, cranial trauma, and infectious diseases.

Adolescent↗
PubMed1999

Conservative surgery for atrioventricular valve myxoma.

Patients with valvular myxoma are usually candidates for surgery because of the high incidence of life-threatening embolism. In some cases, the tumor is sessile or presents with a large peduncle: complete excision may then lead to valve replacement. We report two cases of atrioventricular valve myxoma where replacement was avoided. In one patient, a mitral myxoma appended from the edge of the anterior leaflet close to the chordae insertion; safe excision implied destruction of the two chordae and a peritumoral section of the anterior leaflet. A chordal transposition technique was used to preserve valve competence. In a second patient, a tricuspid myxoma causing syncopal episodes was resected; this was characterized by a large stalk, located on the anterior tricuspid leaflet away from chordal attachment and the valvular annulus. Treatment was by resection and the leaflet reconstructed with a pericardial patch. Techniques for conservative treatment of degenerative valvular disease or endocarditis, when monitored peroperatively by transesophageal echocardiography, may be successful in the surgical resection of atrioventricular myxoma.

Adult↗
PubMed1998

[Efficacy and good tolerance of cotrimoxazole as treatment of cerebral toxoplasmosis in AIDS].

OBJECTIVE: Non-viral opportunistic infections involving the central nervous system in AIDS patients most commonly result from toxoplasmic encephalitis (TE). Combination pyremethamin (pyr)/sulfadiazine (sulf) is the mainstay treatment for TE, but many patients experience severe adverse events occasionally requiring discontinuation of this antitoxoplasmic medication. This investigation assessed the effects of an open, prospective trial of alternative trimethroprim/sulfamethoxazole: cotrimoxazole (CTX) therapy for TE in AIDS patients. PATIENTS AND METHODS: The subjects were 18 AIDS patients with a first presumptive attack of TE (Group 1) and 9 relapsing patients, including 6 out of Group 1 (Group 2). We gave CTX as a therapy at the dose of 960 mg four times a day for 48 hours, then 960 mg three times a day for two weeks, followed by 960 mg twice daily until computed tomography showed complete disappearance of active TE lesions. Life-long maintenance therapy consisted to CTX 960 mg daily. RESULTS: Group 1: Seventeen patients improved clinically and achieved complete resolution on computed tomography scars over a mean period of 33 days (range: 21-56). Only one patient was withdrawn from the study at day 18 due to a severe skin rash. Neither serious hematologic nor liver toxicity were observed. Under maintenance therapy, 7 patients relapsed after an average duration of 15.5 months. Relapses were precipitated either by poor compliance (5/7) or erronenous CTX protocol (2/7). Group 2: There were 15 relapses affecting 9 patients who were treated successfully with CTX. CTX was discontinued in one relapsing patient who experienced a Stevens-Johnson syndrome on day 13. This patient had previously experienced cutaneous intolerance to sulfadiazine. CONCLUSION: A relative low dose regimen of CTX appears to be strongly efficient and safe treatment for toxoplasmic encephalitis in AIDS. Such a study is of particular interest for developing countries where TE is highly prevalent, given the wide availability of CTX which could be proposed as an economic first line therapy.

AIDS-Related Opportunistic Infections↗
PubMed1998

Ossification of the ligamenta flava with severe myelopathy in a black patient. A case report.

STUDY DESIGN: A case of symptomatic ossification of ligamenta flava in a black man from Martinique (French West Indies) is reported. OBJECTIVES: To show that ossification of ligamenta flava may be observed in racial groups other than Japanese people and that the postoperative prognosis of symptomatic ossification of ligamenta flava is not always excellent. SUMMARY OF BACKGROUND DATA: Ossification of ligamenta flava causing slowly progressive myelopathy or radiculopathy is rare. It usually occurs in the lower thoracic spine. Ossification of ligamenta flava has mainly been described as occurring in Japanese people and very rarely in Caucasians. Diagnosis is based on a computed tomographic scan or magnetic resonance imaging, and postoperative prognosis is usually good. METHODS: Low thoracic ossification of ligamenta flava was diagnosed in a black man from Martinique, based on the computed tomographic scan data and on the histopathologic examination of the removed tissue. The patient was clinically evaluated before and 1 year after the operation. A postoperative computed tomographic scan was performed. A magnetic resonance image was not available in this case. RESULTS: The patient exhibited severe subacute myelopathy. After decompression, the neurologic recovery was incomplete. A postoperative computed tomographic scan showed complete excision of ossification of ligamenta flava and decompression of the spinal cord. CONCLUSIONS: Ossification of ligamenta flava may occur in black people. An incomplete postoperative recovery may be observed in such cases of unusual subacute compressive myelopathy.

Aged↗
PubMed1998

[Acute disseminated encephalomyelitis preceding cutaneous lupus].

STUDY DESIGN: A case of acute disseminated encephalomyelitis (ADEM) preceding the features of cutaneous lupus is reported. OBJECTIVES: To suggest that ADEM and cutaneous lupus are pathophysiologically related. SUMMARY OF THE BACKGROUND DATA: Neurological complications of systemic lupus erythematosus are common. However, demyelinative central nervous system manifestations are rare, and restricted to neuromyelitis optica (NMO). NMO is thought to be a partial form of ADEM. METHODS: A clinical, neuroradiological and immunological study was performed in this case. RESULTS: ADEM was diagnosed in a young black female. Three years later, she developed a cutaneous lupus without hypocomplementemia. CONCLUSION: A relationship between ADEM and cutaneous lupus is suggested. The normal serum complement level in our patient might be a predisposing factor for the development of ADEM.

Adult↗
PubMed1998

[Acute renal insufficiency and cerebral infarction during high-dose intravenous immunoglobulin therapy].

INTRODUCTION: Side-effects of immunoglobulins administered via the intravenous route are usually minor. However, acute renal failure and more rarely thrombotic events, including ischemic stroke, have been reported in association with immunoglobulin infusion. To our knowledge, no case of both acute failure and stroke following immunoglobulin has been described until now. EXEGESIS: Two days after immunoglobulin infusion (2 g/kg), a patient who presented with autoimmune thrombocytopenia suffered severe acute renal failure associated with an ischemic stroke in the right anterior choroid artery territory. Moreover, the stroke worsened immediately following a second infusion (1 g/kg). Clinical and neuroradiological examinations were conducted. CONCLUSION: Results indicate the need for both close monitoring of serum creatinine and diuresis before starting immunoglobulin therapy, and limiting the total dose to prevent thrombotic events such as stroke.

Acute Kidney Injury↗
PubMed1998

Ischemic choroidopathy in primary Sjögren's syndrome.

PURPOSE: The authors describe a 68-year-old woman in whom ischemic choroidopathy and optic neuropathy developed in association with primary Sjögren's syndrome with central nervous system involvement. METHODS: Diagnosis of primary Sjögren's syndrome was made upon the association of keratoconjunctivitis sicca, minor salivary gland biopsy and serologic abnormalities. Fluorescein angiography showed signs highly suggestive of ischemic choroidopathy. The authors discuss differential diagnosis and pathophysiology of such choroidal manifestations. Treatment consisted in massive steroid therapy. Secondarily, an immunosuppressant agent (azathioprine) was successfully added because of neurological recurrences. CONCLUSION: Primary Sjögren's syndrome should be considered in the differential diagnosis of ischemic choroidopathy.

Aged↗
PubMed1998

[Optic neuromyelitis and bilateral acute retinal necrosis due to varicella zoster in a patient with AIDS].

We report a case of bilateral acute retinal necrosis (ARN) following an acute optic neuromyelitis (AONM) in an immunodepressed patient (T CD4 lymphocyte count under 50/mm3) suffering from acquired immunodeficiency syndrome (AIDS). Despite the medical treatment the evolution led to blindness by bilateral total retinal detachment. The neuro-ophthalmological features occurred prior to the retinal manifestation, and the acute optic neuromyelitis occurred after a spreading zoster. The varicella-zoster virus (VZV) seemed to be involved because of recurring cutaneous zoster, spreading of this zoster just before the AONM, previous reports showing a link between VZV and AONM, and VZV and ARN. However, our patient had first an AONM responding well to corticosteroid therapy following one month later by an ARN leading to blindness despite the antiviral treatments received as soon as possible. There is a chronical viremia+ in immunodepressed patients with recurring and spreading zoster. The rupture of the hemato-encephalic barrier observed in AONM could facilitate the invasion of the eye by the virus, leading to an ARN. This hypothesis could explain the two complications due to the VZV, the AONM and the ARN, the first one is of dysimmunitary origin and the second one could probably result of a direct viral attack of the retina. This should incite to treat as soon as possible each retrobulbar optic neuritis in patients with AIDS, especially if past history of zoster.

AIDS-Related Opportunistic Infections↗
PubMed1998

[Epileptic seizures, epilepsy and risk factors. Experiences with an investigation in Martinique. Epimart Group].

A prospective incidence study was carried out in the French Caribbean island of Martinique between May 1st 1994 and April 31st 1995. incidence was 80.6 (77.7 when standardized with 1990 U.S. population). This incidence was higher than that observed in the Swiss canton of Geneva where the same methodology was used. The individualized risk factors of first provoked and unprovoked seizures in Martinique were alcoholism, head trauma and cerebro-vascular accidents.

Epilepsy↗
PubMed1998

[Benign intracranial hypertension. Retrospective study of 20 cases].

PURPOSE: We conducted a retrospective study in 20 cases of idiopathic intracranial hypertension (IIH) referred to the ophthalmology out-patients department at the Centre Hospitalier Universitaire of Fort de France. The objectives were to characterize symptomatology, severity, disease course and to discuss treatment. METHODS: We performed a complete ophthalmological and neurological examination, calculated the body mass index (BMI) and evaluated visual field. Each patient underwent a CT scan with injection and a lumbar punction with measurement of cerebrospinal fluid pressure. We also specified the treatment chosen and its duration, and the number of recurrences. RESULTS: This study included 20 cases, 19 women and 1 man, with a mean follow-up of 28.6 months. The average BMI was of 32.2 kg/m2. Fifteen patients out of 20 were obese (75%), all of them women, so 79% of the women were obese. Sixteen patients had headache (80%), 15 a loss of visual acuity (75%) and 5 transient visual loss (25%). For all the clinical examination showed bilateral papilloedema. Visual field disturbances were observed in 11 patients (73.3%). Papilloedema disappeared after medical treatment of a mean 2.4 months duration for each access. In 9 cases of 18 (50%) recurrences were observed after gradual treatment with-drawal. CONCLUSION: The long-term treatment, the side effects, the possible severity of the ocular disease, the difficulties of dietary restriction and the high rate of recurrence, would, for us, indicate a need to reconsider surgical treatment. We propose to perform lumboperitoneal shunt or optic nerve sheath decompression if medical treatment fails at the first onset of IIH or in case of severe visual loss, pregnancy or recurrence.

Adult↗
PubMed1997

Dominant X linked subcortical laminar heterotopia and lissencephaly syndrome (XSCLH/LIS): evidence for the occurrence of mutation in males and mapping of a potential locus in Xq22.

X linked subcortical laminar heterotopia and lissencephaly syndrome (XSCLH/ LIS) is an intriguing disorder of cortical development, which causes classical lissencephaly with severe mental retardation and epilepsy in hemizygous males, and subcortical laminar heterotopia (SCLH) associated with milder mental retardation and epilepsy in heterozygous females. Here we report an exclusion mapping study carried out in three unrelated previously described families in which males are affected with lissencephaly and females with SCLH, using 38 microsatellite markers evenly distributed on the X chromosome. Most of the X chromosome was excluded and potential intervals of assignment in Xq22.3-q23 or in Xq27 are reported. Although the number of informative meioses did not allow a decision between these two loci, it is worth noting that the former interval is compatible with the mapping of a breakpoint involved in a de novo X;autosomal balanced translocation 46,XX,t(X;2)(q22;p25) previously described in a female with classical lissencephaly. In addition, haplotype inheritance in two families showed a grandpaternal origin of the mutation and suggested in one family the presence of mosaicism in germline cells of normal transmitting males.

Adult↗
PubMed1997

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome.

We report a new syndrome that we call "recurrent optic neuromyelitis with endocrinopathies" in eight Antillean women from Martinique and Guadeloupe Ocular involvement was either monocular or binocular, whereas myelopathy was acute or subacute. In seven patients, myelopathic symptoms recurred, and in six patients, visual problems recurred. Spinal cord involvement was a consistent band-like pseudo-syringomyelic dissociated sensory loss. All eight patients had endocrinopathies consisting of amenorrhea, galactorrhea, diabetes insipidus, hypothyroidism, or hyperphagia. Spinal cord MRI revealed cavitation-like images. Various immunosuppressant treatments had little effect on the uniformly deteriorating course, ending in blindness and paraplegia. Six patients died within 5 years of onset, and an autopsy in one patient showed multiple demyelinizing lesions of the spinal cord with thickened blood vessels walls without evidence of inflammation. These cases appear to constitute a syndrome distinct from MS and from classic Devic's syndrome, not only because of the association with endocrinopathies but because of the stereotypy of the recurrences, the absence of MRI lesions in the cerebral white matter, and the unusual image of cavitation of the spinal cord. The syndrome is also distinct from HTLV-I-associated paraparesis, which is endemic in the West Indies.

Adolescent↗
PubMed1997

[Extensive cerebral venous thrombosis resistant to heparin: local fibrinolysis with urokinase].

A 37 year-old man had headaches for 10 days, then a single tonic-clonic seizure and coma due to an extensive cerebral venous thrombosis. In spite of full-dose heparin treatment for 7 days, the clinical picture worsened along with increasing edema on CT-Scan. Direct thrombolytic treatment was then performed using transvenous catheterization and instillation of Urokinase (2.6 MU over 4 days). A near complete repermeabilization of the sinuses was obtained and the patient improved dramatically in a few days. The only adverse effect of Urokinase was hematuria. Based on our experience and review of the literature which includes 26 previous cases, direct thrombolytic therapy appears to be a relatively safe procedure. This treatment should be considered in a patient with extensive dural sinus thrombosis which fails to respond to heparin treatment.

Adult↗