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A Drouet

Publications and source records attributed to A Drouet.

At least 19 recordsLinked to original sources
PubMed2006

[Polyneuropathy involving cranial nerves associated with monoclonal IgM antibodies with anti-MAG/SGPG/SGPLG/sulfatides activity].

INTRODUCTION: A typically distal and symmetrical, slowly progressive sensorimotor demyelinating neuropathy is caused by monoclonal IgM against myelin-associated glycoprotein (MAG) and SGPG, SGLPG glycolipids in the context of a benign IgM paraproteinemia. We studied a patient with a neuropathy that fulfilled the diagnostic criteria for CIDP in whom IgM kappa anti-MAG/SGPG/SGLPG were detected. OBSERVATION: The patient was a 57-year-old man who had developed a slowly progressive distal sensorimotor neuropathy, involving the lower then upper limbs, with cranial nerves palsies (oro-pharyngo-laryngo territory). ENMG showed a demyelinating neuropathy with a disproportionate slowing of conduction in distal segments of motor and axonal features in the lower limbs. The first routine laboratory analysis revealed negative or normal findings. Several serum protein electrophoreses were normal. The third cerebrospinal fluid examination demonstrated a moderate and late rise in CSF protein level with no cells. Monoclonal IgM-kappa against MAG/SGPG/SGLPG, was detected; anti-MAG antibody titre in the serum was 20 059 BTU (N<1000). A small IgM-kappa paraprotein was identified by immunofixation. Electron microscopy failed to show nerve fibers with widening of outer lamellae of the myelin. There is no clinical improvement after different treatments, immunoglobulins IV, cortisteroids, plasma exchange, rituximab. CONCLUSION: It is not known whether this neuropathy is an atypical form of PNMAG or an CIDP associated with anti-MAG. When ENMG show a disproportionate slowing of conduction in distal segments of motor nerves, one should screen the serum with immunofixation to identify small monoclonal components. If IgM-MGUS is present, search should be undertaken for anti-MAG/SGPG/SGLPG antibodies. Diagnosis enables optimal treatment using, in severe cases, expensive current strategies with immunoglobulins IV, plasma exchange, and corticosteroids, or, in the event of no response, rituximab before resorting to more toxic drugs like cyclophosphamide.

Antibodies, Anti-Idiotypic↗
PubMed2006

[Spinal dural arteriovenous fistula with peri-medullary venous drainage: analysis of a series from a single centre and review of the literature].

INTRODUCTION: The optimal diagnosis and therapeutic managements of spinal dural arteriovenous fistulae, a rare disease, is discussed. MATERIAL AND METHODS: We report a series of 10 patients seen in a 7-year period, treated by embolization. RESULTS: There were 6 males and 4 females, with an average age of 58.4 years (range: 31 to 74 years). Diagnosis was made 2 days to 5 years (mean 15.3 months) after symptom onset with a high rate of incomplete or atypical clinical patterns (prolonged isolated sensory disorder, lumbo-sciatalgia, claudication, monoplegia), or sudden-onset deficit. In all cases, spinal MRI showed an intramedullary high-intensity signal on T2-weighted images but enlarged intradural vessels in only 70p.cent of cases. Angiography showed in all cases an arteriovenous fistula at the upper dorsal level (T1 to T7, 5 cases), lower dorsal (T8-T12, in 3 cases) and lumbar levels (2 cases). The fistula was successfully obliterated after initial embolization in 3 cases, but two (2 cases) or 3 procedures (2 cases) were sometimes required. The endovascular therapy failed in 3 cases. CONCLUSION: In this series, the fistula was successfully obliterated in 70p.cent of patients. In the literature, fistula of 96.8p.cent to 97.9p.cent of patients were obliterated by surgery, which is a more invasive treatment. Embolization could be used as the first-line therapy, but incomplete obliteration requires rapid surgery. While the success of embolization is demonstrated, MRI and angiography must still be performed 6 months later.

Adult↗
PubMed2005

[Subacute meningoradiculomyeloencephalitis due to cryptococcosis infection].

INTRODUCTION: If meningoencephalitis with or without mass lesion (granuloma or abscess) is the most common pattern of CNS cryptococcal infection, intramedullary involvement is very uncommon. EXEGESIS: The authors report an 70-year-old male with Hodgkin's disease treated by chemotherapy then corticosteroids because of pulmonary fibrosis who was presenting for eight days ago, an ataxia, pyramidal syndrome, and bradypsychy. Spinal MRI revealed a gadolinium T1 weighted homogeneous enhancing T4 level intramedullary lesion. CSF had showed 190 GB/mm3 of lymphomonocytes, increased protein level (2.28 g/l), decreased glucose level (1.5 mmol/l) and positivity for crytococcal antigen. Treatment by amphotericine B and flucytosine then fluconazole for six months was instituted and symptoms gradually improved. CONCLUSION: A cryptococcus infection must be searched by antigen in CSF in case of myelopathy isolated or associated with meningoradiculoencephalomyelopathy, specially in patients with a cellular immunodeficience. Antimycotic agents must be firstly used, surgery would be restricted to decompression if aggravation of disease and compressive effect on the adjacent structures radiologically (MRI) became evident. Prolonged treatment is necessary in case of immunodeficience.

Aged↗
PubMed2005

[Polyneuropathy associated with neurofibromatosis].

INTRODUCTION: We review the literature on the spectrum of polyneuropathies associated with neurofibromatosis 1 (NF1) and 2 (NF2). BACKGROUND: Symptomatic neuropathies in NF1 are rarer than in NF2, but constitute a potentially severe complication associated with frequent morbidity related to the neuropathy itself, spinal cord compression or peripheral nerve sheath tumor malignant degeneration. Neuropathies are typically observed in young men with subcutaneous neurofibromas (NF1) or cutaneous schwannomas (NF2) and are characterized by a chronic slowly worsening sensorimotor polyneuropathy of the lower limbs. In NF1, demyelinating neuropathy may occur alone or in association with axonal features, whereas in NF2, axonal neuropathies are reported. Large multinodular roots and nerves, which can be easily detected by limb MRI, are characteristic features of NF1-associated polyneuropathies. PERSPECTIVES AND CONCLUSION: Although an associated pathogenic factor may reveal an asymptomatic neuropathy, patients should be monitored carefully because of the increased morbidity and mortality related to the significant proportion of malignant nerve-sheath tumors in these patients with NF1.

Humans↗
PubMed2004

[Paraneoplasic pandysautonomia with anti-Hu antibodies: a presentation of pulmonary adenocarcinoma identified by PET scanning].

A 74-year-old man developed pandysautonomia with severe orthostatic hypotension. Search for a paraneoplastic etiology was confirmed with the positivity of anti-Hu antibodies. [18] Fluorodeoxyglucose positron emission tomography (PET) enabled recognition of a lung adenocarcinoma. We emphasize the usefulness of PET scans for early diagnosis of cancer.

Adenocarcinoma↗
PubMed2004

[Split-cord malformation (diastematolmyelia) presenting in two adults: case report and a review of the literature].

INTRODUCTION: Split cord malformation (SCM) is an uncommon developmental anomaly characterized a cleft spinal cord. In type I, each of the hemicords is contained within an individual dural tube whereas in type II there is a common dural tube housing both hemicords. Commonly diagnosed in childhood, adult presentation is exceptional. METHODS: We report the case of two women whose type II SCM was discovered at the age of 40 and 54 years. RESULTS: The first patient complained of chronic lombar and radicular chronic pain with dysuria. Physical examination revealed a radicular syndrome with abolition of the left Achille reflex and a lombar hair tuft. MRI showed a disc herniation at the L5-S1 level, with a partial SCM at the level of the L2 vertebra, spina bifida and tethered cord. The second patient complained of lombar pain with perineal irradiation for 6 years. Physical examination showed a lombar cutaneous angioma. MRI revealed a thoraco-lombar SCM at the T12 to L1 level, with spina bifida. No spur could not be identified in either patient. No further surgical treatment was given. CONCLUSION: Based on these two observations, we propose a review of literature reporting 90 cases of adult SCM.

Adult↗
PubMed2003

[Acute severe leukoencephalitis with posterior lesions due to Borrelia burgdorferi infection].

INTRODUCTION: Central nervous system manifestations represent 0.54 to 8% of neurological complication in Lyme disease. OBSERVATION: A 78-year-old woman presented a severe meningo-encephalitis with visual disorders (agnosia, alexia) progressing towards coma. Cranial magnetic resonance imaging revealed large areas of hypersignal T2 in the white matter of the lower, parieto-occipital lobes and left temporal lobe. The cerebrospinal fluid (CSF) contained 16 then 293 white corpuscles/mm3 of lympho-monocytes, increased protein level from 2.67 to 5.83 g/l and an increase in IgG index with oligoclonal distribution of IgG. Serological Elisa analysis for Lyme disease was slightly positive in blood (confirmed by western blot) but clearly in the CSF (IgG and IgM). Treatment with ceftriaxone followed by methylprednisolone provided clinical improvement 3 months later. DISCUSSION: Acute meningo-encephalitis is often benign, protein-like and of good prognosis: the gnosic visual disorders with posterior leukoencephalopathy are unusual. A blood level of specific antibodies slightly positive on Elisa at the early stage of the infection warrants confirmation by Western blot in the blood and by Elisa in the CSF. Additional corticosteroid therapy may be required in the severe forms that evoke acute disseminated encephalomyelitis.

Acyclovir↗
PubMed2003

[Analysis of 12 cases of McArdle's disease diagnosed after 30 years].

PURPOSE: McArdle's disease (MAD) or glycogen storage disease type V, usually starts in childhood or adolescence. Generally diagnosis is made before the early adulthood because patients present well defined syndrome and are constrained. METHOD: We retrospectively investigated all MAD cases diagnosed in the biochemical laboratory from Debrousse Hospital in Lyon, during 40 years (1962-2002). We then selected patients whose diagnosis had been made after 30 years. RESULTS: Fifteen patients answered our criteria but only 11 files could be analysed. A twelfth patient (service of internal medicine--Royan) supplemented the series. We sought the reasons of a late diagnosis: early age of beginning but few symptoms (7 cases), age of beginning higher than 20 years (5 cases including 3 after 45 years). The principal symptoms were muscular deficit and muscular pains (8 cases) and second wind phenomenon (7 cases). Creatinine phosphokinase level was constantly high. Ischemic effort test when it was carried out was constantly abnormal. Conversely electromyogram was often normal (5 cases). Several biopsies were necessary in a third of the cases to evoke the diagnosis, particularly among the patients with late onset symptoms. CONCLUSION: Diagnosis of metabolic MAD is generally easy if the interrogation finds inaugural symptoms in childhood or adolescence even if the patient consults very late in the life. The diagnosis can become much more difficult if it begins late in life (atypical symptoms, need for several muscular biopsy).

Adult↗
PubMed2003

[Usher syndrome: a case report].

A 60-year-old Jewish woman with consangineous parents had a history of severe sensorineural hearing loss since the age of 2 years. Hearing loss had not progressed since childhood, but her visual impairment due to pigmentary retinopathy, known since childhood, had worsened 15 years ago. The diagnosis was Usher syndrome type I, a rare heterogeneous disorder of autosomal recessive inheritance. Abnormal vestibular function and ataxia with neuroimaging anomalies including cerebellar atrophy have been reported, suggesting the disease process also involves the brain.

Brain↗
PubMed2003

[Calcified brain metastases of a pulmonary neuroendocine carcinoma].

We describe an unusual case of pulmonary neuroendocrine carcinoma of intermediate cell type, revealed by calcified metastatic nodules of the brain in a 49-year-old man. Histological examination of the brain lesions disclosed necrosis and calcification in the center with small and medium-sized carcinoma cells. Pathophysiological mechanisms of such calcifications in metastasis lesions are reviewed.

Brain Diseases↗
PubMed2003

[Hashimoto's thyroiditis associated with recurrent transverse myelopathy].

A 55-year-old woman presented first episode of paraplegia at D6 level with a marked improvement of neurological signs, but relapse occurred five months later. Hashimoto's thyroiditis with euthyroidism was found. Long-term 9 months treatment with oral corticosteroids was applied. Also she was able to walk with help. Two years after the second palsy she developed a new paraplegia at D6 level with a severe residual deficit five months later in spite of oral corticosteroids. Thyroid antibodies remained positive. Devic's disease without optica neuritis associated with thyroiditis or spinal cord localisation of Hashimoto's encephalopathy was discussed.

Atrophy↗
PubMed2002

[Muscular exercise intolerance syndrome in Becker muscular dystrophy].

OBJECTIVE: To determine whether exertional muscle pain syndrome (EMP) is a benign phenotype or an early stage of Becker-type muscular dystrophy (BMD). METHODS: Muscle dystrophin analysis led to the diagnosis of BMD in 6 patients complaining of EMP. RESULTS: Three patients had a history of X-linked inheritance and age at clinical onset was 4 to 11 years in five, and one patient had a later onset aged 23. Pseudohypertrophy of the calf muscles was absent in one patient, but all had experienced mild (5/6) pelvic weakness and (or) atrophy one to 17 years after the onset. High serum CK level was present (X 14). Normal anti-dystrophin immunostaining in two cases did not rule out the diagnosis that was only made made by Western blot analysis or genetic studies. All exhibited in-frame deletions (exons 45-48) within the dystrophin gene. CONCLUSION: The 36 patients with BMD-EMP analysed in the literature, exhibited different deletions and no worsening in 66.7% of cases. Western blot was more precise than immunolabelling with 96.8% positivity versus 70.5%. Dystrophin analysis by Western blot and (or) DNA analysis should be included in the evaluation of patients with EMP syndrome without deficient muscle energy metabolism, particularly those with pseudohypertrophy of the calf muscles or high serum CK levels.

Adolescent↗
PubMed2002

[Diagnostic value of classical neurophysiologic profile in various phenotypes of hereditary, pressure-sensitive neuropathies].

PURPOSE: We looked for electrodiagnostic features that raise suspicion of hereditary neuropathy with liability to pressure palsies (HNPP). METHOD: A retrospective review of eight cases with confirmed histologic (one case) or chromosome 17 deletion (seven cases) analysis was performed. RESULTS: Autosomal dominant disease was present in 63% of the patients, 75% being men. Mean age at examination and at first symptom was 27 and 22 years respectively. Five patients have one or more acute nerve palsies, without residual deficit, but one presented sensory symptoms in internal saphena territory. Three patients had others phenotypes: a man presented with four episodes of facial palsy and one woman was asymptomatic; another patient with diabetes mellitus presented an associated chronic sensorimotor polyneuropathy. In agreement with other studies of HNPP we found in most patients a diffuse increase in distal motor latence, contrasting with normal or moderately decreased motor nerve conduction velocity, multiple electrophysiologic entrapment and diffuse reduction in sensory nerve action potential and/or velocity. However, this electrophysiological pattern was incomplete or masked in HNPP with cranial nerves palsies, in asymptomatic form of HNPP or in HNPP associated with polyneuropathy. CONCLUSION: These data confirm the clinical phenotypic heterogeneity of the 17p11.2 deletion and highlight the limits of the classic electrophysiological pattern in asymptomatic HNPP or associated with uncommon clinical features.

Adolescent↗