PubMed Health⌕ Search

Biomedical subjects

S Attarian

Publications and source records attributed to S Attarian.

At least 19 recordsLinked to original sources
PubMed2006

Multifocal motor neuropathy with and without conduction block: a single entity?

OBJECTIVE: To assess if multifocal motor neuropathy (MMN) with and MMN without conduction block (CB) are similar or distinct diseases. METHODS: The authors reviewed the clinical features and responses to IV immunoglobulin (IVIg) treatment of patients with MMN with and without CB at diagnosis, after 4 years of follow-up and at the last examination. They included all patients showing clinical features of MMN who had been followed for at least 4 years: All had asymmetric purely motor weakness with a peripheral nerve distribution, without any sensory, bulbar, or respiratory signs and without any upper motor neuron involvement. RESULTS: Twenty patients had CB and 13 had no CB. Median follow-up time was 7 years. There were no differences between the two groups in term of age, sex, time from onset to diagnosis, anti-GM1 antibody titers, or CSF data. Nerve distribution, number of affected limb regions, predominant weakness in distal upper extremities, asymmetric weakness, cramps, fasciculations, and Medical Research Council sum-scores in upper and lower limbs were comparable at diagnosis, 4 years of follow-up, and last examination. Few significant differences were observed. Involvement of median nerve was less frequent at 4 years of follow-up (14/20 vs 4/13; p = 0.027) and at the last examination (17/20 vs 5/13; p = 0.009) in patients without CB. Proximal weakness was less frequent in patients with MMN without CB at the last examination (7/20 vs 0/13; p = 0.027). Fewer nerves were involved in patients without CB at the last examination (4.5 vs 2; p = 0.04). Efficacy of IVIg was similar in MNN patients without CB (8/13) and with CB (14/20; p > 0.05). CONCLUSION: After a median follow-up time of 7 years, patients with and without conduction block showed similar clinical features and a similar response to IV immunoglobulin treatment.

Adult↗
PubMed2006

[Lambert-Eaton myastenic syndrome revealing neuroblastoma in 2 children].

Lambert-Eaton myasthenic syndrome is a paraneoplasic syndrome which can reveal a primitive tumor. Frequently, the first diagnosis is myasthenia gravis. This disease is extremely rare in children. Only 10 cases have been reported in the last 35 years. We report 2 new observations occurring in very young patients, aged 2 and 3 years, with a ganglioneurobastoma as primitive tumor.

Brain Neoplasms↗
PubMed2006

Covariation of corticospinal efficiency and silent period in motoneuron diseases.

For a better understanding of the changes affecting the cortically induced silent period (SP) in motoneuron disease, the excitatory and inhibitory effects of transcranial magnetic stimulation were explored repeatedly in 8 patients with amyotrophic lateral sclerosis (ALS), 3 patients with Kennedy's disease (KD), and 10 healthy subjects. In KD, the background electromyogram (EMG) and the motor evoked potential (MEP) area were both enhanced. However, neither the corticospinal efficiency (MEP gain, the ratio between MEP and background EMG) nor the duration of the SP differed from healthy subjects. In ALS patients, the MEP gain and the SP duration decreased conspicuously with time. We conclude that use of the MEP gain improves detection of corticospinal dysfunction in ALS patients. Part of the SP shortening in ALS seems to reflect the reduced activation of cortical or spinal inhibitory networks by the abnormal corticospinal pathway.

Adult↗
PubMed2006

[Stiff man syndrome: clinical forms, treatment and clinical course].

INTRODUCTION: Stiff-Man syndrome (SMS) is a rare neurological disease first described fifty years ago. There are several clinical forms, which are frequently misdiagnosed. The aim of this study is to review three of the main clinical forms. MATERIAL AND METHODS: Case reports concerning three women suffering from different forms of SMS are presented, giving the main clinical features, their associations with other diseases, and the biological and electrophysiological findings. RESULTS: The first patient presented a symmetric axial muscle rigidity, painful spasms and contractions of the trunk and limbs associated with anti-GAD antibodies. The common form of SMS was diagnosed and the patient was improved by intravenous immunoglobulin (IVIg). The second patient suffered from contractions and spasms localized to the lower limbs. In this patient, anti-GAD antibodies were absent. The Stiff-Leg syndrome was diagnosed and the patient was improved by intrathecal baclofen. The third patient presented rigidity of limb and trunk muscles associated with signs of encephalitis. In this patient, only anti-amphiphysin antibodies were present. The progressive encephalomyelitis with rigidity was diagnosed and the patient was improved by IVIg associated with corticosteroid. CONCLUSION: Identifying patients with SMS makes it possible to propose appropriate medical management. There are several forms of the disease, and the severity of the evolution differs in each case. Treatment with GABA-ergic inhibitory drugs, IVIg and corticosteroid improve both the symptomatology and the quality of life of these patients.

Aged↗
PubMed2005

Transcranial magnetic stimulation in lower motor neuron diseases.

OBJECTIVE: To study the diagnostic value of transcranial magnetic stimulation (TMS) in a group of patients with lower motor neuron disease (LMND). Among LMND, several chronic immune mediate motor neuropathies may simulate amyotrophic lateral sclerosis (ALS). METHODS: Forty patients with LMND were included TMS was performed at the first visit. The patients were seen prospectively every 3 months for a period of 1-4 years. RESULTS: Three different groups were distinguished at the end of follow-up: (1) ALS group with 7 patients, (2) Pure motor neuropathy with 14 patients and (3) Other LMND including 12 patients with hereditary spinal amyotrophy, 3 patients with Kennedy's disease and 4 patients with post-poliomyelitis. On the basis of the results of TMS variables, 6 out of 7 ALS patients had abnormality of silent period (SP) associated or not with abnormality of excitatory threshold or amplitude ratio. Patients with pure motor neuropathy had normal SP and amplitude ratio. Four out of 14 patients had increased central motor conduction time (CMCT), one had increased CMCT and excitatory threshold, and one patient had a slightly increased excitatory threshold. Considering the abnormality of TMS variables in the groups, SP, excitatory threshold, and amplitude ratio were chosen in a post-hoc attempt to select variables yielding high sensitivity and specificity. The overall sensitivity of TMS for diagnosis of ALS among LMND was 85.7%, its specificity was 93.9%. When only the abnormality of SP was taken into account, the sensitivity was unchanged. But the specificity was improved to 100%. CONCLUSIONS: TMS helped to distinguish suspected ALS from pure motor neuropathy.

Adult↗
PubMed2005

Towards the molecular elucidation of congenital myasthenic syndromes: identification of mutations in MuSK.

Congenital myasthenic syndromes (CMS) are rare genetic diseases affecting the neuromuscular junction (NMJ) and characterized by a dysfunction of the neurotransmission. They are heterogeneous at the pathophysiological level and can be classified in three categories according to their origin: presynaptic, synaptic or postsynaptic. The strategy for the diagnosis and characterization of CMS relies on the clinic, EMG, muscle biopsy, identification of mutations in genes known to be responsible for CMS and the demonstration that the gene mutations are the cause of the disease by using experimental approaches. As an example of such strategy, we report briefly here the characterization of the first case of a human neuromuscular transmission dysfunction due to mutations in the gene encoding a postsynaptic molecule, the muscle-specific receptor tyrosine kinase (MuSK). Gene analysis identified two heteroallelic mutations, a frameshift mutation (c.220insC) and a missense mutation (V790M). The muscle biopsy showed marked pre- and postsynaptic structural abnormalities of the neuromuscular junction as well as a severe decrease in acetylcholine receptor epsilon-subunit and MuSK expression. In vitro and in vivo expression experiments were performed using mutant MuSK reproducing the human mutations. The results obtained strongly suggested that the missense mutation, in the presence of a null mutation on the other allele, was responsible for the severe synaptic changes observed in the patient and, hence, is causing the disease. However the molecular origin of a large number of CMS is still unknown. There are hundreds of molecules known to be present at the NMJ and mutations in the genes coding for these synaptic molecules are likely to be responsible for a neuromuscular block.

DNA Mutational Analysis↗
PubMed2004

Myositis in infiltrative lymphocytosis syndrome: clinicopathological observations and treatment.

Diffuse infiltrative lymphocytosis syndrome (DILS) is characterized by the presence of persistent circulating CD8 lymphocytosis and visceral CD8 lymphocytic infiltration, predominantly in the salivary glands and lungs in a subset of human immunodeficiency virus (HIV) patients. Myositis has been rarely documented in DILS. We report here the histopathological abnormalities and treatment response of an HIV patient with DILS associated myositis. Muscle biopsy revealed features of polymyositis but electron microscopy study showed intranuclear filamentous inclusions of 18 nm diameter, without intracytoplasmic inclusion. Treatment with intravenous immunoglobuline was useful.

Antigens, CD↗
PubMed2004

[Neurophysiology of systemic diseases].

Connective tissue diseases represent a varied and challenging group of disorders. Neuromuscular structures are highly susceptible targets for damage. In this review, the neurophysiological explorations of the neuromuscular complications are examined with particular attention to the peripheral nerve system. The most common presentations are sensorimotor polyneuropathy, mononeuritis multiplex, distal symmetric neuropathy, compression neuropathy and trigeminal sensory neuropathy.

Connective Tissue Diseases↗
PubMed2004

Neuromuscular diseases associated with antigliadin antibodies. A contentious concept.

Many neurological syndromes associated with antigliadin antibodies have been published. The most frequent are cerebellar ataxia and peripheral neuropathy. Such cases are reported under the title of gluten ataxia or of coeliac neuropathy. However, associations do not prove cause. Herein, a short review is made of the literature and the author's personal experience. Many unsolved questions remain. Gluten sensitivity is probably an immunological response in genetically susceptible individuals with or without enteropathy. Antigliadin antibodies are present in healthy adults, in some idiopathic ataxias and neuropathies. A gluten-free diet is effective on gastrointestinal but not on neurological symptoms. Whether antigliadin antibodies are elevated is more of a waiting classification than a definite diagnosis. Finally, the relationship between antigliadin antibodies and neurological diseases is very weak.

Antibodies↗
PubMed2003

[Clinical and radiological features and clinical course of orbital myositis].

Orbital myositis is idiopathic inflammation of the extraocular muscles in the absence of thyroid orbitopathy and often is included under broad description pseudotumor. We report here a series of three cases. Data from literature, combined with our own results yield a distinguishing pattern of orbital myositis suggesting that the term "orbital pseudotumor" is no longer a useful concept. The diagnostic criteria purposed here are: acute orbital pain exacerbated on eye movement; enlargement of one or more extraocular muscles with the respect of other orbital structures on muscle CT scan; absence of clinical and biological thyroid dysfunction: absence of signs of anterior uveitis or scleritis or visual decrease; rapid response to immunomodulator treatment.

Adult↗
PubMed2003

[Muscle granuloma: anatomoclinical correlation and immunohistochemistry in seven cases].

Granulomatous inflammation is infrequently observed in muscle biopsy. We report a series of 7 patents presenting with granulomas in muscle. Two of them had a history of sarcoidosis In 4 other cases, muscle Involvement revealed systemic sarcoidosis. Among the 6 cases of sarcoidosis, we observed 2 with acute myositis and 4 chronic forms. The last patient presented with polymyositis in association with melanoma. In sarcoidosis, muscle biopsy showed a granulomatous inflammation of varying intensity, which was generally associated with mononuclear inflammatory cells. Most of granulomas were located in the perimysium and the endomysium and necrosis was absent. Inflammatory cells were predominantly macrophages and CD4 positive lymphocytes. On the contrary, in the case of paraneoplastic polymyositis,granulomas were rare, most of inflammatory cells were CD8 positive lymphocytes and numerous areas of necrosis were observed. Class I MHC molecules were expressed on the membrane of muscle fibers. As a general rule, requisite examinations must be performed to search for sarcoidosis in patients exhibiting granulomas on muscle biopsy.

Adult↗
PubMed2003

[Guillain-Barré syndrome revealing visceral leishmaniasis in an immunocompetent woman].

Visceral leishmaniasis is an endemic parasitic infection rarely observed in association with Guillain-Barré syndrome in immunocompetent patients. A 40-year-old immunocompetent woman was admitted to our unit with recent onset difficulty in walking. The neurological examination and electrophysiological study led to the diagnosis of Guillain-Barré syndrome. During hospitalization, she developed cytopenia involving all three lines revealing visceral leishmaniasis. A few cases of visceral leishmaniasis with neuropathy have been reported, mainly in tropical regions. Neuropathological manifestations of visceral leishmaniasis are probably underestimated. The question is whether Guillain-Barré syndrome and visceral leishmaniasis are causally related.

Adult↗
PubMed2003

[Chorea-acanthocytosis: report of a new family].

Chorea-acanthocytosis is a rare cause of chorea. The genetic origin of the disease was proven with the isolation of the gene on chromosome 9q21. We report the case of two sisters who had two different expressions of this disease. The first sister developed secondary epilepsy associated with abnormal movement of the face. The second presented abnormal movements of the face and apathy. Both improved with serotoninergic treatment. Diagnosis was suggested on the basis of clinical findings and confirmed by identification of acanthocyes and integrity of the Kell blood antigen. Search for acanthocytes should be performed in all patients with chorea. Chorea associated with acanthocytosis has a better prognosis than chorea arising from other causes. Other neurological manifestations or complications may be present or mot; long-term follow-up in a specialized center iq needed for prevention.

Adult↗
PubMed2002

[Acute encephalitis complicating acute polyradiculoneuritis].

We report the case of a 43-year-old man who developed an acute encephalopathy after IVIg therapy for AIDP. Imaging studies showed predominantly posterior leukoencephalopathy. The signs and symptoms of the encephalopathy completely resolved by steroids. Two patients with acute encephalopathy, following IVIg therapy, were reported previously in the literature. However, our observation differed from them by the presence of a marked pleiocytosis of cephalospinal fluid before beginning of IgIV. Two hypothesis may be made: a post-infectious meningo-encephalo-AIDP or a complication of IgIV. This side effect, even rare, has not to be underestimated.

Acute Disease↗
PubMed2002

[Guillain-Barre syndrome and its frontiers].

Knowledge concerning Guillain-Barré syndrome (GBS) has been largely extended these past ten years. The first description by Guillain, Barre and Strohl in 1916 established the main clinical and biological characteristics of the syndrome: acute motor weakness affecting at least one limb associated with areflexia. The syndrome was at first restricted to the concept of an acute inflammatory demyelinating polyneuropathy after the first large electrophysiological and histological studies (Asbury et al., 1969). This restricted definition of the GBS was changed by the report of Feasby in 1986 of an axonal variant of the syndrome. This proposal remained controversial until large studies reported that GBS in Northern China occurs in epidemics, predominantly involving young adults and children and characterized electrophysiologically by pure motor axonal features (AMAN). Additional studies showed that another GBS variant may result from an attack against both motor and sensory axons (AMSAN). Previously in 1956 CM Fisher described a clinical triad of ataxia, areflexia and ophthalmoplegia associated with albumino-cytologic dissociation and excellent recovery which was considered immediately part of the GBS. The physiopathology of the syndrome was recently determined (anti-GQ1b antibodies). A number of clinical variant of more limited scope have also been described. Plasma exchanges and intravenous immunoglobulin (IVIg) are the gold standard therapies for the demyelinating form and probably for the other variants despite the absence of controlled trials.

Acute Disease↗
PubMed2001

[Infectious myopathies].

Infectious myopathies are rare acquired affections which have, generally, a good prognostic. Many types of viral infections can cause transient inflammatory myopathies. HIV myopathy may be present early in the HIV infection, but more often it is a complication of fully developed AIDS. Influenza virus myositis tend to be more severe in adults than in children. Group B coxsackie virus has been isolated from striated muscle of patients with epidemic myalgia. Parasitic infections of muscle include trichinosis, toxoplasmosis, and cysticercosis. Trichinosis is the most frequent parasitic myositis. The ocular, lingual or pharyngeal weakness and/or hypereosinophilia suggest the diagnosis. Pyomyositis, is a located zone of suppuration of muscle due to staphylococcus in 90% of the cases. It is a common occurrence in tropical climates, but has been recognized increasingly in temperate climates.

Bacterial Infections↗