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Response to immunosuppressive therapy in patients with hereditary motor and sensory neuropathy and associated dysimmune neuromuscular disorders.

We encountered 2 patients with hereditary motor and sensory neuropathy (HMSN) type I who had marked weakness developing during several months superimposed on chronic peroneal muscular atrophy. Further studies disclosed a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in one patient and CIDP associated with polymyositis in the other. Both patients responded to prednisone and azathioprine with substantial improvement. Patients with HMSN who develop rapid progression of weakness should be evaluated for superimposed, potentially treatable dysimmune neuromuscular disorders.

Окулистические и нейро-офтальмические проявления хронического воспламенения демилилинизации Полиневолюции.

Хроническая воспламенительная демиелинация полирадикулонэропропотии (CIDP) — иммунопосредованная неврапатия, характеризующаяся демиелинацией и аксонной травмой периферийных нервов. В то время как его отличительная характеристика включает в себя прогрессивные сенсорные недостатки, недопризнанная подгруппа пациентов развивает окулярные и нейро-офтальмические проявления. К ним относятся оптический неуриит, оптическая атрофия, потеря зрения, офтальмоплегия, экстраокульное расширение мышц с проптозом, внутричерепная гипертония и маленькая волокнопатология. Электрофизиологические и визуальные исслед

Совпадение в аннотации оригинала
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated neuropathy characterized by demyelination and axonal injury of peripheral nerves. While its hallmark presentation involves progressive sensorimotor deficits, an underrecognized subset of patients develop ocular and neuro-ophthalmic manifestations. These include optic neuri…

Guillain-Barré syndrome in children.

The Guillain-Barré syndrome is a pediatric neurologic emergency and the most common cause in children of rapidly evolving, usually flaccid, weakness with associated areflexia. Two treatable illnesses, namely tick paralysis and acute cord compression, demand immediate differential diagnosis. Rarely, poliomyelitis still mimics infantile Guillain-Barré syndrome. Specific precursor infections, such as Campylobacter jejuni, are now recognized to affect the clinical presentation of Guillain-Barré syndrome. Cerebrospinal fluid evaluation and electromyography are usually diagnostic; new modalities, su

Focal and entrapment neuropathies.

Of the many patterns of peripheral nerve disorders in diabetes mellitus (DM), isolated clinical involvement of single nerves, though less common than distal symmetric polyneuropathy and perhaps polyradiculoneuropathy, constitute an important collection of characteristic syndromes. These fall into four anatomical regions of the body: cranial, upper limb, truncal, and lower limb territories. Each of these groups of mononeuropathies has its own ensemble of epidemiologic patterns, clinical presentations, laboratory and radiologic findings, differential diagnosis, management principles and prognosi…

Treatment of acute immune-mediated neuropathies: Guillain-Barré syndrome and clinical variants.

The author reviews current treatment approaches for the management of acute immune-mediated peripheral neuropathies, including the Guillain-Barré syndrome together with the clinical variants, which are acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, and the Fisher syndrome. A summary of clinical evidence for drug therapies is provided, with additional recommendations for commonly used treatment modalities including a focus on the approach to using intravenous immune globulin and plasma exchange therapy.

Neuromuscular complications of human immunodeficiency virus infection and antiretroviral therapy.

At least 4 distinct peripheral neuropathy syndromes occur in patients infected with the human immunodeficiency virus. The most common, painful sensory neuropathy, may be related to the viral infection or may be medication induced and is treated symptomatically. The other 3, chronic inflammatory demyelinating polyradiculoneuropathy, mononeuropathy multiplex (some patients), and the progressive polyradiculopathies related to the acquired immunodeficiency syndrome, may all respond to appropriate therapy. Both inflammatory myopathy and zidovudine myopathy also abate with early diagnosis and treatm…

Changes in electrophysiological findings suggestive of demyelination following Guillain-Barré syndrome: A retrospective study.

INTRODUCTION/AIMS: Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common form of Guillain-Barré syndrome (GBS) in Western countries. However, electrophysiological descriptions of changes in abnormalities suggestive of demyelination after an AIDP episode are rare. We aimed to describe the clinical and electrophysiological features of AIDP patients after the acute episode, to investigate changes in abnormalities suggestive of demyelination and to compare with electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: W…

Plasmapheresis Versus Intravenous Immunoglobulin in Patients With Autoimmune Neuromuscular and Neuro-immunological Conditions.

OBJECTIVES: Plasmapheresis (PLEX) and intravenous immunoglobulin (IVIg) are commonly used to treat autoimmune neuromuscular disorders, including myasthenia gravis, acute inflammatory demyelinating polyradiculoneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, and other autoimmune neurological disorders. The side effect profiles of these therapies vary, and concern has been raised regarding the safety of PLEX in the elderly population. In this study, we have examined the pattern of PLEX and IVIg use for autoimmune neurological disorders at a single facility and in a national…

CSF sphingomyelin: a new biomarker of demyelination in the diagnosis and management of CIDP and GBS.

OBJECTIVE: To validate sphingomyelin (SM) dosage in the cerebrospinal fluid (CSF) of patients affected by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Guillain-Barré syndrome (GBS) as a reliably assessable biomarker. METHODS: We prospectively enrolled 184 patients from six Italian referral centres, in whom CSF SM levels were quantified by a fluorescence-based assay optimised and patented in our laboratory. RESULTS: We confirmed increased levels of SM in the CSF of patients affected by typical CIDP (n=35), atypical CIDP (n=18) and acute inflammatory demyelinating polyr…

Nerve and skin biopsy in neuropathies.

PURPOSE OF REVIEW: To give an overview of recent data on the use of nerve and skin biopsy as a diagnostic tool in neuropathies. RECENT FINDINGS: Axonal damage in a biopsy from a patient with chronic inflammatory demyelinating polyradiculoneuropathy may point to the presence of autoantibodies to paranodal proteins. In nonsystemic vasculitis of the peripheral nervous system, nerve biopsy is still the only means to make a definite diagnosis. Increased autophagy has been found in idiopathic neuropathy and may also be a common final pathway in various types of neuropathy. Nerve biopsy has unexpect…

Prognosis of Guillain-Barré Syndrome in Children.

OBJECTIVE: Guillain-Barre Syndrome (GBS) is an acute polyradiculoneuropathy characterized by progressive motor weakness of limbs and areflexia. In this study, our aim was to evaluate the clinical pattern and prognosis of children with Guillain-Barre syndrome. MATERIALS & METHODS: This cross-sectional study was conducted in the Pediatric Neurology Unit of Amirkola Children's Hospital, Babol, Iran during the period of 5 years from October 2008 to September 2013. We assessed the clinical features, results of electrodiagnostic tests, functional status, treatment and outcome of 17 children diagnos…

[Non-infective neurologic complications associated to heroin use].

The spectrum of neurological complications associated with heroin addiction has changed in the past six years because of the progressive kno … y and neuromuscular complications such mononeuropathy, plexopathy, acute inflammatory demyelinating polyradiculoneuropathy, rhabdomyolysis, fibrosing myopathy, musculoskeletal syndrome and acute bacterial myopathy. Some of such complications (i.e. transverse myelitis, polyradiculoneuropathy, leucoencephalopathy) must rise the suspicion of an HIV infection. Likewise, in …

[Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome)].

Guillain-Barré syndrome is an acute immune-mediated polyradiculoneuropathy, which is the most common cause of acute paralysis and requires intensive therapy in one third of cases. Considering the fact that the disease is rare and characterized by low mortality, morphological changes in it are described rather modestly, based on isolated examples. The article analyzes literature data and our own observations of Guillain-Barré syndrome, including an analysis of clinical data, treatment, and the nature of structural changes based on routine, histochemical, and immunohistochemical studies.

Two unusual cases of Guillain-Barre syndrome after snake bite: the rare immune mediated complication of Elapid venom.

In this report, we present a series of two unusual cases of elapid snakebite with acute neuroparalysis, that after initial response to standard anti-snake venom therapy, developed recurrence of disabling quadriparesis with dysautonomia, that on detailed evaluation turned out to be immune-mediated polyradiculoneuropathy (GB syndrome). Both the cases then responded to therapy with intravenous immunoglobulins. These cases bring out the rare immune-mediated late complication of snake venom, which if recognized and treated in time, can significantly reduce the morbidity and mortality.

[Ruptured renal artery aneurysms in microscopic polyangiitis].

INTRODUCTION: Renal artery aneurysms are unusual manifestations of microscopic polyangiitis. OBSERVATION: We report the case of a 55 years old male patient, with a microscopic polyangiitis, which was revealed by a demyelinating polyradiculoneuropathy, the diagnosis was made with association of alveolar hemorrhage, peripheral neuropathy and glomerulonephritis with positive antineutrophil cytoplasmic antibodies (antimyeloperoxidase antibodies). The evolution was marked by a state of shock by rupture of aneurysms of intrarenal arteries. CONCLUSION: This case emphasizes the importance of looking…

Motor dominant neuropathy in Sjögren's syndrome: report of two cases.

Most of the peripheral neuropathies in Sjögren's syndrome (SS) are sensory- or autonomic-dominant. In this report, we present two cases of a rare type of neuropathy, motor dominant neuropathy, in SS. One showed signs similar to those of Guillain-Barré syndrome, and the other showed signs characteristic of chronic inflammatory demyelinating polyradiculoneuropathy. These patients received i.v. immunoglobulin therapy. To our knowledge, this is the first report indicating that i.v. immunoglobulin has beneficial effects on motor dominant neuropathy in SS.

Intravascular malignant histiocytosis mimicking central nervous system vasculitis: an immunopathological diagnostic approach.

A 53-year-old man presented with a lumbosacral polyradiculoneuropathy and developed fluctuating encephalopathy suggestive of multifocal small vessel disease. Postmortem examination demonstrated multifocal vascular occlusion by undifferentiated cells confined to the intravascular space. Extravascular spread was found only in spleen and liver. The presence of lysozyme and absence of factor VIII in the cytoplasm of the malignant cells confirmed their histiocytic nature. This patient had an unusual intravascular form of malignant histiocytosis that must be included in the differential diagnosis of…

POEMS syndrome: Update on diagnosis, risk-stratification, and management.

DISEASE OVERVIEW: POEMS syndrome is a life-threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. DIAGNOSIS: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal…