1/54. Chronic inflammatory demyelinating polyradiculoneuropathy: superiority of protein A immunoadsorption over plasma exchange treatment.We present a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who was treated regularly with plasma exchanges (PE) because response to other therapies including i.v. immunoglobulins was not adequate. To reduce nonspecific protein losses due to repeated PE and increase IgG-removal, immunoadsorption (i.a.)-therapy using sepharose-bound protein A was initiated. Retrospective analysis of clinical data including muscle strength and walking distance shows that IA led to more rapid and greater functional improvement than PE in this patient with no relevant side effects. After 3 years of therapy lymphoma was diagnosed and treated. The patient had no relapses of CIDP for 17 months, when his functional status deteriorated again necessitating further IA-therapy. It is concluded that IgG removal by IA in CIDP is more effective and has fewer complications than PE. Due to the chronic course of CIDP requiring repeated interventions IA is also not more expensive than PE.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/54. Prominent effect of immunoadsorption plasmapheresis therapy in a patient with chronic inflammatory demyelinating polyneuropathy associated with hepatitis b infection.We encountered a patient with chronic inflammatory demyelinating polyneuropathy associated with hepatitis b infection. Immunohistochemical study revealed the deposition of immune complex composed of hepatitis b surface antigen (HBsAg) both around the endoneural capillary and in the endoneurium. Neurological signs were significantly improved by immunoadsorption plasmapheresis (IAPP) treatment without incorporating corticosteroid hormone therapy; weekly long-term IAPP has successfully maintained the patient's condition.- - - - - - - - - - ranking = 2.4591093245743keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
3/54. Asymmetrical polyneuropathy with a stepwise progressive course and well-demarcated areas of demyelination.A female patient was 12 years old when she presented with hemiatrophy and muscle weakness on the right side of her body. Then a stepwise worsening occurred, and at 19 years of age sensory symptoms were also noticed, as well as a mild involvement of the left part of her body. The cerebrospinal fluid (CSF) protein level was elevated without cells. The main electrophysiological abnormality was a marked temporal dispersion of the compound muscle action potentials (CMAPs). Motor nerve conduction velocities were moderately reduced. A superficial peroneal nerve biopsy revealed well-demarcated areas of demyelination with prominent Schwann cell hyperplasia. Neither deletion nor duplication of the PMP22 gene nor mutation of the P0 or connexin 32 genes was found by molecular genetic investigations. immunotherapy was administered, and over the next 6 years the symptomatology fluctuated. This unusual disorder seems to be a variant of chronic acquired demyelinating polyneuropathy and may be immunologically mediated.- - - - - - - - - - ranking = 0.011391679968235keywords = demyelinating (Clic here for more details about this article) |
4/54. Polyneuropathy with osmiophilic membrane-bound, cytoplasmic inclusions in schwann cells (POMCIS).In the cytoplasm of schwann cells of a sural nerve biopsy from a 21-year-old female patient with chronic neuropathy we noted numerous unique, usually double membrane-bound, osmiophilic, granular or globular inclusions, approximately 30-600 microm in diameter. Some of these membrane-bound vesicular or tubular structures contained less dense or no osmiophilic inclusions. Morphometry revealed a reduction of the myelin area per endoneural area to approximately 13% (normal value: 20- 30%) and of the density of myelinated nerve fibers to 5,412/mm(2) (normal value at this age: 6,000-9,000/mm(2)). Large myelinated nerve fibers were predominantly reduced in number, and no myelinated nerve fibers with diameters larger than 4.5 microm were seen. Numerous, usually small onion bulb formations indicated a predominantly demyelinating type of neuropathy. This is to the best of our knowledge the first case of a chronic demyelinating neuropathy in which this kind of presumably pathognostic deposits in the cytoplasm of schwann cells was detected.- - - - - - - - - - ranking = 0.02278335993647keywords = demyelinating (Clic here for more details about this article) |
5/54. Lumbosacral acute demyelinating polyneuropathy following hepatitis b vaccination.We report a patient who presented with an acute inflammatory demyelinating polyneuropathy, that followed the second injection of a hepatitis b vaccination, and characterized by motor and sensory deficit restricted to lower limbs and perineum, and persistent bladder dysfunction. The relationship between the preceding event and neurological disease is discussed.- - - - - - - - - - ranking = 0.17261125364423keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
6/54. Three families with polyneuropathy associated with monoclonal gammopathy.OBJECTIVE: To report familial occurrence of polyneuropathy associated with monoclonal gammopathy. DESIGN: case reports. patients: We describe 6 patients (3 pairs) with a polyneuropathy associated with IgM monoclonal gammopathy. Four of the 6 patients had a demyelinating polyneuropathy on neurophysiological examination. Three patients had elevated antibodies against myelin-associated glycoprotein. No duplication on chromosome 17 or a mutation on chromosome 1 was found in any family. CONCLUSION: Familial occurrence of polyneuropathy without the presence of hereditary motor and sensory neuropathy type I is a reason to search for the presence of monoclonal gammopathy.- - - - - - - - - - ranking = 0.011391679968235keywords = demyelinating (Clic here for more details about this article) |
7/54. Trovafloxacin-induced weakness due to a demyelinating polyneuropathy.fluoroquinolones have been associated with peripheral sensory disorders and weakness, especially in patients with underlying myasthenia gravis or myasthenia-like Eaton-Lambert syndrome. Trovafloxacin is a relatively new quinolone for which these side effects have not been described. We report a case of diffuse weakness due to a demyelinating polyneuropathy that began after initiation of trovafloxacin in a patient without an underlying neurologic disorder.- - - - - - - - - - ranking = 0.056958399841176keywords = demyelinating (Clic here for more details about this article) |
8/54. Predominant arm weakness in acute idiopathic polyneuritis: a distinct regional variant.Acute idiopathic polyneuritis is a heterogeneous disorder with a number of clearly established variants. We describe four patients who present with an acute neuropathy predominantly affecting the arms which we believe should be considered as a distinct variant of acute polyneuritis. In two cases a primary demyelinating process affecting both motor and sensory nerves is suggested whereas in the other two the features were of a pure motor axonal degeneration. The relationship between these neuropathies, other variants of acute idiopathic polyneuritis and multifocal motor neuropathy is considered.- - - - - - - - - - ranking = 0.011391679968235keywords = demyelinating (Clic here for more details about this article) |
9/54. An unusual neuropathy in a diabetic patient: evidence for intravenous immunoglobin-induced effective therapy.We report the case of a 68-year old type-2 diabetic male patient who was admitted to hospital for progressive weakness in the right lower limb. Although his metabolic control was good, he lost more than 20 kg of weight. Despite intensive physio- and vitaminotherapy, his neurological condition kept on degrading with a severe amyotrophy and pain of the right thigh. He was unable to walk and to stand alone. Besides a yet known sensitive polyneuropathy, the electrophysiological study revealed an obvious motor involvement with signs of demyelination and axonal degeneration. Combined with the albuminocytologic dissociation observed in the cerebrospinal fluid, these specific clinical and electrophysiological features led us to postulate a diagnosis of inflammatory neuropathy. The patient underwent a treatment by methylprednisolone and immunoglobins that rapidly induced a striking improvement of his neurological condition. This case report illustrates that rare forms of neuropathy such as inflammatory neuropathies close to chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in diabetic patients and superimpose on the more commonly described forms of neuropathies. It recalls the importance of recognizing CIDP-like neuropathies because unlike other forms of neuropathy, inflammatory neuropathies are perfectly curable.- - - - - - - - - - ranking = 0.49182186491486keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/54. Solitary osteosclerotic plasmacytoma: association with demyelinating polyneuropathy and amyloid deposition.A 51-year-old man presented with a 1-year history of polyneuropathy necessitating the use of a wheelchair. Initial diagnosis was idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) and associated monoclonal gammopathy. Investigations for multiple myeloma, including bone marrow aspiration and biopsy, were negative. What was initially felt to be an incidental osteosclerotic focus noted on the radiographic bone survey was eventually shown to be a solitary osteosclereotic plasmacytoma with associated amyloid. This dramatically altered treatment. This case emphasizes the importance of including osteosclerotic plasmacytoma in the differential diagnosis of a focal sclerotic bone lesion in the clinical setting of polyneuropathy. These lesions are less likely to progress to multiple myeloma than lytic plasma cell neoplasms, and the presence of polyneuropathy often results in earlier diagnosis and treatment with enhanced prospect of cure. The finding of amyloid deposition within the osteosclerotic lesion may be of prognostic importance.- - - - - - - - - - ranking = 0.5373885847878keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
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