Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0152025 (polyneuropathy)
 7,862 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Forty-three consecutive patients with peripheral facial palsy were examined to evaluate the incidence of facial palsy caused by the tick-borne Borrelia spirochete in a non-coastal area in the south of Sweden. Six (14%) of the patients were found to have a Borrelia infection. The incidence of Borrelia-induced facial palsy was 0.39/10,000 inhabitants, which is less than in neighbouring coastal areas but still high enough to cause serious concern when dealing with facial palsies. In contrast to Bell's palsy, a Borrelia infection can cause serious generalized illness if not properly treated. Negative Borrelia serology of the serum and the cerebrospinal fluid (CSF) and a normal CSF analysis did not exclude the Borrelia etiology. Tick bites, signs of polyneuropathy, meningeal symptoms and Borrelia-associated erythema proved to be important signs for the diagnosis.
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PMID:Peripheral facial palsy caused by the Borrelia spirochete. 258 70

Between September 1986 and November 1988, 17 patients were hospitalized and treated for neuro-borreliosis. Ten of them had been admitted with suspected lumbar or cervical root or compression syndrome. Only four patients recalled a tick bite, only three an erythema migrans. Uni- or bilateral facial paresis was a prominent feature in six patients. Three of 14 patients had no IgG antibodies against Borrelia, either in serum or cerebrospinal fluid at the initial examination, two had positive titres in serum only. Despite antibiotic treatment (usually 10 mega U penicillin three times daily) six patients had a recurrence by April, 1989, treated with penicillin again or with twice daily 100 mg doxycycline or 2 g ceftriaxon. In four of them a residual painful polyneuropathy remains.
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PMID:[Neuro-borreliosis or intervertebral disk prolapse?]. 280 90

A 31 year-old inhabitant of French Guiana was prescribed mercuric iodide per os for two and a half months. Shortly before the end of the treatment he developed fasciculations in the trunk and particularly the lower limb muscles, distal painful paresthesias with vasomotor disorders, episodes of excessive perspiration and palmoplantar erythema, moderate fluctuating hypertension, progressive loss of weight and irritability with insomnia. Clinical and electrical signs of neuropathy were lacking. The clinical picture was that of Morvan's fibrillary chorea with acrodynia, the conditions of onset strongly suggesting a mercurial intoxication. Blood and particularly urine mercury levels were elevated. Administration of dimercaprol (BAL) considerably increased urinary excretion of mercury and there was progressive improvement and finally recovery after two months of BAL treatment. This case exemplifies the possible co-existence of fibrillary chorea and acrodynia. Whereas in many cases of fibrillary chorea a precise etiology cannot be determined, the affection can be induced by mercury as by gold administration. The fact that cases of fibrillary chorea due to mercury poisoning are rarely reported may be the result of individual patient hypersensitivity or particular metabolic absorption and excretion features of mercury. This case cannot be included within the continuous activity syndrome of muscle fibers described by Isaacs, since muscle contractures were absent and there was associated acrodynia. Moreover, there was no latent polyneuropathy, in spite of the intense fasciculations. It must be concluded, therefore, that in spite of its rarity fibrillary chorea should keep its semiologic autonomy.
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PMID:[Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment]. 652 13

POEMS syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, POEMS syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that POEMS syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.
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PMID:POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia. 1007 79

POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.
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PMID:POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. 1032 21

Lyme disease is a multisystem infection affecting all age groups. In this study an attempt was made to determine whether the patient's age influences the course of the disease. One hundred and fifty patients with diagnosed Lyme disease were included in the study. Two serological methods were used to detect antibodies to Borrelia burgdorferi and to confirm the diagnosis: an indirect immunofluorescence assay (the Russian strain Ip-21) and Western blot. The course of Lyme disease did not differ from that seen in Europe and North America. However, a few clinical differences between groups were observed. In the first age group (0-15 years) the most common manifestation was flu-like symptoms with fever. Neither radiculoneuritis nor polyneuropathy was observed in this age group. Late manifestations were rare and the outcome of the disease was benign. The course of the disease in the second group (16-40 years) was most similar to that in childhood and the also outcome was similar. Carditis and erythema multiple were significantly more common in the second group (16-40 years) than in the other age groups. No differences were found between the third (41-60 years) and fourth (> than 60 years) group in the frequency of flu-like symptoms, erythema multiple and aseptic meningitis. However, the most important clinical sign in this group was involvement of the nervous system whereas in the third group this was joint damage. This feature deserves attention because, as a rule, the presence of an articular syndrome determines the prognosis of LD.
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PMID:The course of Lyme disease in different age groups. 1066 13

We report a patient with leukocytoclastic-vasculitic neuropathy associated with chronic Epstein-Barr virus (EBV) infection. A 55-year-old man had been suffering from chronic progressive axonal polyneuropathy and skin erythema for 3 years. A skin biopsy showed capillary vasculitis with clustered leukocyte fragments. Findings of serum assays, a polymerase chain reaction for EBV-DNA, and in situ hybridization indicated chronic EBV infection. Immunosuppressive treatment resulted in the gradual lessening of his general and neurologic symptoms.
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PMID:Leukocytoclastic-vasculitic neuropathy associated with chronic Epstein-Barr virus infection. 1250 5

A 67-year-old woman was hospitalized with recurrent fever, arthralgia and erythema of the arms and legs. She had suffered from bronchiectasis for the previous 4 years, and Pseudomonas was persistently detected in her sputum. During the course of her illness, she developed distal sensitive and motor polyneuropathy. Serum test was positive for myeloperoxidase and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibodies. Nerve biopsy showed vasculitis infiltration of the vasa nervorum. She started immunoglobulin therapy, and after methylprednisolone and pulse cyclophosphamide therapy once monthly for 6 months she showed a good response and a reduction in symptoms.
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PMID:[Bronchiectasis worsening by p-ANCA (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature]. 1661 Feb 50

In people with diabetes mellitus, the Charcot foot is a specific manifestation of peripheral neuropathy that may involve autonomic neuropathy with high blood flow to the foot, leading to increased bone resorption. It may also involve peripheral somatic polyneuropathy with loss of protective sensation and high risk of unrecognized acute or chronic minor trauma. In both cases, there is excess local inflammatory response to foot injury, resulting in local osteoporosis. In the Charcot foot, the acute and chronic phases have been described. The former is characterized by local erythema, edema, and marked temperature elevation, while pain is not a prominent symptom. In the latter, signs of inflammation gradually recede and deformities may develop, increasing the risk of foot ulceration. The most common anatomical classification describes five patterns, according to the localization of bone and joint pathology. This review article aims to provide a brief overview of the diabetic Charcot foot in terms of etiology, pathophysiology, and classification.
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PMID:Etiology, pathophysiology and classifications of the diabetic Charcot foot. 2370 58

In recent years the number of disorders known to affect amino acid synthesis has grown rapidly. Nor is it just the number of disorders that has increased: the associated clinical phenotypes have also expanded spectacularly, primarily due to the advances of next generation sequencing diagnostics. In contrast to the "classical" inborn errors of metabolism in catabolic pathways, in which elevated levels of metabolites are easily detected in body fluids, synthesis defects present with low values of metabolites or, confusingly, even completely normal levels of amino acids. This makes the biochemical diagnosis of this relatively new group of metabolic diseases challenging. Defects in the synthesis pathways of serine metabolism, glutamine, proline and, recently, asparagine have all been reported. Although these amino acid synthesis defects are in unrelated metabolic pathways, they do share many clinical features. In children the central nervous system is primarily affected, giving rise to (congenital) microcephaly, early onset seizures and varying degrees of mental disability. The brain abnormalities are accompanied by skin disorders such as cutis laxa in defects of proline synthesis, collodion-like skin and ichthyosis in serine deficiency, and necrolytic erythema in glutamine deficiency. Hypomyelination with accompanying loss of brain volume and gyration defects can be observed on brain MRI in all synthesis disorders. In adults with defects in serine or proline synthesis, spastic paraplegia and several forms of polyneuropathy with or without intellectual disability appear to be the major symptoms in these late-presenting forms of amino acid disorders. This review provides a comprehensive overview of the disorders in amino acid synthesis.
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PMID:Amino acid synthesis deficiencies. 2865 76


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