1/13. Acute mercury vapour poisoning in a shipyard worker--a case report.Acute mercury vapour poisoning is a serious, potentially fatal but fortunately rarely encountered problem. It is most commonly due to industrial accidents. The vapour is a direct respiratory tract irritant as well as a cell poison, exerting its greatest effects in the lungs, nervous system, kidneys and liver. We present a case of mercury vapour poisoning in a shipyard workers presenting as an acute chemical pneumonitis, which resolved with aggressive supportive therapy. Further investigations later revealed transient mild neuropsychiatric symptoms, and residual peripheral neuropathy. No chelation therapy was instituted. The detailed investigative work that led to the discovery of the source of mercury is also presented. This case alerts us to the potential hazard to shipyard workers who may work in ships previously carrying oil contaminated with mercury. There have been no previous reports of mercury poisoning in shipyard workers. A high index of suspicion leading to early diagnosis and institution of appropriate supportive measures in suspected cases can be life-saving.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/13. Morphometric evaluation of paraneoplastic neuropathies associated with carcinomas, lymphomas, and dysproteinemias.Paraneoplastic peripheral neuropathies are caused by indirect effects of carcinomas, mainly small cell bronchogenic carcinomas, lymphoproliferative disorders (lymphomas, myelomas, polycythemia vera), and dysproteinemias (benign monoclonal paraproteinemia, Waldenstrom's macroglobulinemia) including cryoglobulinemias. Those associated with carcinomas are usually considered as severe, those associated with benign gammopathies (monoclonal gammopathies of unknown significance, MGUS) as mild, and those with cryoglobulinemias as of variable severity. In a larger series of 104 autopsy and biopsy cases, we noted a wide range of severity concerning various morphometric parameters of peripheral nerve fibers by evaluating sural nerves. There were no apparent morphometric differences between the groups of disorders. The most valuable parameter of optic-electronic evaluation and comparison turned out to be the myelin area expressed as a percentage of the endoneurial area because this measure comprises the relative number, size, and myelin thickness of the myelinated nerve fibers. In the 104 cases of the three disease groups, most of the cases (38 cases; 36.5%) showed a moderate reduction of the myelin sheath area per endoneurial area of sural nerves. This was followed by 34 cases (32.7%) with severe and very severe reduction. Twenty-nine cases (27.9%) presented with mild reduction. It is concluded that the severity of the neuropathy depends largely on the stage of the disease and the time of progression rather than on the type of the underlying disorder.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/13. adult-onset hereditary sensory and autonomic neuropathy accompanied by anosmia but without skin ulceration.We report a novel type of hereditary sensory and autonomic neuropathy (HSAN) with adult onset in a Japanese family. One male and 2 females of 6 siblings were affected. They developed anosmia initially at the ages of 20-50 years, followed by anhidrosis and sensory loss. skin ulceration was absent. Both superficial and deep sensation were impaired in the most distal parts of all 4 limbs. Orthostatic hypotension was present in all patients. This is a unique subtype of HSAN distinct from the HSAN I-V described by Dyck.- - - - - - - - - - ranking = 263.59694105238keywords = family (Clic here for more details about this article) |
4/13. Monocytoid B cell lymphoma associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside.Monocytoid B cell lymphoma (MBCL) is an immunologically and morphologically well-defined low-grade lymphoma with a predilection for lymph nodes of the parotid region. We describe an association of MBCL with anti-myelin-associated glycoprotein (MAG) polyneuropathy in a 53-year-old male. The diagnosis of stage IV MBCL with nodular bone marrow infiltration, sjogren's syndrome and sensorimotor polyneuropathy was made in October 1996. serum immunoelectrophoresis demonstrated IgMkappa paraprotein. This was then cross-reacted with epitopes of MAG and sulphated glucuronyl paragloboside (SGPG) on myelin sheaths, and detected by thin layer chromatography and Western blot. Direct immunofluorescence of a sural nerve biopsy showed loss of myelin fibres, segmental demyelinization and IgM deposits on the myelin sheaths. The cerebrospinal fluid was normal. After six cycles of chemotherapy (ChlVPP protocol), all the patient's haematological parameters normalized accompanied by an improvement in neurological signs. The improvement of the polyneuropathy after chemotherapy indicates that the autoimmune anti-MAG and anti-SGPG antibodies resulted from the neoplastic lymphoid proliferation.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/13. Sensory defensiveness: a theory of its effect on breastfeeding.This article describes one type of sensory integrative disorder: sensory defensiveness. An examination of the possible relationship of this disorder to an infant's difficulties with breastfeeding is conducted. A family case study is detailed, followed by treatments implemented by the authors. Possible long-term sequelae, as well as the need for more interdisciplinary research, are discussed.- - - - - - - - - - ranking = 263.59694105238keywords = family (Clic here for more details about this article) |
6/13. multiple sclerosis and oral care.multiple sclerosis is a complex neurological condition affecting sensory and motor nerve transmission. Its progression and symptoms are unpredictable and vary from person to person as well as over time. Common early symptoms include visual disturbances, facial pain or trigeminal neuralgia and paraesthesia or numbness of feet, legs, hands and arms. These, plus symptoms of spasticity, spasms, tremor, fatigue, depression and progressive disability, impact on the individual's ability to maintain oral health, cope with dental treatment and access dental services. Also, many of the medications used in the symptomatic management of the condition have the potential to cause dry mouth and associated oral disease. There is no cure for multiple sclerosis, and treatment focuses on prevention of disability and maintenance of quality of life. Increasingly a multi-disciplinary team approach is used where the individual, if appropriate his/her carer, and the specialist nurse are key figures. The dental team plays an essential role in ensuring that oral health impacts positively on general health.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/13. Autosomal recessive spinocerebellar ataxia and peripheral neuropathy with raised alpha-fetoprotein.We describe three patients from two families with progressive spinocerebellar ataxia, peripheral neuropathy, raised alpha-fetoprotein (AFP) and hypercholesterolaemia. Two siblings had identical clinical features, with late childhood onset of symptoms and slow progression, requiring crutches to walk at ages 37 and 38 years. Another patient developed ataxia aged 13 years and became wheel-chair bound by 20 years of age. Although they all had raised serum AFP levels, their clinical, immunological, biochemical, cytogenetic and molecular genetic studies failed to support a diagnosis of ataxia telangiectasia. Extensive investigation including imaging, biochemical and genetic studies excluded other known ataxias. Their clinical features most closely resemble the phenotype of a single consanguineous Japanese family with four individuals affected by spinocerebellar ataxia, peripheral neuropathy, raised AFP and hypercholesterolaemia. Homozygosity mapping has identified a locus in this Japanese family at 9q34. Haplotype analysis of our cases demonstrated possible linkage to 9q34, suggesting these may be the first Caucasian families described with this disorder.- - - - - - - - - - ranking = 527.19388210475keywords = family (Clic here for more details about this article) |
8/13. Primary AL (kappa-light chain) amyloidosis manifesting as peripheral neuropathy in a young male without increase in serum and urine immunoglobulin load: a diagnostic challenge.Primary systemic or AL amyloidosis is a multisystem disorder characterized by diffuse extracellular infiltration of a fibrillar protein of monoclonal light chain origin (AL). Majority of the patients have monoclonal immunoglobulin in serum and/or urine and some have clonal proliferation of plasma cells in their bone marrow. This disease has the widest spectrum of organ involvement, most commonly affecting the kidneys, heart and liver. Involvement of peripheral nervous system is not infrequent and may be the presenting feature of the disease process. Thus, recognition of peripheral neuropathy and affecting the kidney as an early symptom of AL amyloidosis may widen the scope for therapeutic intervention. We describe here a rare case of primary amyloidosis (AL) kappa-light chain presenting with clinical features of peripheral neuropathy and affecting the kidney and heart at an early age of 18 years, hitherto unreported in literature. The case was further interesting as it was not associated with increased serum/urine immunoglobulins or plasma cells in bone marrow. Diagnosis was confirmed using immuno-electron microscopy on sural nerve biopsy.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
9/13. Histoid leprosy - unusual presentation.A 39-year-old man presented with a chronic nonhealing toe ulcer and multiple skin-colored papules on the back, arms, and knees. From an initial small erosion, the toe lesion ulcerated over a 1.5-year period, while the papules progressed over a 6-month period, first appearing on the back and then spreading to the arms and knees. The past medical and family history were non-contributory. Pertinent findings included the aforementioned well-defined, asymmetric, shiny papules (Fig. 1). There were multiple, discrete, erythematous, pedunculated, tumor-like masses of various sizes over the right thigh (Fig. 2) and a well-defined 4 cm x 3 cm ulcer on the second toe of the left foot, the floor of which was covered by necrotic slough. There was distal loss of sensation to temperature, touch, and pain. The greater auricular, ulnar, radial cutaneous and common peroneal nerves were thickened bilaterally, but non-tender. A clinical diagnosis of histoid leprosy was made. The differential diagnosis for the tumor-like thigh masses included dermatofibroma, neurofibroma, and Kaposi's sarcoma. The hemogram, liver/renal function tests, chest X-ray, and abdominal ultrasound were normal. Human immunodeficiency virus enzyme-linked immunosorbent assay (hiv ELISA) was negative. The ear lobe smear (ELS) for acid-fast bacilli showed a bacterial index (BI) of 6 [> 1000 organisms/oil immersion field (oif)] and a morphological index (MI) of 50%. The skin-colored papules on the back and the pedunculated masses showed a BI of 5 (100-1,000 organisms/oif) and an MI of 50%. The normal skin showed a BI of 4 (10-100 organisms/oif) and an MI of 5%. biopsy of the papules and pedunculated tumors showed a well-circumscribed area of the dermis packed with many acid-fast organisms and foamy macrophages, consistent with histoid leprosy (Fig. 3). Fite-Faraco stain demonstrated cells packed with lepra bacilli. A final diagnosis of lepromatous leprosy, histoid variant, was made. dapsone, clofazimine, rifampicin, and, later, ofloxacin were started.- - - - - - - - - - ranking = 263.59694105238keywords = family (Clic here for more details about this article) |
10/13. motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction.We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian systems; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.- - - - - - - - - - ranking = 263.59694105238keywords = family (Clic here for more details about this article) |
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