Cases reported "Polyneuropathies"

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1/7. hajdu-cheney syndrome with growth hormone deficiency and neuropathy.

    A hajdu-cheney syndrome is a very rare congenital dysplastic bone disease including acro-osteolysis, short stature, characteristic facies, osteopenia, abnormalities of spine, skull and long bones. A 9 year-old boy presented at our clinic with a chief complaint of short stature and frequent lower respiratory tract infections. He had typical physical and radiographic features of hajdu-cheney syndrome associated with growth hormone (GH) deficiency and peripheral motor neuropathy. To our knowledge, this is the first report describing GH deficiency and neuropathy in hajdu-cheney syndrome.
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2/7. Microvasculitic paraproteinaemic polyneuropathy and B-cell lymphoma.

    Microvasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material. A 2-stage pathogenic cascade is postulated and explored with a review of the relevant literature.
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3/7. Toxic effects of epidural analgesia with ropivacaine 0.2% in a diabetic patient.

    A 51-year-old ASA physical status II, non-insulin-dependent diabetic male patient manifested lower limb nerve injury after receiving postoperative epidural analgesia with ropivacaine 0.2%. The case is presented, including a discussion of the relation between local anesthetic toxicity and diabetic neuropathy.
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4/7. Functional outcome in patients with critical illness polyneuropathy.

    PURPOSE: To evaluate the functional outcome of intensive care patients with critical illness polyneuropathy (CIP), 6 and 12 months after the onset. methods: DESIGN: A prospective observational cohort study and a cross-sectional study. SETTING: University hospital in the netherlands. patients: Eight consecutive intensive care patients with CIP for the prospective study and eight patients diagnosed with CIP in the past 6 months for the cross-sectional study. MAIN OUTCOME MEASURES: Functional outcome regarding body functions and structure, activities, participation and perceived quality of life. RESULTS: Nine patients (56%) died within one year. Functional outcome, participation and subjective health status in survivors varied widely at 6 and 12 months. After 12 months, physical functioning was improved in all patients. However activities related to mobility outdoors, autonomy, participation and quality of life were restricted in most patients. CONCLUSIONS: The majority of survivors have persistent functional disabilities in activities, reduced quality of life and restrictions in autonomy and participation one year after the onset of CIP. Prolonged rehabilitation treatment is necessary for an increasing number of intensive care patients who develop CIP, in order to reduce handicaps and achieve optimal autonomy and social participation.
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5/7. A case of encephalomyelopolyneuropathy in vitamin B12 deficiency.

    A case of a 44-years-old patient with unusual clinical presentation of encephalomyelopolyneuropathy in vitamin B12 deficiency is presented. The disease manifested itself with gastrointestinal bleeding, which necessitated emergency hospitalisation in surgical clinic. Clinical examinations revealed atrophic gastritis, pernicious anemia, neurological and mental complications. The diagnosis was made according to the following criteria: physical examination--smooth tongue, atrophic gastritis, mild hepatosplenomegaly; laboratory findings--pernicious anemia, low vitamin B12 serum levels; neurological examination--syndrome of combined damage of the posterior and lateral columns of the spinal cord; magnetic resonance imaging--typical hyperintense areas on T2-weighted images in the posterior columns in the cervical regions of the spinal cord; transcranial magnetic stimulation--prolonged central motor conduction time of the motor evoked potentials bilaterally; psychological examination--cognitive decline. After treatment with vitamin B12 an improvement of the hematological findings, neurological deficit and cognitive impairments was found. CONCLUSION: Neurological complications could be an early manifestation of vitamin B12 deficiency. In diagnostic aspect similar complaints require examination of the serum levels of vitamin B12. The delay in diagnosis and inadequate therapy bear the risk of incomplete recovery of the neurological deficit. The current problem of "cognitive decline" necessitates routine examination of the serum levels of vitamin Bl2 in all patients with initial cognitive impairments and their prompt and approapriate treatment.
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6/7. Polyneuropathy from thiamin deficiency associated with thyrotoxicosis.

    Polyneuropathy from thiamin deficiency can occur in persons who consume a diet consisting mainly of polished rice with low protein and thiamin content in the setting of excessive physical activity or hypermetabolic states. The authors report here a 17-year-old fisherman who presented with a 3-month history of symptoms and signs consistent with polyneuropathy. There were also clinical features of thyrotoxicosis which was confirmed by thyroid function test. His dietary intake consisted mainly of polished rice and fish both of which contain a small amount of thiamin. This could not cope with his hypermetabolic condition from thyrotoxicosis resulting in thiamin deficiency with polyneuropathy.
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7/7. Acute cranial polyneuritis with vertigo after stapedectomy.

    Acute vertigo occurring 48 hours after stapedectomy is assumed to be related to inner ear trauma. Similarly, acute vertigo occurring weeks after stapedectomy could be related to a fistula of the oval window. No one has tested the hypothesis that some of these cases could represent concomitant cranial polyneuritis. We report the development after stapedectomy of five cases of acute cranial polyneuritis with vertigo diagnosed by physical examination of the cranial nerves. In another case we determined that vertigo occurring after stapedectomy was not related to concomitant cranial polyneuritis. Although all the patients were treated with corticosteroids, the vertigo resolved within 12 to 24 hours only in those whose vertigo we had ascribed to polyneuritis.
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