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1/506. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.

    We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area. ( info)

2/506. Monocular involuntary eyelid closure.

    OBJECTIVES: Monocular blepharospasm (MBLS) is precipitated by ipsilateral ocular pathology and becomes bilateral over time. Two patients are reported with monocular visual disturbance exhibiting monocular involuntary eyelid closure (MIEC), resembling MBLS. MATERIAL AND methods: A 31-year-old female with hyperthyroidism developed monocular polyopia with left eye vision on binocular fixation followed by ipsilateral visual extinction. Monocular vision was otherwise normal. A 57-year-old male with renal failure developed monocular blindness secondary to retinal hemorrhage. RESULTS: The first patient had anisocoria and proptosis. brain magnetic resonance (MR) was normal. The second patient had involuntary movements of the left eye. Head computerized tomography (CT) was normal. Both patients exhibited MIEC of the eye with visual impairment that could be overcome voluntarily, but reappeared upon distraction. Neither of the two developed BLS in long-term follow-up. CONCLUSION: MIEC represents a complication of ipsilateral ocular pathology in the absence of other involuntary movements, apraxia of eyelid opening, or blepharospasm (BLS). MIEC needs to be distinguished from MBLS, since not all MBLS becomes bilateral. ( info)

3/506. skin conductance responses in paediatric Harlequin syndrome.

    We report a novel and simple application of skin conductance response (SCR) testing for diagnosis of a new-onset iatrogenic Harlequin syndrome in an infant. Isolated ipsilateral facial pallor, complicated by thermally induced systemic sympathetic vasodilatation, and preferential lateral decubitus positioning, mimics harlequin colour change. Correct diagnosis as Harlequin syndrome with facial sympathetic interruption was demonstrated by diminution of SCR. ( info)

4/506. Ischaemic enterocolitis complicating idiopathic dysautonomia.

    A previously fit 23 year old adult male who presented with a sudden onset of profound autonomic neuropathy, for which no cause could be found, is described. The patient subsequently developed ischaemic enterocolitis that ultimately necessitated colectomy and subtotal enterectomy. Potential neural and humoral mechanisms are discussed. ( info)

5/506. Autonomic nerve tumour with skeinoid fibres: ultrastructure of skeinoid fibres examined by quick-freezing and deep-etching method.

    A case of gastrointestinal autonomic nerve tumour with skeinoid fibres (SFs) of the jejunum in a 79-year-old Japanese man, was examined by the quick-freezing and deep-etching (QF-DE) method. The tumour consisted of spindle cells with immunohistochemical reactions for vimentin, NSE and CD34. Electron microscopically, features of the neural cells of the myenteric plexus were observed. The QF-DE method demonstrated intercellular meshwork structures, consisting of thin filaments (7-15 nm), with granular deposits. Fully developed parts of the deposits formed nodular aggregates composed of irregularly surfaced thick fibrils (30-48 nm) with a tendency to linear arrangement (SFs). We detected many interconnecting thin filaments (ICTFs) between the SFs, which were pre-existing components in the meshwork, avoiding the granular deposits. The focal thickening formed by the connection between SFs and ICTFs revealed a periodicity typical of SFs (33-45 nm). We conclude that SFs are formed by decoration of the granular deposits along pre-existing intercellular meshwork structures. ( info)

6/506. autonomic dysreflexia presenting as a severe headache.

    hypertension, bradycardia, and severe headache have been associated with autonomic dysreflexia. autonomic dysreflexia affects those with spinal transection above the level of T6 after plastic changes of the afferent pathways. This restructuring in the presence of noxious stimuli below the level of the lesion leads to autonomic dysreflexia. The onset of the first episode of autonomic dysreflexia has been documented as soon as 30 days and as late as 13 years after the injury. This report presents a case study of a paraplegic man 8 years after injury with autonomic dysreflexia associated with a urinary tract infection. ( info)

7/506. I-123 MIBG cardiac imaging in diabetic neuropathy before and after epalrestat therapy.

    I-123 metaiodobenzylguanidine (MIBG) scintigraphy is a new method to evaluate cardiac sympathetic nerve disturbance in patients with diabetes mellitus. Epalrestat specifically inhibits aldose reductase and improves diabetic neuropathy. The authors report a case of improvement in cardiac sympathetic dysfunction using MIBG scintigraphy with epalrestat therapy. In this case, epalrestat effectively reversed diabetic neuropathy, and MIBG scintigraphy was useful to evaluate the effect of epalrestat. ( info)

8/506. The use of oral vasopressors in the management of autonomic dysfunction and orthostatic hypotension.

    OBJECTIVE: To describe a patient with hypotension secondary to autonomic dysfunction who was successfully treated with oral vasopressors. CASE SUMMARY: A 76-year-old African-American man with a history of cerebrovascular accident with right hemiparesis 30 years prior to admission was admitted from another hospital four days after a new posterior inferior cerebellar artery occlusion and poor distal flow as manifested by weakness and hypotension. This was treated with intravenous fluids and dopamine. The dopamine was weaned and changed to phenylephrine to maintain systolic blood pressure >80 mm Hg. fludrocortisone 0.3 mg orally once daily was initiated; pressure support garments were used for the management of orthostatic hypotension. ephedrine 25 mg po tid was added and titrated up to 50 mg p.o. tid. yohimbine 5.4 mg po every eight hours was added due to continued dependence on phenylephrine to maintain adequate blood pressure. yohimbine was titrated up to 10.8 mg p.o. tid in an unsuccessful effort to wean the patient from phenylephrine. fludrocortisone was decreased to 0.1 mg po tid and the phenylephrine was tapered off. The patient developed a pan-sensitive escherichia coli urinary tract infection that was treated with oral trimethoprim/sulfamethoxazole. Over the subsequent days, an 80% left subclavian stenosis was detected; yohimbine and pressure support garments were discontinued. Subsequently, oral ephedrine was tapered off over two days, and fludrocortisone was tapered to 0.1 mg p.o. bid. The patient was transferred in a stable normotensive condition to an inpatient rehabilitation unit. The fludrocortisone was later discontinued with no further hypotension or orthostatic symptoms. DISCUSSION: In this case, orthostatic hypotension associated with autonomic dysfunction was successfully managed with a combination of intravenous vasopressors and hydration, pressure support garments, oral mineralocorticoids, and oral vasopressors. Oral vasopressors and mineralocorticoids are effective treatment options in the management of the vasopressor-dependent patient. In our patient the adverse effects were tolerable. After continued therapy, the oral vasopressors were withdrawn without a return of orthostatic symptoms. CONCLUSIONS: Orthostatic hypotension due to autonomic dysfunction may be successfully managed with combination oral therapy after initial treatment with intravenous vasopressors as evidenced by the absence of orthostasis. ( info)

9/506. Acute dysautonomia following mumps.

    Pure acute or subacute dysautonomia is a rare entity. Its etiology is as yet unknown. However, majority of these cases have a preceding viral infection such as herpes simplex, infectious mononucleosis, rubella or coxsackie B. A unique patient in whom acute dysautonomia followed mumps is reported. ( info)

10/506. The 4A syndrome association with osteoporosis.

    4A syndrome is characterised by adrenocortical insufficiency, achalasia, alacrima, autonomic and other neurological abnormalities. We report an 18-year-old boy with 4 A syndrome and having all classical features of the disease including sensorimotor neuropathy. In addition, the patient had low aldosterone levels and signs of osteoporosis, which apparently developed without glucocorticoid replacement therapy. Although it is speculated that the lack of local growth factors, nutritional deficiency secondary to achalasia or receptor abnormalities regarding bone metabolism contribute to osteoporosis, its etiopathogenesis still needs to be clarified. ( info)
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