1/138. 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.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/138. 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.- - - - - - - - - - ranking = 0.00032870005349067keywords = lead (Clic here for more details about this article) |
3/138. 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.- - - - - - - - - - ranking = 0.0018095513921327keywords = neurologic (Clic here for more details about this article) |
4/138. panic disorder or temporal lobe epilepsy: A diagnostic problem in an adolescent girl.Similarities in the clinical presentation of panic disorder and temporal lobe epilepsy suggest that the two disorders are related and can lead to difficulties in a differential diagnosis. We describe the case of a young girl suffering from paroxysmal anxiety, derealization-depersonalization and autonomic symptoms, lasting from seconds to several minutes; these episodes were very frequent and disabling. The interictal EEGs and MRI were normal. After having diagnosed panic disorder based mainly on the duration of the attacks and the family history, a pharmacological treatment was started.- - - - - - - - - - ranking = 0.00032870005349067keywords = lead (Clic here for more details about this article) |
5/138. intestinal pseudo-obstruction and acute pandysautonomia associated with Epstein-Barr virus infection.We report the association of neurological and intestinal disorders with the reactivation of Epstein-Barr virus (EBV) in a child. This previously healthy 13-yr-old boy presented with pharyngitis and acute abdominal ileus. laparotomy excluded a mechanical obstruction. Postoperatively, he suffered from prolonged intestinal obstruction, pandysautonomia, and encephalomyelitis. Histological examination of the appendix and a rectal biopsy taken 3 months after the onset showed an absence of ganglion cells (appendix) and hypoganglionosis (rectum), with a mononucleate inflammatory infiltrate in close contact with the myenteric neural plexuses. EBV-PCR was positive in the blood and cerebrospinal fluid, and in situ hybridization with the Epstein-Barr virus encoded rna probe showed positive cells throughout the appendix wall including the myenteric area, in a mesenteric lymph node, and in the gastric biopsies. EBV spontaneous lymphocytic proliferation was noted in the blood. The serology for EBV showed previous infection but anti-early antigen antibodies were present. No immunodeficiency was found. Neurological and GI recovery occurred after 6 months of parenteral nutrition and bethanechol. The omnipresence of EBV associated with the neurointestinal symptoms suggest that the virus was the causal agent. This is the first documented case of acquired hypoganglionnosis due to EBV reactivation.- - - - - - - - - - ranking = 0.0018095513921327keywords = neurologic (Clic here for more details about this article) |
6/138. Undiagnosed central anticholinergic syndrome may lead to dangerous complications.This report describes two cases of central anticholinergic syndrome, the first after general anaesthesia and the other during a prolonged stay in the intensive care unit. The symptoms in both patients resolved soon after physostigmine administration. There was a delay in the diagnosis of central anticholinergic syndrome, which resulted in acute lung injury and unanticipated intensive care unit admission. It is suggested that in cases of abnormal mental recovery after anaesthesia or sedation, the diagnosis of central anticholinergic syndrome should be considered.- - - - - - - - - - ranking = 0.0013148002139627keywords = lead (Clic here for more details about this article) |
7/138. Baroreceptor denervation presenting as part of a vagal mononeuropathy.A 48-year-old woman presented with a history of progressive cough, dysphonia, dysphagia, and postural symptoms. Subsequent neurological investigations were consistent with a bilateral vagal mononeuropathy, and neurosarcoidosis was diagnosed after scalene node biopsy. Autonomic investigations including microneurography, neurohormones, and heart rate variability demonstrated arterial and cardiopulmonary baroreflex failure. In addition, parasympathetic control of heart rate was absent and consistent with a bilateral, nonselective lesion in the proximal vagus.- - - - - - - - - - ranking = 0.0018095513921327keywords = neurologic (Clic here for more details about this article) |
8/138. Familial mitochondrial intestinal pseudo-obstruction and neurogenic bladder.Intestinal dysmotility and neurogenic bladder have been described as part of two autosomal-recessive mitochondrial disorders assumed to be due to a defect in communication between the nuclear and mitochondrial genomes: myoneurogastrointestinal encephalopathy (MNGIE) and diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (wolfram syndrome). Partial cytochrome c oxidase deficiency has been described in both. We describe three Ashkenazi Jewish siblings with progressive intestinal dysmotility, neurogenic bladder, and autonomic manifestations but no central nervous system involvement. Cytochrome c oxidase deficiency was demonstrated in peripheral and multiple intestinal muscle biopsies. Mitochondrial dna analysis of an intestinal biopsy of patient 1 showed heteroplasmy consisting of a normal 16.5-kb band and an approximately 28-kb band, suggestive of a duplication. Mitochondrial dna analysis of a muscle biopsy of patient 2 showed multiple deletions, mainly 10- and 11-kb bands. We suggest that this unique combination of intestinal pseudo-obstruction and neurogenic bladder could comprise a new autosomal-recessive mitochondrial disorder.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/138. Idiopathic myenteric ganglionitis underlying intractable vomiting in a young adult.Inflammatory infiltration of intestinal myenteric plexuses (i.e. myenteric ganglionitis), along with severe intestinal motor abnormalities, may accompany paraneoplastic syndromes, neurological disorders and gastrointestinal infections, although rare cases can be idiopathic. In this report, we describe the case of a patient who presented with chronic intractable vomiting and weight loss associated with idiopathic myenteric ganglionitis mainly involving the stomach. Tissue analysis showed that the inflammatory infiltrate comprised T lymphocytes (CD4 and CD8 ), and peptide immunolabelling revealed a marked decrease of substance p/tachykinin immunoreactive staining in nerve fibres and myenteric neurones. Following systemic steroid therapy, the patient's symptoms dramatically improved, and after one year of follow-up his general condition remains satisfactory. The possible mechanisms leading to symptom generation and gastric dysmotility in the context of an idiopathic myenteric ganglionitis are discussed.- - - - - - - - - - ranking = 0.0021382514456234keywords = neurologic, lead (Clic here for more details about this article) |
10/138. pure autonomic failure in association with human alpha-synucleinopathy.We studied an autopsy case with pure autonomic failure, using anti-alpha-synuclein antibody. Until now there has been no report about the immunohistochemical properties of alpha-synuclein in pure autonomic failure. In conventional stainings, both pre- and post-ganglionic lesions of the sympathetic and parasympathetic nervous systems were found. lewy bodies and Lewy neurites were abundant especially in the sympathetic nervous system. These inclusions were immunoreactive to anti-alpha-synuclein antibody. The intensity of alpha-synuclein immunoreactivity was stronger in the halos than in the cores of the lewy bodies. The edges of the swollen neurites had strong immunoreactivity. The substantia nigra was well preserved, and no cortical lewy bodies were seen. These findings indicate that pure autonomic failure is one of the Lewy body type alpha-synucleinopathies, such as Parkinson's disease and dementia with lewy bodies, targeting the peripheral autonomic nervous system.- - - - - - - - - - ranking = 3keywords = nervous system (Clic here for more details about this article) |
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