1/33. 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) |
2/33. critical illness neuropathy in pediatric intensive care patients.critical illness neuropathy is an axonal polyneuropathy recognized more frequently in adult intensive care patients with sepsis and multiple organ dysfunction. In children the diagnosis is rarely made. Within 1 year the authors observed two children with critical illness neuropathy. Both patients, a male 6 years, 6 months of age with a brain contusion and a male 2 years, 6 months of age who underwent craniectomy for Crouzon's disease, required prolonged mechanical ventilation and developed sepsis with multiple organ dysfunction. Three to 4 weeks after successful treatment of the sepsis, a flaccid tetraparesis was noticed in both patients. Laboratory investigations of blood and cerebrospinal fluid and spinal magnetic resonance imaging revealed normal results. Electrophysiologic examinations were indicative of an axonal polyneuropathy. Spontaneous improvement occurred within several months. It is likely that critical illness neuropathy occurs more often in critically ill children than previously thought. Careful neurologic examination and early electrophysiologic investigations are necessary to establish the diagnosis. Important differential diagnoses of acquired lower motor neuron weakness in pediatric intensive care medicine are discussed.- - - - - - - - - - ranking = 0.081185123302369keywords = brain (Clic here for more details about this article) |
3/33. Reversible combined cognitive impairment and severe polyneuropathy resulting from primary hyperparathyroidism.Central and peripheral nervous systems may be involved in primary hyperparathyroidism (PHP). The efficacy of parathyroidectomy in reversing neurological symptoms is still a matter of controversy. We describe the case of a 71-year-old white male with a 10-year history of PHP who developed progressive cognitive dysfunction and severe sensorimotor axonal polyneuropathy. Successful parathyroidectomy reversed with a different temporal course both the central and peripheral nervous system involvements.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
4/33. carbon disulfide vasculopathy: a small vessel disease.We present the clinical manifestations of 4 male patients with acute stroke-like symptoms and polyneuropathy after long-term exposure to carbon disulfide (CS2) in a viscose rayon plant. The ages of onset of polyneuropathy ranged from 42 to 45 years with a duration of CS2 exposure between 6 and 21 years. The ages of onset of stroke were from 42 to 48 years. The risk factors for stroke including heart disease and diabetes were denied, except for smoking in 4, hyperlipidemia in 2 and hypertension in 1. At the initial visit in 1992, only 2 patients developed sudden onset of hemiparesis suggesting a lacunar stroke before the diagnosis of CS2 intoxication. Brain computed tomography (CT) scans showed low-density lesions in the basal ganglia in 2 patients, cortical atrophy in 1 and normal in 1. Brain magnetic resonance image (MRI) study disclosed multiple lesions in the corona radiata and basal ganglia on T(2)-weighted images in 3 patients and cortical atrophy in 1. After the diagnosis, they left their jobs for a CS2-free environment, and improvement of the working conditions was noted. During 5 years follow-up period, another 2 patients also developed an acute episode of stroke with hemiparesis. Brain CT and/or MRI follow-up studies in these 2 patients revealed new lesions in the basal ganglia and corona radiata. Intriguingly, a patient with previous stroke also developed new lesions in the bilateral thalami and brainstem. Carotid Doppler scan, transcranial Doppler scan and/or cerebral angiography did not show any prominent stenosis or occlusion in the major intracranial large arteries. We conclude that encephalopathy may occur in patients after long-term CS2 exposure, probably due to impaired cerebral perfusion. The lesions tend to occur in the basal ganglia, corona radiata and even brainstem, particularly involving the small-sized vessels. In addition, the cerebral lesions may progress even after cessation of CS2 exposure. Therefore, we suggest that CS2 exposure may be a risk factor for stroke.- - - - - - - - - - ranking = 0.16237024660474keywords = brain (Clic here for more details about this article) |
5/33. Severe axonal polyneuropathy after a FK506 overdosage in a lung transplant recipient.FK506-induced polyneuropathies are rarely encountered. We report a case of axonal sensorimotor polyneuropathy in a lung transplant recipient that occurred during a FK506 overdosage. Onset was acute in the form of severe areflexic tetraparesis and resolution was observed after reduction of dosage. Because of increasing use of FK506 in solid organ transplantation, caution should be paid with FK506 dosage monitoring in cases of peripheral nervous system symptoms.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
6/33. Chronic demyelinating polyneuropathy in graft-versus-host disease following allogeneic bone marrow transplantation.In recent years a novel problem has arisen in organ transplantation medicine, namely GVHD. The nervous system has been involved mainly at the level of the CNS and this can lead to a serious outcome for the patient. In rare cases, peripheral nerves may be affected and show acute or chronic polyneuropathy. Here a case is reported of polyneuropathy associated with chronic GVHD. A 32-year-old man, suffering from chronic GVHD following an allogeneic bone marrow transplantation (BMT) for malignant lymphoma at the age of 25, developed a motor dominant polyneuropathy 5 years later. Electrophysiologic studies demonstrated the demyelinating type of polyneuropathy. biopsy specimens from skin and skeletal muscle disclosed perivascular lymphocytic infiltrates expressing T-cell markers. The sural nerve showed a loss of myelinated nerve fibers with epineurial fibrosis and rare occurrence of T cells, but without obvious vasculitic changes. The present case suggested that polyneuropathy could develop in association with chronic GVHD in some patients with a long-standing disease course.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/33. The relationship between isofenphos cholinergic toxicity and the development of polyneuropathy in hens and humans.Species differences have been observed between hen and human clinical manifestations of isofenphos toxicities. Hens treated with the insecticide isofenphos (90 mg/kg p.o.) developed severe cholinergic toxicity followed by mild organophosphate-induced delayed polyneuropathy (OPIDP). However, a patient developed severe OPIDP, which was preceded by very mild cholinergic signs, after an attempted suicide with a commercial formulation containing isofenphos and phoxim, an insecticide not causing OPIDP (estimated doses were 500 and 125 mg/kg, respectively). To explain this difference the following hypotheses were tested: (1) phoxim is a promoter of isofenphos-induced OPIDP; (2) whereas neuropathy target esterase (NTE) is thought to be the target of OPIDP, activation of isofenphos by liver microsomes causes the formation of more potent NTE inhibitor(s) in humans than in hens; (3) in contrast to hen NTE, the sensitivity of the human enzyme to such inhibitor(s) is higher than that of acetylcholinesterase (AChE), the target of cholinergic toxicity. Results showed that phoxim (22.5 mg/kg p.o.) was not a promoter of OPIDP in hens and that the ratio AChE inhibition:NTE inhibition by microsome-activated isofenphos was similar for both hen and human enzymes. The schedule of antidotal treatment in hens is the likely explanation for the observed difference from the patient. Peak AChE inhibition was maintained in hen brain up to 6 days after a single dose of isofenphos, suggesting prolonged pharmacokinetics. However, the AChE reactivator pyridine-2-aldoxime (2-PAM) was given to hens before isofenphos and then every 8 h, whereas continuous 2-PAM infusion was provided to the patient. When 2-PAM was given to hens every hour after isofenphos (90 mg/kg p.o.), the birds remained asymptomatic. Since other organophosphates may have a prolonged pharmacokinetics, testing procedures for the potential of these insecticides to cause OPIDP may underestimate the risk for humans.- - - - - - - - - - ranking = 0.081185123302369keywords = brain (Clic here for more details about this article) |
8/33. Organophosphate-induced delayed neuropathy: case report.Organophosphate induced delayed neuropathy (OPIDN) is an uncommon clinical condition. It occurs in association with the ingestion of great amounts of organophosphate after the stimulation of cholinergic receptor. The clinical picture is characterized by a distal paresis in lower limbs associated with sensitive symptoms. Electrodiagnostic studies show a motor axonal neuropathy. Involvement of the central nervous system may occur. We describe a 39 years-old female patient who developed hyperesthesia associated with lower limbs paresis, fourteen days after she had ingested a dichlorvos-based insecticide. Electrophysiological study was characterized by an axonal polyneuropathy pattern. Pyramidal tract dysfunction was observed later in upper limbs. Considering that both peripheral and central nervous systems are involved we believe that the more appropriated term would be organophosphate induced delayed neuropathy (OPIDN) instead of organophosphate induced delayed polyneuropathy (OPIDP).- - - - - - - - - - ranking = 3.4565515195686keywords = nervous system, central nervous system (Clic here for more details about this article) |
9/33. rehabilitation of a patient with critical illness polyneuropathy (CIP) following acute respiratory failure: a case report and review of literature.critical illness polyneuropathy (CIP), a neurologic complication that may occur secondary to cardio-respiratory distress, surgery, trauma and coma, is associated with sepsis or multiple organ failure. CIP is characterized by an axonal distal degeneration of sensory and motor fibres. The patients will often become neurologically conspicuous when weaning from mechanical ventilation is unexpectedly difficult. There are just a few cases reported with description of the functional outcome and rehabilitation issues of this condition. An additional CIP case of a 62-year old man complicated with anoxic brain damage during the respiratory distress is reported here. He was referred for rehabilitation, made a remarkable recovery (FIM gain 45!) and returned home after 79 days of treatment in the ward. A review of the pertinent literature is provided. rehabilitation specialists and other professionals working within ICU's should be aware of this condition and be able to recognize and treat CIP at early possible stage.- - - - - - - - - - ranking = 0.081185123302369keywords = brain (Clic here for more details about this article) |
10/33. Simulation of brain death from fulminant de-efferentation.BACKGROUND: guillain-barre syndrome (GBS) classically presents with a subacutely evolving areflexic paralysis, with typical laboratory findings of elevated cerebrospinal fluid protein and abnormal nerve conduction studies. There is now an increasing recognition of GBS variants that differ in clinical presentation, prognosis, electrophysiology and presumed pathogenesis. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling "brain death". methods: A retrospective analysis of two such cases of fulminant neuropathy are described, that includes the clinical course, electrophysiology and neuropathology where available. RESULTS: We describe two patients that presented with a rapid course of neurological deterioration, lapsing into what resembled a "clinically brain-dead" state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy. CONCLUSIONS: A fulminant neuropathy can result in a clinical state resembling "brain death" through diffuse de-efferentation. Although generally attributed to aggressive demyelination with secondary axonal degeneration, a primary axonopathy can also lead to a similar clinical presentation.- - - - - - - - - - ranking = 0.56829586311658keywords = brain (Clic here for more details about this article) |
| | Next -> |