1/46. Event-related potentials in patients with total locked-in state due to fulminant guillain-barre syndrome.A series of electrophysiological investigations were performed over a 6-month period in two patients affected by fulminant Guillain-Barre polyradiculoneuropathy, who developed an ascending paralysis leading, within 72 h, to flaccid quadriplegia, internal and external ophthalmoplegia, absence of all brainstem reflexes and no respiratory effort: the clinical state resembled brain death. brain CTs were normal and spinal fluid examination revealed albuminocytological dissociation. All motor nerves tested were unexcitable, whereas sensory responses were markedly abnormal but present. Sequential EEG recordings revealed normal, partially reactive alpha rhythm in both patients. In one patient, normal auditory event-related potentials (ERPs: peak N1, P2, N2, P3, evoked in an 'oddball' paradigm) and CNV-like potentials could be recorded not earlier than the 20th day into the illness. In earlier recordings, N1 and P2 peaks as well as mismatch negativity (MMN) were present over the frontal and central scalp electrodes. This patient has now partially recovered motor functions and no cognitive defects are present, but he has little recollection of the events occurring in the first 2 weeks spent in the ICU, when he was completely paralyzed. The other patient generated normal N1 and P2 ERP peaks, but no N2, P3 and MMN were detected in a series of recordings. He died without having ever regained appropriate behavioral responses. The ERP abnormalities observed raise the matter of the origin of cognitive dysfunction in patients with severe and prolonged de-efferentation/de-afferentation. ERPs allow monitoring the level of alertness and attention and appear more specific than EEG in identifying a state of awareness in patients in which communication is severely impaired as a consequence of neurological disorders.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
2/46. guillain-barre syndrome presenting with severe pain: report of one case.Most neurologists are familiar with pain in guillain-barre syndrome but some pediatricians may still miss or underestimate it. We report a 10-year-old girl who presented with headache and numbness in lower extremities. On examination, she could walk and showed hyporeflexia, mild muscle weakness, prominent neck rigidity and right facial nerve palsy. headache and pain in neck, low back and calves were so severe that she could not fall asleep and the response to analgesic was very poor. On 8th day of illness, she needed mechanical ventilation and suffered tetraplegia. Symptoms of autonomic dysfunction including hypertension and sinus tachycardia appeared thereafter and lasted for a week. After receiving a high dose of intravenous immunoglobulin (2 g/kg in 2 days), her condition improved gradually although joint pain persisted for about three weeks. She could walk unaided by day 40 and run by day 70, and recovered completely 6 months later. We emphasize that pain in GBS needs proper evaluation and management in children as well as in adults.- - - - - - - - - - ranking = 0.31730172975661keywords = palsy (Clic here for more details about this article) |
3/46. guillain-barre syndrome: perspectives with infants and children.An acute flaccid paraparesis or ascending quadriparesis in an infant or child constitutes a very important pediatric neurology emergency. The guillain-barre syndrome (GBS) is the most frequent cause. This is primarily an autoimmune, post-infectious, demyelinating, peripheral nervous system process. A small percentage of children develop a primary axonal process not unlike that identified more commonly in china. Because of the potential for acute respiratory compromise, any child suspected of having GBS needs immediate hospitalization. The major considerations in differential diagnosis include transverse myelitis, toxic neuropathies, tick paralysis, infantile botulism, myasthenia gravis, and dermatomyositis. On occasion, some younger children present with an acute severe pain syndrome that may mask as a pseudo-encephalopathy. Another clinical variant is the Miller-Fisher syndrome characterized by ataxia, ophthalmoparesis, and areflexia. This is associated with a high frequency of the anti-GQ-1-b antibodies. Although most children with GBS have a relatively benign clinical course, some become very ill and require intubation with intensive care monitoring. Immunomodulating treatment should be used for any child who loses the ability to walk. To date, no well-controlled study has been completed analyzing the relative merits of the two most commonly used therapies, namely plasmapheresis or intravenously administered immunoglobulin.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
4/46. Perineurium contributes to axonal damage in acute inflammatory demyelinating polyneuropathy.OBJECTIVE: To assess if axonal damage in severe acute inflammatory demyelinating polyneuropathy (AIDP) correlates with the appearance of epiperineurium in nerve trunks. BACKGROUND: Increase of endoneurial fluid pressure in nerve trunks possessing epiperineurium may be an important mechanism of axonal damage in AIDP. methods: A 79-year-old man had a 2-day history of acroparesthesias and ascending paralysis culminating in quadriplegia, bilateral facial palsy, and mechanical ventilation. Five intravenous immunoglobulin cycles were given without response. He died on day 60. Electrophysiologic studies (days 4, 17, and 50) initially showed normal nerve conduction velocities with further slowing, progressive attenuation of compound muscle action potentials, and profuse denervation. The authors studied the preforaminal anterior and posterior L3 and L5 spinal roots, third and fifth lumbar nerves and their branches, and femoral and sural nerves. RESULTS: Density of myelinated fibers was preserved in L5 ventral and dorsal roots and reduced in sural nerve. Mild de-remyelination was observed in lumbar roots. In both lumbar nerves and their branches, there were extensive de-remyelination and centrofascicular or wedge-shaped areas with marked loss of large myelinated fibers. Axonal degeneration was the predominant lesion in sural nerve. CONCLUSION: The presence of epiperineurium correlates with a drastic change of pathology with superimposed ischemic lesions and distally accentuated axonal loss, suggesting that endoneurial fluid pressure increase could cause axonal damage in AIDP.- - - - - - - - - - ranking = 1.3173017297566keywords = paralysis, palsy (Clic here for more details about this article) |
5/46. Sensory guillain-barre syndrome.OBJECTIVE: To report eight cases of sensory guillain-barre syndrome (GBS). BACKGROUND: The concept of sensory equivalent to ascending paralysis of GBS was raised in 1958, and the diagnostic criteria for a sensory loss and areflexia variant of GBS were proposed in 1981. However, clinical cases meeting these criteria have been relatively scarce. methods: During a 13-year period between 1986 and 1999, the authors collected eight cases of an acute sensory demyelinating neuropathy that met most of the proposed diagnostic criteria of a sensory variant of GBS. RESULTS: In all patients, sensory neuropathy was sudden at onset and peaked to maximal deficit within 4 weeks. In five (63%) cases, there was an antecedent viral illness. All patients had objective sensory loss and diminished or absent reflexes. None showed any muscle weakness. In all four patients in whom the spinal fluid was examined during the first 4 weeks, there was albuminocytologic dissociation. All of the patients had electrophysiologic evidence of demyelination in at least two nerves. Demyelination was demonstrated in motor nerve conduction in seven patients and in sensory nerve conduction in one, indicating that motor nerve conduction studies were the key for the diagnosis of demyelinating neuropathy. All patients had sensory nerve conduction abnormalities in at least one nerve. Three patients responded to immunotherapies. All had a favorable outcome, with a monophasic course of disease and no sign of relapse. CONCLUSION: The current study confirms the existence of sensory GBS.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
6/46. Angiotensin converting enzyme inhibitor induced hyperkalaemic paralysis.Secondary hyperkalaemic paralysis is a rare condition often mimicking the guillain-barre syndrome. There have been a few case reports of hyperkalaemia caused by renal failure, trauma, and drugs where the presentation has been with muscle weakness. A case of hyperkalaemic paralysis caused by an angiotensin converting enzyme inhibitor is reported.- - - - - - - - - - ranking = 6keywords = paralysis (Clic here for more details about this article) |
7/46. Rapidly progressive polymyositis with elevated antiacetylcholine receptor antibody activity.We report a 51-year-old woman with polymyositis accompanied by a high titer of antiacetylcholine receptor antibody. The patient presented with weakness of grip strength followed by rapidly progressive dyspnea, which required mechanical ventilation. She was treated with a glucocorticoid and came off the respirator one week later. Antiacetylcholine receptor antibody activity was elevated in the acute phase and decreased during recovery, although other signs of myasthenia gravis were negative. This patient suggested that in cases of rapidly progressive bulbar palsy and limb muscle weakness, it is necessary to include polymyositis associated with elevated antiacetylcholine receptor antibody activity in the differential diagnosis.- - - - - - - - - - ranking = 444.31259498267keywords = bulbar palsy, palsy (Clic here for more details about this article) |
8/46. guillain-barre syndrome in a child with acute lymphoblastic leukemia.A 4-year-old boy with acute lymphoblastic leukemia receiving maintenance treatment developed quadriparesis, facial palsy, difficulty in swallowing, and hypertension following a respiratory infection and candida septicemia. Examination of the cerebrospinal fluid was normal initially but later showed albuminocytologic dissociation, the characteristic finding of guillain-barre syndrome. Complete recovery occurred after treatment with intravenous immunoglobulin. Differential diagnosis of guillain-barre syndrome from vincristine toxicity in patients with leukemia and possible association with the infections are discussed.- - - - - - - - - - ranking = 0.31730172975661keywords = palsy (Clic here for more details about this article) |
9/46. barium carbonate poisoning mimicking guillain-barre syndrome.Areflexic quadriplegia due to barium carbonate (rat poison) poisoning is described in two young patients. These cases very closely resembled guillain-barre syndrome. The various effects of barium carbonate along with the pathogenesis of hypokalaemic paralysis are highlighted.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
10/46. Two cases of influenza with impaired ocular movement.Complications of influenza include respiratory disorders (pneumonia, bronchitis and croup) and occasionally myocarditis, myositis, encephalitis, encephalopathy and Reye's syndrome, which may be life-threatening and cause various sequelae. We report two patients who developed unusual complications of influenza infection: one had ptosis and impaired ocular movement, and the other suffered from guillain-barre syndrome with paralysis of the extraocular muscles.- - - - - - - - - - ranking = 1keywords = paralysis (Clic here for more details about this article) |
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