1/202. Familial guillain-barre syndrome.There are few reports of guillain-barre syndrome (GBS) occurring in families. We have encountered a mother, who developed acute inflammatory demyelinating polyradiculoneuropathy at age 35 years, whose son developed the bulbar form of GBS 7 years later. Both shared HLA DR2.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/202. Neonatal guillain-barre syndrome: blocking antibodies transmitted from mother to child.OBJECTIVE: To investigate the role of blocking antibodies in neonatal guillain-barre syndrome (GBS) occurring 12 days postpartum in a child born to a mother with ongoing GBS. methods: We studied plasma filtrate, purified IgG, and monovalent Fab fragments from the affected mother and serum from the neonate as well as serum samples after recovery from disease 3 months later. Experiments were performed on the hemidiaphragms of adult mice and neonatal and juvenile rats. Quantal endplate currents were recorded with the perfused macro-patch clamp electrode. RESULTS: A dual effect was seen. serum from mother and infant depressed quantal content by approximately 90% and reduced the amplitude of postsynaptic currents by 30 to 40%. The antibody nature of the blockade could be confirmed by showing that monovalent Fab fragments were similarly effective as purified immunoglobulin (Ig) G. No IgG antibodies to gangliosides, fetal or adult nicotinic acetylcholine receptor, or voltage-gated calcium channels could be detected, but IgM antibodies to the ganglioside GM1 were present. After recovery from GBS no blocking activity was seen in the sera of mother and infant. To elucidate why neonatal disease onset was delayed we examined the possible influence of early developmental changes in functional properties of the neuromuscular junction and applied the mother's active serum to postnatal rats. Although blockade was present in 23-day-old rats, it was absent in 5-day-old rats. CONCLUSION: Transplacentally transferred blocking antibodies may be specifically directed at epitopes of the mature but not the fetal neuromuscular junction.- - - - - - - - - - ranking = 1.25keywords = syndrome (Clic here for more details about this article) |
3/202. Acute axonal polyneuropathy in chronic alcoholism and malnutrition.In contrast to the classic, slowly progressive polyneuropathy in alcoholic patients, acute forms, clinically mimicking guillain-barre syndrome, are rare. We present a patient who developed motor weakness and sensory loss in all four limbs within four days. Laboratory data were consistent with long-term alcohol abuse and documented thiamine deficiency. Repeated cerebrospinal fluid examinations were normal. Electrophysiological studies showed an acute sensorimotor polyneuropathy with predominantly axonal involvement. We conclude that acute alcoholic neuropathy has to be distinguished from guillain-barre syndrome and other forms of acute polyneuropathy by using clinical, laboratory, and electrophysiological data. Both ethanol toxicity and vitamin deficiency could play a role in the pathogenesis.- - - - - - - - - - ranking = 0.5keywords = syndrome (Clic here for more details about this article) |
4/202. guillain-barre syndrome: delayed diagnosis following anaesthesia.guillain-barre syndrome following anaesthesia or surgery is rare. Diagnosis is often delayed, which may lead to an increase in morbidity. There is now good evidence that early diagnosis and treatment reduces this morbidity. The two cases highlight the difficulties with diagnosis in the perioperative period and further discuss the aetiology, diagnostic features and complications of childhood guillain-barre syndrome.- - - - - - - - - - ranking = 1.5keywords = syndrome (Clic here for more details about this article) |
5/202. Benign acute childhood myositis: laboratory and clinical features.BACKGROUND: Benign acute myositis of childhood is a disorder of midchildhood, typically affecting boys. Symptoms include calf pain and difficulty walking after a viral illness. There is an epidemiologic association with influenza. OBJECTIVES: To describe the clinical and laboratory features of benign acute myositis. RESULTS: Thirty-eight children (32 boys, 6 girls) were seen with 41 episodes of myositis between 1978 and 1997. Two were siblings and three had recurrent episodes. Mean age at onset of symptoms was 8.1 years. Children remained ambulant during 33 of 41 episodes. Two characteristic gaits were noted: toe-walking in 13, with a wide-based stiff-legged gait in another 7. Muscle tenderness was isolated to the gastrocnemius-soleus muscles in 82% of episodes. Recovery occurred within 1 week. creatine kinase levels were elevated during all episodes. Viral studies were positive in 10 of 24 episodes, 5 because of influenza B. CONCLUSION: Benign acute myositis is a syndrome of midchildhood that can be differentiated from more serious causes of walking difficulty by the presence of calf tenderness, normal power, intact tendon reflexes, and elevated creatine kinase. The gait patterns noted may minimize power generation of the calf muscles by splinting the ankles. Onset in childhood may reflect an age-related response to viral infection, and occurrence primarily in boys may reflect a genetic predisposition or an as-yet unknown metabolic defect.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
6/202. Detection of antibodies against campylobacter jejuni serogroup PEN O:19 purified flagellar protein in a patient with guillain-barre syndrome.C. jejuni serogroup PEN O:19 was isolated from a stool specimen from a patient with guillain-barre syndrome (GBS). Flagellar protein was isolated and purified from reference strain C. jejuni PEN O:19, ATCC 43,446, as well as from a homologous patient strain. antibodies against flagellar protein were detected by means of immunoblotting, enzyme-linked immunosorbent assay (ELISA) and tube agglutination test. The antibody titres were found to be directly correlated at the beginning and in the recovery phase of GBS. antibodies of IgG and IgA classes were present from the very onset of the disease as well as 5 months later, but with a lower titre population. However, antibodies of the IgM class were persistent only at the onset of the infection and disappeared during the following 5 months. Our results strongly support the hypothesis that in GBS patients, antiflagellar antibodies are induced during C. jejuni infection and can be used in the diagnosis of C. jejuni-associated GBS.- - - - - - - - - - ranking = 1.25keywords = syndrome (Clic here for more details about this article) |
7/202. Ganglioside-induced antiganglioside antibodies from a neuropathy patient cross-react with lipopolysaccharides of campylobacter jejuni associated with guillain-barre syndrome.Antiganglioside serum antibodies from a patient treated with gangliosides were examined for cross-reactivity with lipopolysaccharides (LPSs) of campylobacter jejuni strains associated with guillain-barre syndrome (GBS). The patient had no preceding infection with C. jejuni and developed chronic progressive motor polyneuropathy following parenteral ganglioside treatment. serum IgG antibodies recognised GM1 and GD1b gangliosides as well as asialo-GM1, and cross-reactivity was observed with LPSs from C. jejuni O:2, O:4, O:19 and O:41. The results give a clear indication that gangliosides and LPSs from C. jejuni serotypes associated with GBS share common epitopes.- - - - - - - - - - ranking = 1.25keywords = syndrome (Clic here for more details about this article) |
8/202. Autonomic instability and hypertension resulting in subarachnoid haemorrhage in the guillain-barre syndrome.We report the case of a 47-year-old woman with guillain-barre syndrome who developed autonomic instability and hypertension and subsequently developed a subarachnoid haemorrhage. This was manifested clinically by a seizure which began focally and became generalised. Computer tomography demonstrated a localised haemorrhage in the left central sulcus. Control of the hypertension was achieved with intravenous labetolol. Autonomic instability and hypertension are frequently reported in guillain-barre syndrome. Subarachnoid haemorrhage is an uncommon but serious complication.- - - - - - - - - - ranking = 1.5keywords = syndrome (Clic here for more details about this article) |
9/202. An uncommon cause of lower limb weakness.We describe a case of a severely mentally disabled patient diagnosed as suffering from guillain-barre syndrome and treated with repeated plasma exchange. However, the abrupt onset of a cardiovascular collapse prompted a more in-depth diagnostic workup which demonstrated that the neurologic symptoms were likely to be ascribed to poisoning with heavy metals from a large number of ingested coins and other metallic items.- - - - - - - - - - ranking = 0.25keywords = syndrome (Clic here for more details about this article) |
10/202. Neurogenic stunned myocardium in guillain-barre syndrome.Neurogenic stunned myocardium (NSM), a syndrome of reversible left ventricular dysfunction best described after subarachnoid hemorrhage, has not been associated with peripheral neuropathy. We describe a woman with guillain-barre syndrome in whom a syndrome compatible with NSM developed in the setting of a physiologically documented increase in sympathetic cardiovascular tone. This case supports the presumed unifying role of excessive sympathetic nervous system activation in the pathogenesis of NSM.- - - - - - - - - - ranking = 1.75keywords = syndrome (Clic here for more details about this article) |
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