The Experts below are selected from a list of 549 Experts worldwide ranked by ideXlab platform
J Wilson - One of the best experts on this subject based on the ideXlab platform.
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progressive Bulbar Paralysis of childhood a reappraisal of fazio londe disease
Brain, 1992Co-Authors: M A Mcshane, S Boyd, B Harding, E M Brett, J WilsonAbstract:Fazio-Londe disease is a label sometimes applied to a degenerative disease of the motor neurons characterized by progressive Bulbar Paralysis in children. It is very rare with only 22 case reports describing 24 children including four sibling pairs. In two reports mothers and sons were affected. The neuropathology is described in only four cases. Previous authors have recognized that the condition is very heterogeneous. The clinical features of five children with this type of progressive Bulbar Paralysis, diagnosed at this hospital between 1969 and 1989, are reviewed, and in two cases neuropathological findings are detailed. Based on this experience, suggested criteria for diagnosis include clinical features of a pure motor neuronopathy affecting the Bulbar nuclei, exclusion of other causes of progressive Bulbar Paralysis and positive support for the diagnosis from electromyography and/or pathological examination. A review of the literature, combined with the present series, suggests that there are at least three distinct subtypes: a very rare autosomal dominant form (as described by Fazio) and two variants with probable autosomal recessive inheritance either with early onset of respiratory symptoms and rapid progression to death or later onset, less prominent respiratory symptoms and protracted clinical course. There is strong concordance for each clinical pattern within families.
Hiroshi Asakura - One of the best experts on this subject based on the ideXlab platform.
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a childhood onset intestinal toxemia botulism during chemotherapy for relapsed acute leukemia
Annals of Clinical Microbiology and Antimicrobials, 2017Co-Authors: Noriko Ohyama, Michiko Torio, Kentaro Nakashima, Yuuki Koga, Shunsuke Kanno, Hisanori Nishio, Kei Nishiyama, Momoko Sasazuki, Haru Kato, Hiroshi AsakuraAbstract:Botulism is a potentially fatal infection characterized by progressive muscle weakness, Bulbar Paralysis, constipation and other autonomic dysfunctions. A recent report suggested that cancer chemotherapy might increase the risk for the intestinal toxemia botulism in both adults and children. We report a 5-year-old boy, who developed general muscle weakness, constipation, ptosis and mydriasis during the third induction therapy for relapsed acute myeloid leukemia. He had recent histories of multiple antibiotic therapy for bacteremia and intake of well water at home. Repeated bacterial cultures identified Clostridium botulinum producing botulinum neurotoxin A. Botulinum toxin A was isolated from his stools at 17, 21, and 23 days after the onset. Symptoms were self-limiting, and were fully recovered without anti-botulinum toxin globulin therapy. This is the second report of a pediatric case with cancer chemotherapy-associated intestinal toxemia botulism. Our case provides further evidence that the immunocompromised status due to anti-cancer treatments increases the risk for the development of botulism at all ages in childhood.
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A childhood-onset intestinal toxemia botulism during chemotherapy for relapsed acute leukemia
'Springer Science and Business Media LLC', 2017Co-Authors: Noriko Ohyama, Michiko Torio, Kentaro Nakashima, Yuuki Koga, Shunsuke Kanno, Hisanori Nishio, Kei Nishiyama, Momoko Sasazuki, Haru Kato, Hiroshi AsakuraAbstract:Abstract Background Botulism is a potentially fatal infection characterized by progressive muscle weakness, Bulbar Paralysis, constipation and other autonomic dysfunctions. A recent report suggested that cancer chemotherapy might increase the risk for the intestinal toxemia botulism in both adults and children. Case presentation We report a 5-year-old boy, who developed general muscle weakness, constipation, ptosis and mydriasis during the third induction therapy for relapsed acute myeloid leukemia. He had recent histories of multiple antibiotic therapy for bacteremia and intake of well water at home. Repeated bacterial cultures identified Clostridium botulinum producing botulinum neurotoxin A. Botulinum toxin A was isolated from his stools at 17, 21, and 23 days after the onset. Symptoms were self-limiting, and were fully recovered without anti-botulinum toxin globulin therapy. Conclusion This is the second report of a pediatric case with cancer chemotherapy-associated intestinal toxemia botulism. Our case provides further evidence that the immunocompromised status due to anti-cancer treatments increases the risk for the development of botulism at all ages in childhood
M A Mcshane - One of the best experts on this subject based on the ideXlab platform.
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progressive Bulbar Paralysis of childhood a reappraisal of fazio londe disease
Brain, 1992Co-Authors: M A Mcshane, S Boyd, B Harding, E M Brett, J WilsonAbstract:Fazio-Londe disease is a label sometimes applied to a degenerative disease of the motor neurons characterized by progressive Bulbar Paralysis in children. It is very rare with only 22 case reports describing 24 children including four sibling pairs. In two reports mothers and sons were affected. The neuropathology is described in only four cases. Previous authors have recognized that the condition is very heterogeneous. The clinical features of five children with this type of progressive Bulbar Paralysis, diagnosed at this hospital between 1969 and 1989, are reviewed, and in two cases neuropathological findings are detailed. Based on this experience, suggested criteria for diagnosis include clinical features of a pure motor neuronopathy affecting the Bulbar nuclei, exclusion of other causes of progressive Bulbar Paralysis and positive support for the diagnosis from electromyography and/or pathological examination. A review of the literature, combined with the present series, suggests that there are at least three distinct subtypes: a very rare autosomal dominant form (as described by Fazio) and two variants with probable autosomal recessive inheritance either with early onset of respiratory symptoms and rapid progression to death or later onset, less prominent respiratory symptoms and protracted clinical course. There is strong concordance for each clinical pattern within families.
Laura De Leon Flores - One of the best experts on this subject based on the ideXlab platform.
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clinical diagnosis in karwinskia humboldtiana polyneuropathy
Journal of the Neurological Sciences, 1998Co-Authors: Hector R Martinez, Ricardo A Rangelguerra, Maria Victoria Bermudez, Laura De Leon FloresAbstract:Intoxication by Karwinskia humboldtiana presents a neurological picture similar to that for Guillain-Barre syndrome or other polyradiculoneuropathies. Clinical diagnosis in poisoned humans may be difficult if no evidence of previous fruit ingestion is available. We present our experience in the clinical diagnosis of Karwinskia humboldtiana polyneuropathy, as confirmed by toxin detection in blood. We designed an open trial at the Pediatric Neurology service and included all cases with acute ascending Paralysis that were admitted to our hospital in the last two years. In all cases, we performed hematological, immunological and biochemical profiles, CSF analysis including immunological studies, oligoclonal bands and myelin basic protein determinations. Electrodiagnostic studies were performed, including motor conduction velocities, distal latencies, F-wave latency and compound muscle action potential (CAMP) amplitude. The presence of Karwinskia humboldtiana toxins in blood were determined by thin layer chromatography. In six cases, T-514 Karwinskia humboldtiana toxin was detected. These cases had a symmetric motor polyneuropathy with the absence of tendon reflexes and no sensory signs or cranial nerve involvement. Only one patient required assisted ventilation due to Bulbar Paralysis. In two of these cases, a sural nerve biopsy revealed a segmental demyelination with swelling and phagocytic chambers in Schwann cells and without lymphocytic infiltration. All six cases survived, with complete recovery in five. We conclude that this intoxication is common in Mexico. The availability of toxin detection in blood samples allows the clinician to establish an accurate diagnosis and should be included in the study of children with polyradiculoneuropathy, especially in countries where this poisonous plant grows.
Noriko Ohyama - One of the best experts on this subject based on the ideXlab platform.
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a childhood onset intestinal toxemia botulism during chemotherapy for relapsed acute leukemia
Annals of Clinical Microbiology and Antimicrobials, 2017Co-Authors: Noriko Ohyama, Michiko Torio, Kentaro Nakashima, Yuuki Koga, Shunsuke Kanno, Hisanori Nishio, Kei Nishiyama, Momoko Sasazuki, Haru Kato, Hiroshi AsakuraAbstract:Botulism is a potentially fatal infection characterized by progressive muscle weakness, Bulbar Paralysis, constipation and other autonomic dysfunctions. A recent report suggested that cancer chemotherapy might increase the risk for the intestinal toxemia botulism in both adults and children. We report a 5-year-old boy, who developed general muscle weakness, constipation, ptosis and mydriasis during the third induction therapy for relapsed acute myeloid leukemia. He had recent histories of multiple antibiotic therapy for bacteremia and intake of well water at home. Repeated bacterial cultures identified Clostridium botulinum producing botulinum neurotoxin A. Botulinum toxin A was isolated from his stools at 17, 21, and 23 days after the onset. Symptoms were self-limiting, and were fully recovered without anti-botulinum toxin globulin therapy. This is the second report of a pediatric case with cancer chemotherapy-associated intestinal toxemia botulism. Our case provides further evidence that the immunocompromised status due to anti-cancer treatments increases the risk for the development of botulism at all ages in childhood.
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A childhood-onset intestinal toxemia botulism during chemotherapy for relapsed acute leukemia
'Springer Science and Business Media LLC', 2017Co-Authors: Noriko Ohyama, Michiko Torio, Kentaro Nakashima, Yuuki Koga, Shunsuke Kanno, Hisanori Nishio, Kei Nishiyama, Momoko Sasazuki, Haru Kato, Hiroshi AsakuraAbstract:Abstract Background Botulism is a potentially fatal infection characterized by progressive muscle weakness, Bulbar Paralysis, constipation and other autonomic dysfunctions. A recent report suggested that cancer chemotherapy might increase the risk for the intestinal toxemia botulism in both adults and children. Case presentation We report a 5-year-old boy, who developed general muscle weakness, constipation, ptosis and mydriasis during the third induction therapy for relapsed acute myeloid leukemia. He had recent histories of multiple antibiotic therapy for bacteremia and intake of well water at home. Repeated bacterial cultures identified Clostridium botulinum producing botulinum neurotoxin A. Botulinum toxin A was isolated from his stools at 17, 21, and 23 days after the onset. Symptoms were self-limiting, and were fully recovered without anti-botulinum toxin globulin therapy. Conclusion This is the second report of a pediatric case with cancer chemotherapy-associated intestinal toxemia botulism. Our case provides further evidence that the immunocompromised status due to anti-cancer treatments increases the risk for the development of botulism at all ages in childhood
