Idiopathic stringhalt in a Colombian Creole horse / Arpeo idiopático en un caballo Criollo Colombiano / Harpejamento idiopático em um cavalo Crioulo Colombiano

Anamnesis: an adult horse that showed hind limb hyperflexion was examined. Clinical and laboratory findings: at locomotion examination bilateral hyperflexion was observed; the right hind limb was more severely affected than the left. Electromyographic and histopathological examination revealed neural denervation and muscular atrophy supporting the idiopathic stringhalt diagnosis. Treatment approach: a lateral digital extensor tenectomy and partial myectomy was practiced in both hind limbs, accompanied by medical treatment and implementation of a mild exercise plan. The effectiveness of surgery is still controversial in these cases; however, this patient evidenced slow improvement after surgery and exercise seemed to be instrumental in the recovery of his normal locomotion. Conclusion: to our knowledge, this is the first report of a clinical case compatible with idiopathic stringhalt in Colombian Creole horses, but further studies are necessary to clarify the etiology and pathogenesis of stringhalt in Colombia.

Anamnesis: se examinó un caballo adulto que mostraba hiperflexión de ambos miembros posteriores. Hallazgos clínicos y de laboratorio: al examen locomotor se observó hiperflexión de ambos miembros posteriores pero el miembro posterior derecho parecía estar más afectado. El examen histopatológico y la electromiografía revelaron denervación neural y atrofia muscular soportando el diagnóstico de arpeo idiopático. Abordaje terapéutico: se practicó tenectomía y miectomía parcial del extensor digital lateral en ambos miembros posteriores, acompañada de tratamiento médico con la implementación de un plan de ejercicio ligero. La eficacia de la cirugía es controversial aún, sin embargo, en este caso, una lenta recuperación fue evidente y el ejercicio pareció ser un factor clave. Conclusión: el presente caso clínico es para nuestro conocimiento el primero compatible con arpeo idiopático en el Caballo Criollo Colombiano, pero se deben realizar más estudios para clarificar la etiología y patogenia del arpeo en Colombia.

Anamnese: foi examinado um cavalo adulto que mostrava hiperflexão dos dois membros posteriores. Achados Clínicos e de laboratorio: o exame foi observado hiperflexão de ambos os membros posteriores, estando mais afectado o membro posterior direito. O exame histopatológico e eletromiografia revelou denervação neural e atrofia muscular levando ao diagnóstico de harpejamento idiopático. Abordagem terapêutica: foi realizada tenectomia e miectomia parcial do músculo extensor digital lateral em ambos os membros posteriores, acompanhada de tratamento médico com a implementação de um plano de exercícios leves. A eficácia da cirurgia é ainda controversa, no entanto, neste caso foi evidente uma recuperação lenta e o exercício pareceu ser um fator fundamental. Conclusão: este relato de caso é, a nosso conhecimento, o primeiro compatível com harpejamento idiopático, mas devem ser realizados mais estudos para esclarecer a etiologia e patogenia do harpejamento no Cavalo Crioulo Colombiano.

Leucoencefalopatía hipóxico-isquémica retardada: caso clínico y revisión de la literatura / Hypoxic-ischemic delayed leukoencephalopathy: case report and review of the literature

Delayed Hypoxic-isquemic Leucoencephalopathy described in 1976 by Ginsberg is a brain white matter demyelinization phenomenon that occurred days or weeks after a hypoxic-isquemic injury followed by a complete recovery of the episode. The pathogenesis process remains unknown. We describe a 48 year old woman with cervico-uterine cancer in palliative treatment with opoids. She enters the emergency room with a respiratory depression, a prolonged hypotension and confusion, that it was recovered. At admission exhibits a recurrent pneumonia. Two weeks later, in conditions of discharge, initiates with agitation in context with rapidly progressive decline cognition, with concordant lesions of Leucoencephalopathy defined in the Magnetic Resonance (MR) study The metabolic profile, the cerebrospinal fluid and the electroencephalogram allowed dismissing other etiologic hypothesis. In front to the suspicious of Ginsberg syndrome, she had normal levels of Arylsulfatase. This acute post-hypoxic demyelinization process has been pathogenic interpreted as an arylsulfatase deficiency. Although numerous cases develop with normal arylsulfatase and the experimental studies of hypoxia, has support the hypothesis of a central hypoxic axonopathy due to failing in axonal transport as the base of the demyelinization phenomenal.

La leucoencefalopatía hipóxico-isquémica retardada (EHIR), descrita por Ginsberg en el año 1976, es un fenómeno desmielinizante de la sustancia blanca cerebral, que se origina días o semanas después de un daño hipóxico-isquémico que había sido seguido de una recuperación completa del episodio. La patogenia del proceso no está completamente establecida. Se presenta una mujer de 48 años portadora de cáncer cérvicouterino, en tratamiento paliativo con opiáceos. Ingresa al Servicio de Urgencia por una depresión respiratoria, hipotensión prolongada y compromiso de conciencia, donde es recuperada. Se hospitaliza por una neumopatía intercúrrente. Dos semanas más tarde, estando en condiciones de alta, se inicia agitación psicomotora en el contexto de un deterioro cognitivo rápidamente progresivo, que el estudio de Resonancia Magnética (RM) definió como lesiones concordantes con una leucoencefalopatía. El perfil metabólico, el líquido cefalorraquídeo y el electroencefalograma, permitieron descartar otras hipótesis etiológicas. Frente a la sospecha de un síndrome de Ginsberg, los niveles de arilsulfatasa fueron normales. Este proceso desmielinizante agudo post-hipóxico, ha sido interpretado patogénicamente como un déficit de arilsulfatasa. Sin embargo, la existencia de numerosos casos que cursan con arilsulfatasa normal y los estudios experimentales de hipoxia, ha avalado la hipótesis de una axonopatía central hipóxica, atribuible a fallas del transporte axoplásmico, como base para el fenómeno desmielinizante.

Paraparesia espástica progresiva asociada a HTLV-I en Chile: estudio y seguimiento de 121 pacientes por diez años / Progresive spastic paraparesis associated to HTLV-I in Chile

Revision is made to 121 Chilean patients with progressive adult spastic paraparesis (PSPs) associated to HTLV-I. Epidemiologic, clinical, diagnosis and associated illnesses aspects are analyzed as well as the pathogenesis. The follow-up of patients during several years allowed defining the evolutional profile, establishing the causes of death and studying the virus' behavior. Pathogenesis hypothesis arose from the neuropathological search to define the mechanisms of damage supported on immunohystochemical studies. It was confirmed that the CNS illness is a degenerative process linked to a central axonopathy which expresses flaws in the axoplasmic transport, particularly affecting the corticospinal tracts, although there is a more extended myeloencephalic involvement. Furthermore, the virus is capable of producing a multisystemic illness that may simultaneously involve the nervous system; the hematological system; the exocrine glands; the hepatic, lung, muscular and bone parenchymas.

Se revisan las paraparesias espásticas progresivas del adulto (PEPAs) producidas por el HTLV-I, en 121 pacientes chilenos. Se analizan los aspectos epidemiológicos, clínicos, diagnósticos, las enfermedades asociadas, y la patogenia. El seguimiento de los pacientes durante varios años permitió definir el perfil evolutivo, establecer las causas de muerte y estudiar el comportamiento del virus. De los casos con anatomía patológica surgieron hipótesis, que han permitido definir mecanismos de daño, sustentados en estudios inmunohistoquímicos. Se pudo confirmar que la enfermedad del SNC es un proceso degenerativo, vinculado a una axonopatía central que expresa fallas del transporte axoplásmico, que afecta particularmente la vía corticoespinal, aunque existe un compromiso más extenso mielo-encefálico. Además, el virus es capaz de producir una enfermedad multisistémica, que puede comprometer simultáneamente el sistema nervioso, el sistema hematológico, las glándulas exocrinas, el parénquima hepático, pulmonar, muscular y óseo.

Pathogenic Mechanisms and Causable Genes in Charcot-Marie-Tooth Disease / 한양의대학술지

Charcot-Marie-Tooth (CMT) disease is the most common form of inherited motor and sensory neuropathy. CMT is a genetically heterogeneous disorder of the peripheral nervous system; thus, many genes have been identified as CMTcausative genes. Traditionally, subclassification of CMT has been divided into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). Recently, intermediate type (CMT-Int) with NCVs between CMT1 and CMT2 is considered as a CMT type. There are several related peripheral neuropathies, such as Dejerine- Sottas neuropathy (DSN), congenital hypomyelination (CH), hereditary neuropathy with pressure palsies (HNPP), and giant axonal neuropathy (GAN). Great advances have been made in understanding the molecular basis of CMT, and 17 distinct genetic causes of CMT have been identified. The number of newly discovered mutations and identified genetic loci is rapidly increasing, and this expanding list has proved challenging for physicians trying to keep up with the field. In addition, the encouraging studies have been published on rational potential therapies for the CMT1A.

Electrophysiologic Assessement of Axonopathy and Demyelination in Diabetic Neuropathy according to the Severity

OBJECTIVE: To assess the axonopathy and demyelination neuropathy according to the electrophysiologic severity in diabetic neuropathy. METHOD: Electrophysiologic data of 246 patients who had been diagnosed with diabetic neuropathy was obtained and classified into suspected, possible, and definite groups by the criteria of our laboratory. Nerve conduction study was performed in the median, ulnar motor and sensory nerves, peroneal and tibial motor nerves, and sural nerve. Statistics were done with the results from the median motor and sensory, tibial motor and sural nerves. According to the severity of diabetic neuropathy, correlation and Chi-square analysis between amplitudes and latencies were performed. RESULTS: Frequencies of diabetic neuropathy according to electrophysiologic severity were as follows: 24 cases of suspected, 141 cases of possible, and 81 cases of definite neuropathy. The correlation ratios between amplitude and latency were 0.41- 0.79 (p<0.05) in the definite group of all the nerves examined, and below 0.3 in the suspected and possible groups. By Chi-square analysis, amplitude reduction was the predominant finding in the suspected and possible groups.si CONCLUSION: In the early stage of diabetic neuropathy, axonopathy might be the preceding pathogenesis, while with progression of diabetic neuropathy, axonopathy and demyelination may coexist.

Critical Illness Polyneuropathy: A Review of Seven Cases

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.

Critical Illness Polyneuropathy: A Review of Seven Cases

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.

Nerve Conduction studies of Sunacute combined Degeneration

OBJECT: There have been some controversies about the nature of peripheral neuropathy in patients with subacute comblned degeneration. Mayer concluded that the neuropathy was essentially demyelinating. And other reports which were based on pathologic or electrophyslological filldings have been saying axonopathy. We tried to find the nature of perlpheral neuropathy by doing conventional nerve conduction studies in 19 patients with subacute combined degeneration. SUBJECT AND METHOD: We included 19 patients with subacute combined degeneration, who were diagnosed by decreased serum vitamin B12(200pg/ml) and abnormal neurologic symptoms and/or signs. The patients were between 26 and 86 years of age. Eleven of them were male. We performed conventional nerve conduction studies Including H-reflex, When nerve conduction parameters deviated by more than 2SD from the normal mean value, they were consider as abnormal. RESULTS: nerve conduction studies were abnormal in 13/19. 11/13 with abnormal nerve conduction studies showed the pattern of peripheral polyneuropathy. Ten of them showed decreased amplitudes of sensory nerve action potentials or compound nerve action potentials with/wlthout mild slowing of nerve condcution. The abnormalities of the three patients with nerve conduction parameters of demyelinating range were confined to the distal segments of the median nerves. CONCLUSION: We thought that the results of the nerve conduction studies of our cases were compatible with axonopathy rather than demyelinopathy as a principal ]esion of the peripheral nervous system.