Acute Sensory and Autonomic Neuronopathy: A Devastating Disorder Affecting Sensory and Autonomic Ganglia.

Acute-onset and severe sensory and autonomic deficits with no motor dysfunction, typically preceded by a febrile illness, with poor recovery, and often fatal outcome are the hallmark features of acute sensory and autonomic neuronopathy (ASANN). Pathologically and electrophysiologically, ASANN is characterized by an extensive ganglionopathy affecting sensory and autonomic ganglia with preservation of motor neurons. Consequently, patients, usually children or young adult, develop acute-onset profound widespread loss of all sensory modalities resulting in automutilations, as well as autonomic failure causing neurogenic orthostatic hypotension, neurogenic underactive bladder, and gastroparesis and constipation. The diagnosis is clinical with support of nerve conduction studies and autonomic testing, as well as spinal cord magnetic resonance imaging showing characteristic posterior cord hyperintensities. Although the presumed etiology is immune-mediated, further studies are required to clarify the physiopathology of the disease. We here performed a systematic review of the epidemiology, pathophysiology, diagnosis, and management of ASANN, with three representative cases that recently presented at our clinic. All three patients had the typical clinical manifestations of ASANN but in different combinations, illustrating the variable phenotype of the disorder. Immunosuppression is seldom effective. Management options are limited to supportive and symptomatic care with the goal of minimizing complications and preventing death.

Key-Gaskell syndrome in Brazil: first case report / Síndrome de Key-Gaskell: primeiro relato de caso no Brasil

Feline dysautonomia is a devastating disease characterized by neuronal degeneration in autonomic ganglia that results in clinical signs related to dysfunction of the sympathetic and parasympathetic nervous systems. The cause is unknown and this disease has a poor prognosis and no definitive treatment. Most reports have been described in few countries around the world, but the prevalence may be underestimated in countries like Brazil. This study describes the progression and clinicopathological changes of dysautonomia in a 17-month-old female Brazilian shorthair cat...

Disautonomia felina é uma doença devastadora, caracterizada por degeneração neuronal em gânglios autonômicos, a qual resulta em sinais clínicos relacionados à disfunção dos sistemas simpático e parassimpático. Sua causa é desconhecida, o prognóstico desfavorável e não há tratamento definitivo disponível. A maioria dos relatos foi descrita em países ao redor do mundo, mas sua prevalência pode estar subestimada em países como o Brasil. Este estudo descreve a progressão e as alterações clínico-patológicas da disautonomia em um gato de pelo curto brasileiro, do sexo feminino, de 17 meses de idade...

Key-Gaskell syndrome in Brazil: first case report / Síndrome de Key-Gaskell: primeiro relato de caso no Brasil

Feline dysautonomia is a devastating disease characterized by neuronal degeneration in autonomic ganglia that results in clinical signs related to dysfunction of the sympathetic and parasympathetic nervous systems. The cause is unknown and this disease has a poor prognosis and no definitive treatment. Most reports have been described in few countries around the world, but the prevalence may be underestimated in countries like Brazil. This study describes the progression and clinicopathological changes of dysautonomia in a 17-month-old female Brazilian shorthair cat.(AU)

Disautonomia felina é uma doença devastadora, caracterizada por degeneração neuronal em gânglios autonômicos, a qual resulta em sinais clínicos relacionados à disfunção dos sistemas simpático e parassimpático. Sua causa é desconhecida, o prognóstico desfavorável e não há tratamento definitivo disponível. A maioria dos relatos foi descrita em países ao redor do mundo, mas sua prevalência pode estar subestimada em países como o Brasil. Este estudo descreve a progressão e as alterações clínico-patológicas da disautonomia em um gato de pelo curto brasileiro, do sexo feminino, de 17 meses de idade.(AU)

Morphoquantitative study of the submucous plexus (of Meissner) of the jejunum-ileum of young and old guinea pigs.

OBJECTIVE: To study the aging of submucous plexus of the small intestine (jejunum-ileum) of the guinea pigs from the quantitative, structural and ultrastructural perspective. METHOD: Chemical preparations of membrane of the jejunum-ileum of old and young animals with the use of light and electronic microscope. RESULTS: The ganglia of young animals presented between 1 and 56 neurons and the old animals presented from 1 to 30 neurons. The mean density of the ganglia by cm(2) in the young jejunum-ileum was of 551±36.89 and in the old one 413±11.86. The density of the neurons was 5011±291.11 neurons/cm(2) average in young animals and 2918±120.70 neurons/cm(2) in the old ones. The size of the neurons varied in both age groups. The collagen fibers in the ganglia of old animals they were condensed. Degenerated mitochondrias in the interior of the cell were frequent in the old animals. CONCLUSION: In submucous plexus of the jejunum-ileum there is a loss of 38% of the neurons with aging.

Morphoquantitative study of the submucous plexus (of Meissner) of the jejunum-ileum of young and old guinea pigs / Estudo morfoquantitativo do plexo submucoso (de Meissner) do jejuno-íleo de cobaias jovens e velhas

OBJECTIVE: To study the aging of submucous plexus of the small intestine (jejunum-ileum) of the guinea pigs from the quantitative, structural and ultrastructural perspective. METHOD: Chemical preparations of membrane of the jejunum-ileum of old and young animals with the use of light and electronic microscope. RESULTS: The ganglia of young animals presented between 1 and 56 neurons and the old animals presented from 1 to 30 neurons. The mean density of the ganglia by cm² in the young jejunum-ileum was of 551±36.89 and in the old one 413±11.86. The density of the neurons was 5011±291.11 neurons/cm² average in young animals and 2918±120.70 neurons/cm² in the old ones. The size of the neurons varied in both age groups. The collagen fibers in the ganglia of old animals they were condensed. Degenerated mitochondrias in the interior of the cell were frequent in the old animals. CONCLUSION: In submucous plexus of the jejunum-ileum there is a loss of 38 percent of the neurons with aging.

OBJETIVO: Estudar o envelhecimento do plexo submucoso do intestino delgado (jejuno-íleo) das cobaias do ponto de vista quantitativo, estrutural e ultra-estrutural. MÉTODO: Preparados de membrana do jejuno-íleo de animais jovens e velhos com a utilização de microscopia de luz e eletrônica. RESULTADOS: Os gânglios de animais jovens apresentaram entre 1 e 56 neurônios e os animais velhos apresentaram de 1 a 30 neurônios. A densidade média dos gânglios por cm² no jejuno-íleo jovem foi de 551±36,89 e no velho foi de 413±11,86. A densidade dos neurônios foi de 5011±291,11 neurônios/cm² em média nos animais jovens e 2918±120,70 neurônios/cm² nos velhos. O tamanho dos neurônios variou em ambos os grupos etários. As fibras colágenas nos gânglios de animais velhos estavam mais condensadas. Mitocôndrias degeneradas no interior da célula foram freqüentes nos animais velhos. CONCLUSÃO: No plexo submucoso do jejuno-íleo há uma perda de 38 por cento dos neurônios com o envelhecimento.

Efectos del losartán sobre el glomus carotídeo en ratas normotensas adultas / Effects of losartan on the carotid glomus in adult normotensive rats

Recientemente comunicamos lesiones graves en el glomus carotídeo y los ganglios autonómicos de ratas SHR y sugerimos que este efecto se debía más al aumento de la presión arterial que al envejecimiento. Posteriormente demostramos, en SHR, que el ramipril, en comparación con el atenolol, ejerce un efecto protector sobre estas estructuras más allá de la reducción de la presión arterial. Teniendo en cuenta que no existen trabajos que describan los cambios que origina el bloqueo AT1 sobre la morfología del glomus en ratas normotensas, se realizó el presente estudio con el objetivo de evaluar el efecto del losartán sobre esta estructura de ratas Wistar macho tratadas durante 8 meses. Se emplearon 14 ratas de 4 semanas de edad, divididas en grupos control y losartán (10 mg/kg/día en el agua de bebida). La presión sistólica (PAS) se registró al inicio y luego mensualmente. A la edad de 9 meses se sacrificaron las ratas y se extrajeron los glomus carotídeos, se tiñeron con hematoxilina-eosina y tricrómico de Masson y se procesaron para histomorfometría con un analizador de imágenes. El grupo control registró una PAS de 115 ± 8,1, mientras que en el grupo losartán fue de105 ± 8,3 mm Hg (p = 0,0375). Histomorfométricamente, el grupo tratado mostró un área mayor del glomus con respecto al control (497.931 ± 48.783 versus 59.668 ± 6.196 Am2; p <0,0001) y una relación pared/luz en las arteriolas glómicas de 0,7 ± 0,1 versus 2,7 ± 0,6,respectivamente (p < 0,0001). El grupo control mostró disminución del área glómica y un aumento de la relación pared/luz, lo cual sugiere que la atrofia de las estructuras estudiadas a través del aumento de la edad se vincula con el aporte nutricio arterial. (AU)

Efectos del losartán sobre el glomus carotídeo en ratas normotensas adultas / Effects of losartan on the carotid glomus in adult normotensive rats

Recientemente comunicamos lesiones graves en el glomus carotídeo y los ganglios autonómicos de ratas SHR y sugerimos que este efecto se debía más al aumento de la presión arterial que al envejecimiento. Posteriormente demostramos, en SHR, que el ramipril, en comparación con el atenolol, ejerce un efecto protector sobre estas estructuras más allá de la reducción de la presión arterial. Teniendo en cuenta que no existen trabajos que describan los cambios que origina el bloqueo AT1 sobre la morfología del glomus en ratas normotensas, se realizó el presente estudio con el objetivo de evaluar el efecto del losartán sobre esta estructura de ratas Wistar macho tratadas durante 8 meses. Se emplearon 14 ratas de 4 semanas de edad, divididas en grupos control y losartán (10 mg/kg/día en el agua de bebida). La presión sistólica (PAS) se registró al inicio y luego mensualmente. A la edad de 9 meses se sacrificaron las ratas y se extrajeron los glomus carotídeos, se tiñeron con hematoxilina-eosina y tricrómico de Masson y se procesaron para histomorfometría con un analizador de imágenes. El grupo control registró una PAS de 115 ± 8,1, mientras que en el grupo losartán fue de105 ± 8,3 mm Hg (p = 0,0375). Histomorfométricamente, el grupo tratado mostró un área mayor del glomus con respecto al control (497.931 ± 48.783 versus 59.668 ± 6.196 µm2; p <0,0001) y una relación pared/luz en las arteriolas glómicas de 0,7 ± 0,1 versus 2,7 ± 0,6,respectivamente (p < 0,0001). El grupo control mostró disminución del área glómica y un aumento de la relación pared/luz, lo cual sugiere que la atrofia de las estructuras estudiadas a través del aumento de la edad se vincula con el aporte nutricio arterial.

Plasminogen activator inhibitor-1 and transforming growth factor-beta 1 in carotid glomus and autonomic ganglia from spontaneously hypertensive rats.

BACKGROUND: Baroreflex and chemoreflex mechanisms play an important role in the dynamic adjustments of circulation and ventilation during daily life. Recently, we have observed atrophy and marked fibrosis in carotid glomus (CG) from old patients with carotid atherosclerosis who died following stroke. However, a possible limitation to interpretation of the results in that study was the superposition of arterial hypertension, atherosclerosis and aging in the patients. Taking this into account, spontaneously hypertensive rats (SHR) were used in order to study the CG in an experimental model with only hemodynamic stress. OBJECTIVE: To evaluate whether transforming growth factor-beta 1 (TGF-beta 1) and plasminogen activator inhibitor-1 (PAI-1) were involved in the extracellular matrix expansion in CG and autonomic ganglia (AG) in young, male, adult SHR. METHODS: Male SHR (n = 10) and Wistar-Kyoto (WKY) rats (n = 10) were used. Systolic blood pressure (SBP) was measured monthly up to 8 months of age, when the animals were killed; then, CG and AG were excised and processed for light microscopy and immunohistochemistry (TGF-beta 1, PAI-1 and protein S100). RESULTS: SBP was highly correlated (P < 0.01) with CG fibrosis (r = 0.90), AG fibrosis (r = 0.96) and neuron number (r = -0.97). PAI-1 and TGF-beta 1 in CG and AG were significantly increased (P < 0.01) in SHR. CONCLUSION: Severe damage was observed in CG and AG in SHR, which was, in addition, correlated with SBP. These results suggest that permanent high blood pressure produces remarkable lesions in these structures, even when the animals are not old. In view of the fact that CG and AG are of utmost importance in the genesis of cardiocirculatory reflexes, they might be considered as 'target organs' in arterial hypertension.

Cardiac autonomic dysfunction and neuroganglionitis in a rat model of chronic Chagas' disease.

OBJECTIVE: The aim was to evaluate whether the cardiac parasympathetic function in a rat model of chronic Chagas' disease is impaired as in the human disease, and to correlate the functional state to histopathology of the intrinsic autonomic innervation of heart. METHODS: 70 male Wistar rats 8 months infected with strains Y (n = 22), São Felipe (n = 18), and Colombia (n = 30) of Trypanosoma cruzi, were compared with 20 age and sex matched non-infected controls. Baroreflex bradycardia was quantified after multiple bolus injections of phenylephrine (3 to 12 micrograms). For each rat studied a mean was obtained of the absolute and relative (delta %) ratio (index) between the maximum heart rate decrease and the maximum systolic blood pressure increase. RESULTS: For the relative index the means were smaller (p less than 0.05) in the Y [-0.52(SD 0.19)%], São Felipe [-0.45(0.28)%], and Colombia [-0.53(0.21%)] subgroups, as well as in the pooled chagasic group [-0.51(0.22)%], than in the control group [-0.64(0.13)%]. In 32% (7/22), 33% (6/18), and 20% (6/30) of rats infected with Y, São Felipe, and Colombia strains, respectively, and in 27% (19/70) of the pooled group rats, the index exceeded the control group mean by -2 SD. After atropinisation, a similar pronounced reduction (p less than 0.01) in the index was observed in all groups [-84(28)% to -95(17)%]; however, rats with depressed bradycardia showed a smaller (p less than 0.05) reduction in the relative index than control rats, at -70(34) v -92(16%). Inflammatory and degenerative lesions of the intrinsic cardiac innervation were observed in 87% of the rats with autonomic dysfunction. Rats with the lesions showed a mean relative index that was smaller than those without lesions, at -0.44(0.23) v -0.64(0.20)% (p less than 0.01), and also smaller than in the controls. CONCLUSIONS: Cardiac autonomic dysfunction expressed by reduced baroreflex bradycardia was detected in rats chronically infected with T cruzi, as in human Chagas' disease. The disturbance, shown for the first time in an animal model of chagasic infection, resulted primarily from impaired efferent parasympathetic activity caused by intrinsic neuroganglionar lesions.

Estudo comparativo das lesöes dos gânglios e nervos atriais em chagásicos crônicos / Comparative study of atrial ganglia and nerves lesions in patients with chronic Chagas' disease

Através de cortes seriados do terço superior do septo interatrial de coraçöes de chagásicos crônicos estudaram-se comparativamente as alteraçöes dos gânglios e filetes nervosos constatando-se que: sinais de degeneraçäo hidrópica e destruiçäo neuronal foram encontrados em todos os chagásicos, enquanto os sinais de degeneraçäo de fibras nervosas ocorreram em apenas 20% dos casos; o exsudato leucocitário constituído por mononucleares mais freqüentemente associou-se aos gânglios que os filetes nervosos, sendo, em ambos, mais freqüentemente localizado na periferia (periganglionite e perineurite) que na intimidade (ganglionite e neurite) sem relaçäo topográfica com as demais alteraçöes do tecido nervoso atrial. A pesquisa de parasitos foi negativa em todos os casos. O presente estudo nos permite concluir que o processo inflamatório que acomete predominantemente a periferia dos gânglios nervosos atriais é eletivo e näo secundário à epicardite, näo parecendo responsável pela degeneraçäo e destruiçäo neuronal e se formando independentemente do parasito local

Effect of alcohol on the rat juxtaprostatic pelvic ganglia and gonads.

In the present paper, it was investigated the aspect of the intrapelvic autonomic neuronal system (juxtaprostatic ganglion) and gonads of rats submitted to a experimental alcoholism. The animals which had water substituted for 35% sugar cane spirit for 30 d, presented testicular lesions as well as numeric and degenerative alterations in the juxtaprostatic pelvic ganglia neurons.

Híbridos de gânglio e paragânglio em coraçäo de chagásico crônico hipertenso: relato de caso / Hybrid ganglion and paraganglion in the heart of a hypertensive patient with chronic Chagas' disease: a case report

No septo interatrial de um paciente chagásico crônico e hipertenso, com ninho de formas amastigotas de T. cruzi na musculatura lisa da veia central da supra-renal, foram encontradas duas estruturas híbridas de gânglio nervoso e paragânglio, nas quais os tecidos nervoso e paraganglionar se fundiram. Além destes, havia mais um paragânglio isolado na gordura interatrial. Exsudato inflamatório constituído por mononucleares foi observado nos gânglios nervosos intracardíacos, nos híbridos de gânglio e paragânglio, no paragânglio e na medular das supra-renais. Embora o exsudato mononuclear pareça relacionado com a tripanossomíase, a abundância de tecido paraganglionar cardíaco pode ter relaçäo com a hipertensäo arterial sistêmica

Postural hypotension in idiopathic Parkinson's disease. Etiopathology.

Postural changes in blood pressure were recorded in all 391 patients suffering from Parkinson's syndrome over a period of six years. Intraarterial blood pressure studies were carried out in those with significant postural hypotension. Histological examination of the entire central nervous system and the sympathetic ganglia was performed in six patients suffering from idiopathic Parksinson's disease. Five of the six patients had Lewy bodies in the sympathetic ganglia. Loss of nerve cells was noted in the sympathetic ganglia in those patients that demonstrated postural hypotension. The severity of the lesions in the ganglia correlated with the severity of postural hypotension in idiopathic Parkinson's disease, One case of Shy-Drager syndrome was similarly studied to demonstrate the differences in spinal cord and sympathetic ganglia lesions in the two conditions.