RESUMEN
Cerebellar ataxia, neuropathy and vestibular areflexia syndrome (CANVAS) is a recently recognized neurodegenerative ganglionopathy. Prompted by the presence of symptomatic postural hypotension in two patients with CANVAS, we hypothesized that autonomic dysfunction may be an associated feature of the syndrome. We assessed symptoms of autonomic dysfunction and performed autonomic nervous system testing among 26 patients from New Zealand. After excluding three patients with diabetes mellitus, 83% had evidence of autonomic dysfunction; all patients had at least one autonomic symptom and 91% had more than two symptoms. We also found a higher rate of downbeat nystagmus (65%) than previously described in CANVAS. We confirmed that sensory findings on nerve conduction tests were consistent with a sensory ganglionopathy and describe two patients with loss of trigeminal sensation consistent with previous pathological descriptions of trigeminal sensory ganglionopathy. Our results suggest that autonomic dysfunction is a major feature of CANVAS. This has implications for the management of patients with CANVAS as the autonomic symptoms may be amenable to treatment. The findings also provide an important differential diagnosis from multiple system atrophy for patients who present with ataxia and autonomic failure.
Asunto(s)
Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Ataxia Cerebelosa/fisiopatología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Enfermedades Vestibulares/fisiopatología , Adolescente , Adulto , Edad de Inicio , Anciano , Anciano de 80 o más Años , Enfermedades del Sistema Nervioso Autónomo/complicaciones , Ataxia Cerebelosa/complicaciones , Mareo/fisiopatología , Femenino , Fuerza de la Mano/fisiología , Frecuencia Cardíaca/fisiología , Humanos , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Examen Neurológico , Nueva Zelanda , Nistagmo Patológico/etiología , Nistagmo Patológico/fisiopatología , Enfermedades del Sistema Nervioso Periférico/complicaciones , Reflejo Vestibuloocular/fisiología , Síndrome , Maniobra de Valsalva , Enfermedades Vestibulares/etiología , Pruebas de Función Vestibular , Vitamina E/sangre , Adulto JovenRESUMEN
Bleeding is the most important complication of treatment with intravenous tissue plasminogen activator for acute ischemic stroke. Neurologists are familiar with intracranial hemorrhage, the most feared site for bleeding following thrombolysis, but extracranial bleeding can also occur resulting in substantial morbidity and mortality. We describe an 88-year-old woman with an acute stroke who developed bleeding into the left arm complicated by hemodynamic instability and compartment syndrome following intravenous thrombolysis. The patient was treated conservatively in view of the risks associated with fasciotomy and her other medical comorbidities.
Asunto(s)
Brazo/patología , Síndromes Compartimentales/inducido químicamente , Síndromes Compartimentales/diagnóstico , Accidente Cerebrovascular/tratamiento farmacológico , Terapia Trombolítica/efectos adversos , Anciano de 80 o más Años , Femenino , Humanos , Accidente Cerebrovascular/diagnósticoRESUMEN
Multiple lines of evidence point to mitochondrial oxidative stress as a potential pathogenic cause for Parkinson's disease (PD). MitoQ is a powerful mitochondrial antioxidant. It is absorbed orally and concentrates within mitochondria where it has been shown to protect against oxidative damage. We enrolled 128 newly diagnosed untreated patients with PD in a double-blind study of two doses of MitoQ compared with placebo to explore the hypothesis that, over 12 months, MitoQ would slow the progression of PD as measured by clinical scores, particularly the Unified Parkinson Disease Rating Scale. We showed no difference between MitoQ and placebo on any measure of PD progression. MitoQ does not slow the progression of PD, and this finding should be taken into account when considering the oxidative stress hypothesis for the pathogenesis of PD.