A multidisciplinary approach to assess recovery of consciousness in a patient with moyamoya disease.

INTRODUCTION: Moyamoya disease (MMD) meaning "hazy puff of smoke" in Japanese is a rare chronic cerebrovascular syndrome characterized by progressive stenosis and occlusion of the internal carotid arteries (ICAs) anterior cerebral arteries (ACAs), and middle cerebral arteries (MCAs). METHODS: Our moyamoya patient with severely impaired cognitive and motor functions underwent a specific motor and neuropsychological rehabilitative treatments to assess the recovery of consciousness. RESULTS: Results obtained showed an improvement of clinical and neuropsychological examination. These findings highlighted the importance of an intensive rehabilitation techniques used in the care of disorders of consciousness patients. CONCLUSIONS: The use of sensory methods provides advantages for the rehabilitation. In fact, in this study, we showed a correlation between sensory stimulation and changes in patient's clinical status.

Spontaneous occlusion of the circle of Willis in a young woman with epilepsy: epileptic-type Moyamoya disease.

The authors report a case of a 31-year-old woman from India with history of seizure disorder who presented with sudden onset right hemiparesis and right-sided upper motor neuron type facial palsy. No identifiable risk factors were noted on admission and all laboratory investigations were negative. MR angiography helped in arriving at the diagnosis of moyamoya disease as the aetiology of her symptoms.

Long-term social outcome in children with moyamoya disease who have reached adulthood.

OBJECT: Although the reported surgical outcome is favorable, there is little information regarding the long-term quality of life in pediatric patients with moyamoya disease (MMD) when they grow to adulthood. The authors conducted a survey to provide details of social adaptation and satisfaction in adults who underwent revascularization surgery for MMD during childhood. METHODS: One hundred twenty-three patients with MMD who had undergone surgery during childhood and were older than 18 years of age were recruited for this study. The authors mailed self-administered questionnaires regarding each patient's education, employment, marriage, driver's license, physical condition, and satisfaction. Sixty-five patients (53%) responded. RESULTS: Compared with the general population, the patients showed a similar rate of attaining a higher education. Forty-nine (80%) of 61 patients who were not currently high school students had entered college or university. However, the presence of neurological deficits on preoperative examination was a negative predictor of entrance into a college or university, as well as employment. Some patients had difficulty in planning marriage because of physical handicaps, and the rate of acquiring a driver's license and actual driving were relatively low. Approximately 80% of the responders were satisfied with their treatment outcomes, but more than one-half reported subjectively assessed neurological problems. CONCLUSIONS: In this study, pediatric patients with MMD showed favorable social adaptation in adulthood. The findings also indicated that early diagnosis and intervention before the establishment of neurological deficits are essential to children with MMD in becoming better socially adapted. Further, more thorough clinical examinations are required during follow-up in patients with MMD.

Moyamoya disease.

A 4 years old boy presented with acute left hemiplegia. Preliminary neuroimaging suggested an arterial ischemic process. Clinical and laboratory evaluation excluded haematologic, metabolic and vasculitic causes. Cerebral angiography confirmed the diagnosis of Moyamoya disease. Treatment included physiotherapy and close follow-up for recurrence.

Tratamiento rehabilitador en la CADASIL: a propósito de un caso / Rehabilitation treatment in CADASIL disease. A case report

La arteriopatía autosómica dominante con infartos subcorticales y leucoencefalopatía (CADASIL) es una angiopatía con herencia autosómica dominante por mutación en el gen NOTCH3 del cromosoma 19. La enfermedad produce migraña con aura, alteraciones psiquiátricas, episodios isquémicos y déficit cognitivos. Su evolución es progresiva y escalonada. Su diagnóstico requiere confirmación detectando la mutación genética característica y/o cambios anatomopatológicos en la piel. El caso que se presenta es un varón de 63 años con cuadros neurológicos episódicos de hemiparesia izquierda y síndrome seudobulbar, con secuelas progresivas. En febrero de 2004 se le diagnosticó CADASIL con mutación G428A (Gys171Tyr) en el exón 4 del gen NOTCH3. Se pautó tratamiento de rehabilitación durante 3 meses utilizando técnicas de fisioterapia y terapia ocupacional. Al año el resultado del tratamiento ha sido satisfactorio. El curso progresivamente incapacitante y la ausencia de tratamiento etiológico hace fundamental un programa adecuado y multidisciplinario de rehabilitación(AU)

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is a dominant autosomal hereditary angiopathy produced by the mutation of NOCTH 3 gene in chromosome number 19. The symptoms of the disease include migraine with aurea, psychiatric problems, ischemic episodes and cognitive deficits. The typical course of CADASIL is slowly progressive. The diagnosis must be genetically confirmed through the detection of its characteristic mutations and/or some cutaneous pathological changes. We present the case of a 63-year-old man who suffered from episodic neurological deficits, left hemiparesis, pseudobulbar syndrome, and progressive sequelae. In February 2004, the diagnosis of CADASIL was reached, due to mutation G428A (Gys171Tyr) in exon number 4 of NOTCH 3 gene. Treatment was performed with physical and occupational therapy for 3 months. At one year of follow-up, the result was satisfactory. Due to the disabling and progressive outcome of the disease, and to the lack of etiological treatment, physical and occupational therapy are paramount to minimize its functional impact and sequelae(AU)

Disminución de la presión arterial en pacientes con arteriopatía periférica mediante el masaje reflejo del tejido conjuntivo / Decrease in blood pressure in patients with peripheral arterial disease by connective tissue reflex massage

Objetivos: Evaluar la eficacia del masaje reflejo del tejido conjuntivo en la presión arterial sistólica y diastólica en pacientes con enfermedad arterial periférica. Metodología: Participaron en el estudio 26 pacientes con enfermedad arterial periférica del Distrito Sanitario de Granada durante un período de intervención de 15 semanas. La muestra de estudio se dividió de forma aleatoria en grupo experimental y grupo control. Los criterios de exclusión fueron los siguientes: insuficiencia arterial periférica en estadios más avanzados, insuficiencia venosa periférica, insuficiencia cardíaca, renal o hepática. La variable independiente considerada ha sido la aplicación del masaje reflejo del tejido conjuntivo según el método de Elizabeth Dicke. Asimismo, la variable dependiente estudiada ha sido la evaluación de la presión arterial sistólica y diastólica en ambas extremidades superiores. Resultados: Se observan diferencias significativas entre la valoración basal y las valoraciones posbasales, entre los dos grupos de estudio, en la presión arterial diastólica derecha (basal, p<0,043; primera valoración, p<0,041; segunda valoración, p<0,047) y la presión arterial sistólica izquierda (basal, p<0,042; primera valoración, p<0,04; segunda valoración, p<0,049). Conclusiones: El masaje reflejo del tejido conjuntivo genera un descenso de la presión arterial en pacientes con enfermedad arterial periférica en estadio I(AU)

Aims: Evaluate the effectiveness of connective tissue reflex massage on systolic and diastolic blood pressure in patients with peripheral arterial disease. Methodology: Twenty-six patients with peripheral arterial disease from the Health District of Granada participated in the study during a 15-week intervention period. The study sample was randomly divided into an experimental group and a control group. Exclusion criteria were peripheral arterial insufficiency at more advanced stages, peripheral venous insufficiency, cardiac, renal or hepatic insufficiency. Application of the connective tissue reflex massage according to the method of Elizabeth Dicke was regarded as the independent variable. The dependent variable was evaluation of systolic and diastolic blood pressure in both upper limbs. Outcomes: Significant differences could be observed between the baseline and post-baseline evaluations between the two study groups in the right diastolic blood pressure (baseline, p<0.043; 1st evaluation, p<0.041; 2nd evaluation, p<0.047), and left systolic blood pressure (basal, p<0.042; 1st evaluation, p<0.04; 2nd evaluation, p<0.049). Conclusions: Connective tissue reflex massage causes a blood pressure decrease in patients with stage I peripheral arterial disease(AU)

[A case of recurrence of cerebral hemorrhage in a patient with adult moyamoya disease in the recovery period rehabilitation ward].

A 61-year-old woman with a history of repeated cerebral hemorrhage due to moyamoya disease was admitted to our recovery period rehabilitation ward for walking training. She suddenly noticed paresthesia in her left forearm and the palm seven days later. Brain computed tomography on that day and magnetic resonance imaging on the next day after the onset of paresthesia revealed right thalamic hemorrhage. It was suggested that the hematoma localized to a small part of the central portion of the ventroposterior lateral nucleus caused paresthesia limited to the forearm and the palm. In a recovery period rehabilitation ward, we should carefully listen to the complaints of patients who are in advanced age and have a risk like our patient. Then adequate neurological examination should be performed for the pertinent inspection diagnosis, if needed.

Neurorehabilitation outcome in moyamoya disease.

Moyamoya is a disease characterized by occlusion of the internal carotid, anterior, and middle cerebral arteries with associated rich collateral flow that presents a cloudy appearance on angiogram resembling a puff of smoke. The disease is most often progressive with associated hemiparesis and cognitive impairment. The functional outcome of patients with moyamoya is not well described in the literature. We describe four women (ages 25-36) who were transferred to a rehabilitation service after an average 17 days (12-26 days) in an acute care setting. Initial functional impairment was estimated using the Functional Independence Measure (FIM) score after discharge from inpatient rehabilitation (23-53 days) and was compared to the Uniform Data System for Medical Rehabilitation (UDSMR) for "first stroke" patients. Average admission FIM scores were similar in the two groups. The patients with moyamoya had a higher discharge FIM, longer length of stay, and slower rate of progress. Data on long-term survival and functional level would be useful, but it appears patients with moyamoya disease may benefit from rehabilitation oriented toward neurological deficits.

[Follow-up study on the relationship between age at onset of illness and outcome in patients with moyamoya disease].

Follow-up studies over more than one and a half years (average 6.5 years) were performed in 51 patients with "moyamoya disease". Activities of daily living (ADL) was assessed and graded into five levels both at the time of admission into hospital and at the last assessment in the follow-up study, as follows. Grade I: Fully capable of work or study with no or minimal difficulty, II: Capable of limited work or study with difficulty, III: Incapable of work or study but capable of home life alone, IV: Incapable of home life without help, V: Vegetative state or dead. Patients were divided into three groups by the age of clinical onset: younger pediatric, less than 5 years old (21 patients); older pediatric, 5 to 15 years old (14 patients); and adult, 16 years old and over (16 patients). Although analysis of the angiographical and neurological pictures is important, the present paper concerns only the age of clinical onset and whether reconstructive vascular surgery was performed, because of the small number of clinical materials. Seven of 16 adults suffered hemorrhagic attacks and 2 of them died of hemorrhagic insults. In contrast, none of the pediatric cases had hemorrhagic attacks and none died. Both at the time of admission and last assessment, ADL of younger pediatric cases was much lower than that of older ones. These results suggest that clinical course in patients with "moyamoya disease" closely related with the age of the onset of illness, and the border age of onset of illness between patients with good outcome and with poor outcome is around 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)