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BACKGROUND AND OBJECTIVES: Few studies have attempted to delineate the clinical profile of myasthenia gravis (MG) among people of Arab ancestry. Therefore, we sought to clarify the clinical profile, the outcome of treatment and the role of thymectomy in non-thymomatous MG in Saudi Arabia. PATIENTS AND METHODS: We retrospectively studied 104 patients followed over a mean period of 7.2 years (range, 1 to 22 years) at the King Khaled University Hospital, Riyadh, Saudi Arabia. Disease outcomes were compared among thymectomized and non-thymectomized patients according to the post-intervention status criteria of the Myasthenia Gravis Foundation of America (MGFA). RESULTS: Age of onset was 22.5+/-9.3 years (meanA+/-SD) in females and 28.2+/-15.9 years in males, with peaks in the second and third decades among females and the third and fourth decades among males. At diagnosis, a majority of patients had moderate generalized weakness, equivalent to MGFA class III severity. After medical treatment with or without thymectomy, 9.6% of all patients had achieved complete stable remission, 3.8% had pharmacological remission, 27.9% had minimal manifestations, 23.1% were improved, 20.2% were unchanged and 15.4% were worse. Only thymectomized patients without a thymoma achieved remission, a significant benefit over those who had no thymectomy (P=.02). CONCLUSION: MG presents at a younger age among Saudi Arabs compared to other racial groups. Thymectomy conferred significant benefits towards achievement of remission.
Asunto(s)
Árabes , Miastenia Gravis/terapia , Timectomía , Adolescente , Adulto , Edad de Inicio , Anciano , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/etnología , Inducción de Remisión/métodos , Estudios Retrospectivos , Arabia Saudita/epidemiología , Arabia Saudita/etnología , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Adulto JovenRESUMEN
OBJECTIVE: To examine the utility of the sympathetic skin response SSR as a measure of impaired autonomic function among diabetic patients in Saudi Arabia. METHODS: In this case-control study, baseline SSR was obtained from 18 healthy subjects, followed by nerve conduction studies, and SSR testing on a consecutive cohort of 50 diabetic patients with peripheral neuropathy. The SSR in diabetic patients was compared between those with autonomic neuropathy and those without autonomic neuropathy. This study was conducted at the King Khaled University Hospital, Riyadh, Saudi Arabia, from June 2006 to June 2007. RESULTS: The SSR was present in all healthy subjects, and in 32 diabetic patients. Among 16 patients with autonomic neuropathy, the SSR was absent in 14 and present in 2, while 4 of 34 patients lacking evidence of autonomic neuropathy had absent SSR. Using Fisher's exact test, we found a strong association between absent SSR and autonomic neuropathy p<0.001, however, not with age or duration of diabetes mellitus. As a diagnostic test of autonomic neuropathy, the SSR had a sensitivity of 87.5%, a specificity of 88.2%, a positive predictive value of 77.8%, and a negative predictive value of 93.7%. CONCLUSION: Absence of the SSR is a reliable indicator of autonomic neuropathy among patients with diabetes mellitus in Saudi Arabia.
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Enfermedades del Sistema Nervioso Autónomo/fisiopatología , Neuropatías Diabéticas/fisiopatología , Piel/inervación , Sistema Nervioso Simpático/fisiopatología , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Conducción Nerviosa/fisiología , Valor Predictivo de las Pruebas , Sensibilidad y EspecificidadRESUMEN
Multiple sclerosis is an autoimmune demyelinating disease that is rarely associated with aseptic meningitis. However, certain syndromes causing aseptic meningitis are often associated with central nervous system demyelination that mimics multiple sclerosis (MS). Since many of these syndromes are potentially treatable, unmasking an alternative diagnosis is essential whenever an MS-like illness and recurrent meningitis are encountered in the same patient. Yet, the search for an alternative diagnosis may be elusive sometimes, despite extensive and appropriate investigations. We present a young woman with an MS-like illness associated with recurrent meningitis over a 7-year period. After an exhaustive evaluation, we conclude that recurrent meningitis is an atypical manifestation of MS. If neurologists would appreciate this point, unrewarding and costly investigations may be avoided and appropriate therapy instituted when similar cases are encountered in clinical practice.
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OBJECTIVE: To assess the value of brainstem auditory evoked potentials and event related evoked potential (3rd positive component of evoked related potentials with latency of 300 millisecond, in evaluating cognitive dysfunction in patients with chronic respiratory failure. METHODS: Thirty-two patients with chronic obstructive pulmonary disease and respiratory failure of mild to moderate severity, were assessed regarding their mental function, utilizing mini-mental state examination, arterial blood gases including PH, partial pressure of carbon dioxide, partial pressure of oxygen, and both brainstem auditory evoked potentials and event related evoked potential response. Twenty-five normal subjects, matched for age and sex, were also studied as a control group. The study was carried out during the year 1999 to 2000 in 3 hospitals; King Khalid University Hospital, King AbdulAziz University Hospital and Sahara Hospital, Riyadh, Kingdom of Saudi Arabia. RESULTS: There were significant delay of event related evoked potential response in patients compared with controls (P<0.05). No significant difference was noted for brainstem auditory evoked potentials and mini-mental state examination scores were within normal limits in 78% of patients. When event related evoked potential were analyzed in comparison with blood gases and mini-mental state examination, there was a clear moderate correlation with severity of hypoxemia (r = -0.697). Correlation was also noted, but to a lesser degree with partial pressure of carbon dioxide (r = 0.52) and PH (r = 0.53). There was no correlation with mini-mental state examination. CONCLUSION: The significant delay of event related evoked potential, which is considered the neuro-physiological correlate of cognition, points clearly to the presence of a certain degree of mental dysfunction in many of these patients, namely sub-clinical encephalopathy. These subtle changes commonly evade detection by conventional bed side test (mini-mental state examination), while detailed neuropsychological assessment is cumbersome and time consuming. So, event related evoked potential measurement may be an objective and practical test of subtle cognitive dysfunction in mild respiratory failure. Unfortunately, absolute event related evoked potential values may not be useful in individual patients, in view of its wide range. However, it is probably very helpful in the assessment of a group of subjects, such as trials of a new therapeutic modality. A follow-up study utilizing a larger group of patients, and formal neuropsychological mental assessment, will be expected to confirm and expand the present study`s conclusions.