Background: Stroke is the second leading cause of chronic disability and death in the world. Educating the public and physicians about stroke-related symptoms are critical success factors in early stroke treatment. Aim: This study was carried out in order to assess acute stroke management strategies in a teaching hospital. Patients and Methods:The study included all consecutive patients admitted to the general medical ward during the period of March to November 2008 who had a diagnosis of acute stroke. The total number was 217 patients, presumed to have stroke during their initial evaluation in the emergency room. However, ward evaluation confirmed only 193 patients to have a diagnosis of acute stroke (sudden onset of any neurological deficit). Brain imaging was performed in 167 out of the 193 patients. Results were obtained for 140 patients. Results: The age of those with clinical and radiological evidence of acute stroke (n=140) was 65±12 years. Sixty-five were females. The median time elapsed between the onset of symptoms and the arrival to the hospital was 12 hours (range: 1-336 h). Females presented earlier than males (19 hours versus 35 hours; p<0.05). Forty-eight patients (34%) presented within the first four hours and 71 (51%) arrived after eight hours, whilst 8 patients (6%) were not able to determine the time of onset of symptoms. The mean time for brain image from hospitalization was 3.5±2.3 days with no gender difference. Ischemic infarction was reported in 104/140 (74%) patients, intracerebral hemorrhage was reported in 16 (11%) patients and brain ischemia with hemorrhagic transformation was reported in 14 (10%). Normal brain images were found in 6 (5%) patients. 108 patients (78%) received in-hospital aspirin treatment with a mean time to start aspirin 1.2±1.4 days. The mean length of stay in hospital was 7.5±3.7 days. Ninety-nine (80%) of the patients were discharged on aspirin. The in-hospital outcome was assessed by the Rankin Disability Status scale as static course (in 107 patients; 85%), improvement of neurological function
Soft-tissue masses, other than malignant masses, of the head and neck rarely present with invasion of the base of the skull. [1] Inflammatory pseudotumor should be considered, while dealing with soft-tissue mass in the retropharyngeal area as it may mimic malignant neoplasm. Previous report showed four cases of nasopharyngeal inflammatory pseudotumor with skull base invasion in diabetic patients. [2] We describe a case of soft-tissue mass infiltrating the nasopharynx and extending up to the cavernous sinus. Knowledge about diversity of such case increases the awareness of neurologists, otolaryngologists, and radiologists and help in directing the appropriate management protocol.Retropharyngeal lesions have different spectrum of presentations. We herein present a case with step-wise progression of ambiguous symptoms and signs of polycraniopathy, caused by a soft-tissue mass in the retropharyngeal space extending into the cavernous sinus, as detected by magnetic resonance imaging. Initially, he was presented with hemifacial pain and lately progress rapidly to involve all cranial nerves; 3 rd through 12 th cranial nerves. The differential diagnosis was malignant tumor or aggressive infectious mass, which were excluded by histopathological examination. The diagnosis of inflammatory pseudotumor was a diagnosis of exclusion and decided based on a combination of clinical profile, blood test, radiological, and histopathological results.
PurposeProgressive myoclonic epilepsy type one is a neurodegenerative disorder characterized by action- and stimulus-sensitive myoclonus, tonic–clonic seizures, progressive cerebellar ataxia, preserved cognition, and poor outcome. The authors report clinical, neurophysiological, radiological, and genetic findings of an Emirati family with five affected siblings and review the literature.MethodsAll data concerning familial and clinical history, neurologic examination, laboratory tests, electroencephalogram, brain imaging, and DNA analysis were examined.ResultsGenetic testing confirmed the diagnosis of autosomal recessive progressive myoclonic epilepsy type 1 (EPM1) in two males and three females. The median age at onset was three years. Action- or stimulus-sensitive myoclonus and generalized seizures were recorded in 100% of our patients, at median age at onset of 3 and 4 years, respectively. Multisegmental myoclonus and generalized status myoclonicus were observed in 80% of our patients. Dysarthria and ataxia developed in 100% of our patients. Vitamin D deficiency and recurrent viral infections were noticed in 100% of our cohort. Cognitive, learning, and motor dysfunctions were involved in 100% of our patients. The sphincters were affected in 60% of our patients. Abnormal EEG was recorded in 100% of our cohort. Generalized brain atrophy progressively occurred in 60% of our patients. Phenytoin and carbamazepine were used in 60% of our patients with worsening effect. Valproate and levetiracetam were used in 100% of our patients with improving effect.ConclusionsThis is the first to report a family with EPM1 in UAE. Our study emphasized a particular phenotype expressed as earlier disease onset, severe myoclonus, and generalized seizures. Cognitive, cerebellar, motor, and autonomic dysfunctions and brain atrophy were also earlier at onset and more severe than previously reported. Recurrent viral infections are another unique feature. This constellation in tout à fait was not previously reported in the literature.
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