Objectives: To describe the clinical profile of dystonic syndromes seen in the Sultanate of Oman.... more Objectives: To describe the clinical profile of dystonic syndromes seen in the Sultanate of Oman. Design: A retrospective hospital study of patients with dystonic syndromes seen in the Sultan Qaboos University Hospital during the period from 1990-1994. Results: We encountered 30 patients with dystonia; generalized in 13 (43%), focal in 10 (33%) and hemidystonia in 7 (24%). The predominant CT scan brain abnormalities were diffuse cerebral cortical atrophy in generalized dystonias and contralateral cerebral cortical atrophy in hemidystonias. Heredodegenerative diseases were the commonest cause in this series.
Twenty seven children with intractable seizures in the age group of 5 months - 14 years were trea... more Twenty seven children with intractable seizures in the age group of 5 months - 14 years were treated with vigabatrin (VGB) and Lamotrigene (LTG) as an add-on therapy. Fourteen children (6M:8F) were given VGB and thirteen children (9M:4F) received LTG. The drug selection was made according to the type of seizures and associated neurological abnormalities. Attempt was made to exclude children with progressive neurologic disorders or severe systemic disease. EEG, CT scan head and relevant metabolic work up was done in all. VGB was found to be most effective in seizures associated with tuberous sclerosis. It was always useful in infantile spasm (IS) and tonic clonic (TC) seizures. LTG was effective in complex partial seizure (CPS) and TC seizures withmyoclonus.
Objective: To look for pattern of non-convulsive status epilepticus in children with epilepsy at ... more Objective: To look for pattern of non-convulsive status epilepticus in children with epilepsy at the Sultan Qaboos University Hospital, Sultanate of Oman. Methods: Twelve children (6 male and 6 female) with non-convulsive status epilepticus were seen at Sultan Qaboos University Hospital from December 1992 until December 1997. The diagnosis was suspected in children with previous history of seizures who had recent change in behavior, memory, automatism or ataxia, or children presenting with unexplained ataxia, speech loss and prolonged inattention for the first time. All the patients were observed in the hospital. Electroencephalogram correlation with the above clinical presentation, of at least 30 mintues was used to confirm the diagnosis. Results: Complex partial seizures status and Lennox-Gastaut syndrome status constituted the majority (7 cases). Intravenous midazolam and phenytoin sodium were used to control status in 9 cases. On follow-up, 5 were adequately controlled, while 6 had recurrence. One patient was lost to follow-up. Conclusion: Non-convulsive status epilepticus should be suspected in children with recent onset change in speech, memory, school performance, unexplained ataxia and particularly in children with epilepsy who undergo an otherwise inexplicable change in behavior.
Fifty cases of eating epilepsy (EE) are reported. Mastication of food produced seizures in 49 (98... more Fifty cases of eating epilepsy (EE) are reported. Mastication of food produced seizures in 49 (98%) and swallowing in 1 (2%). Complex partial seizure was found in 48 cases, the commonest form encountered (96%). EEG was abnormal in 15 cases (30%). The literature and possible mechanisms for seizure are discussed.
Abstract Kuthar valley in the Anantnag district of south Kashmir (North‐west India) was surveyed ... more Abstract Kuthar valley in the Anantnag district of south Kashmir (North‐west India) was surveyed to ascertain the prevalence of completed strokes. In a population of 63,645, the survey was from July to November 1986; 91 cases of completed stroke were detected giving a crude prevalence of 143/100,000. Only 10 (10.9%) cases (7W : 3M) were in the age group 15–39 years, a prevalence rate of 41/100000.
DOAJ (DOAJ: Directory of Open Access Journals), 1999
ABSTRACT Segmental spinal myoclonus (SSM) involving a child's lower limb revealed the pre... more ABSTRACT Segmental spinal myoclonus (SSM) involving a child's lower limb revealed the presence of a spinal cord tumour from T8 to T12. The clinical and electrophysiological features of SSM-during the child's development were studied. The presence of this disorder at two months of age did not disturb the development of walking, but did lead to orthopaedic complications. Four electromyographic recordings performed between 23 months and 7 years 10 months of age revealed the topographical extension of the SSM, its passage to rhythmicity, variable frequency and change in the sequence of activation of the muscles. These electroclinical features enable discussion of the pathophysiology of SSM by reference to neonatal motor automatisms and spinal generators.
Five children (3F:2M), in the age group 1 years to 11 years, with Munchausen syndrome by proxy ar... more Five children (3F:2M), in the age group 1 years to 11 years, with Munchausen syndrome by proxy are reported from the Sultanate of Oman. They were seen over a four years period from 1996-1999. In all these children, the mother came up with history of uncontrolled epilepsy. Carbamazepine was the most common antiepileptic drug used. One of these children remained hospitalized elsewhere for nearly 9 months, as a case of uncontrolled status epilepticus. It took 18 months to 6 years (mean 2.8 years) to establish the diagnosis and the mother was the offender in all. The main lead to diagnosis, was the disparity between history and clinical presentation to hospital. The carbamazepine levels were several times above the upper limit of therapeutic range. Munchausen syndrome by proxy very much exists here, but is possibly less recognized and needs immediate attention to formulate policies to identify and manage these children. It is necessary to create awareness even in the medical community, to recognise this problem. There is an urgent need to develop a child protection council at the national or regional level.
Dandy-Walker syndrome in monozygotic twins is reported. The twins reported, presented with delaye... more Dandy-Walker syndrome in monozygotic twins is reported. The twins reported, presented with delayed development, big head and dysmorphic features. In addition, there were significant cafe-au-lait spots on the trunk and other minor features consistent with the diagnosis of neurofibromatosis. To the best of our knowledge, Dandy-Walker syndrome in combination with neurofibromatosis in monozygotic twins has not been previously reported.
Journal of Neurosciences in Rural Practice, Nov 2, 2022
Seizures are not common in cases with chronic liver disease. Overall seizures have been reported ... more Seizures are not common in cases with chronic liver disease. Overall seizures have been reported in 20–30% of cases in chronic liver disease associated with hepatic encephalopathy. We report two cases of chronic liver disease patients who presented with new-onset refractory focal status epilepticus (SE). Both patients had encephalitis and seizures which responded only when acyclovir was added to the treatment with antiepileptic medication. Herpes encephalitis should be considered as a possible diagnosis in new-onset focal seizures or focal SE in patients with chronic liver disease with or without hepatic encephalopathy, pending further investigations.
Objectives: To describe the clinical profile of dystonic syndromes seen in the Sultanate of Oman.... more Objectives: To describe the clinical profile of dystonic syndromes seen in the Sultanate of Oman. Design: A retrospective hospital study of patients with dystonic syndromes seen in the Sultan Qaboos University Hospital during the period from 1990-1994. Results: We encountered 30 patients with dystonia; generalized in 13 (43%), focal in 10 (33%) and hemidystonia in 7 (24%). The predominant CT scan brain abnormalities were diffuse cerebral cortical atrophy in generalized dystonias and contralateral cerebral cortical atrophy in hemidystonias. Heredodegenerative diseases were the commonest cause in this series.
Twenty seven children with intractable seizures in the age group of 5 months - 14 years were trea... more Twenty seven children with intractable seizures in the age group of 5 months - 14 years were treated with vigabatrin (VGB) and Lamotrigene (LTG) as an add-on therapy. Fourteen children (6M:8F) were given VGB and thirteen children (9M:4F) received LTG. The drug selection was made according to the type of seizures and associated neurological abnormalities. Attempt was made to exclude children with progressive neurologic disorders or severe systemic disease. EEG, CT scan head and relevant metabolic work up was done in all. VGB was found to be most effective in seizures associated with tuberous sclerosis. It was always useful in infantile spasm (IS) and tonic clonic (TC) seizures. LTG was effective in complex partial seizure (CPS) and TC seizures withmyoclonus.
Objective: To look for pattern of non-convulsive status epilepticus in children with epilepsy at ... more Objective: To look for pattern of non-convulsive status epilepticus in children with epilepsy at the Sultan Qaboos University Hospital, Sultanate of Oman. Methods: Twelve children (6 male and 6 female) with non-convulsive status epilepticus were seen at Sultan Qaboos University Hospital from December 1992 until December 1997. The diagnosis was suspected in children with previous history of seizures who had recent change in behavior, memory, automatism or ataxia, or children presenting with unexplained ataxia, speech loss and prolonged inattention for the first time. All the patients were observed in the hospital. Electroencephalogram correlation with the above clinical presentation, of at least 30 mintues was used to confirm the diagnosis. Results: Complex partial seizures status and Lennox-Gastaut syndrome status constituted the majority (7 cases). Intravenous midazolam and phenytoin sodium were used to control status in 9 cases. On follow-up, 5 were adequately controlled, while 6 had recurrence. One patient was lost to follow-up. Conclusion: Non-convulsive status epilepticus should be suspected in children with recent onset change in speech, memory, school performance, unexplained ataxia and particularly in children with epilepsy who undergo an otherwise inexplicable change in behavior.
Fifty cases of eating epilepsy (EE) are reported. Mastication of food produced seizures in 49 (98... more Fifty cases of eating epilepsy (EE) are reported. Mastication of food produced seizures in 49 (98%) and swallowing in 1 (2%). Complex partial seizure was found in 48 cases, the commonest form encountered (96%). EEG was abnormal in 15 cases (30%). The literature and possible mechanisms for seizure are discussed.
Abstract Kuthar valley in the Anantnag district of south Kashmir (North‐west India) was surveyed ... more Abstract Kuthar valley in the Anantnag district of south Kashmir (North‐west India) was surveyed to ascertain the prevalence of completed strokes. In a population of 63,645, the survey was from July to November 1986; 91 cases of completed stroke were detected giving a crude prevalence of 143/100,000. Only 10 (10.9%) cases (7W : 3M) were in the age group 15–39 years, a prevalence rate of 41/100000.
DOAJ (DOAJ: Directory of Open Access Journals), 1999
ABSTRACT Segmental spinal myoclonus (SSM) involving a child's lower limb revealed the pre... more ABSTRACT Segmental spinal myoclonus (SSM) involving a child's lower limb revealed the presence of a spinal cord tumour from T8 to T12. The clinical and electrophysiological features of SSM-during the child's development were studied. The presence of this disorder at two months of age did not disturb the development of walking, but did lead to orthopaedic complications. Four electromyographic recordings performed between 23 months and 7 years 10 months of age revealed the topographical extension of the SSM, its passage to rhythmicity, variable frequency and change in the sequence of activation of the muscles. These electroclinical features enable discussion of the pathophysiology of SSM by reference to neonatal motor automatisms and spinal generators.
Five children (3F:2M), in the age group 1 years to 11 years, with Munchausen syndrome by proxy ar... more Five children (3F:2M), in the age group 1 years to 11 years, with Munchausen syndrome by proxy are reported from the Sultanate of Oman. They were seen over a four years period from 1996-1999. In all these children, the mother came up with history of uncontrolled epilepsy. Carbamazepine was the most common antiepileptic drug used. One of these children remained hospitalized elsewhere for nearly 9 months, as a case of uncontrolled status epilepticus. It took 18 months to 6 years (mean 2.8 years) to establish the diagnosis and the mother was the offender in all. The main lead to diagnosis, was the disparity between history and clinical presentation to hospital. The carbamazepine levels were several times above the upper limit of therapeutic range. Munchausen syndrome by proxy very much exists here, but is possibly less recognized and needs immediate attention to formulate policies to identify and manage these children. It is necessary to create awareness even in the medical community, to recognise this problem. There is an urgent need to develop a child protection council at the national or regional level.
Dandy-Walker syndrome in monozygotic twins is reported. The twins reported, presented with delaye... more Dandy-Walker syndrome in monozygotic twins is reported. The twins reported, presented with delayed development, big head and dysmorphic features. In addition, there were significant cafe-au-lait spots on the trunk and other minor features consistent with the diagnosis of neurofibromatosis. To the best of our knowledge, Dandy-Walker syndrome in combination with neurofibromatosis in monozygotic twins has not been previously reported.
Journal of Neurosciences in Rural Practice, Nov 2, 2022
Seizures are not common in cases with chronic liver disease. Overall seizures have been reported ... more Seizures are not common in cases with chronic liver disease. Overall seizures have been reported in 20–30% of cases in chronic liver disease associated with hepatic encephalopathy. We report two cases of chronic liver disease patients who presented with new-onset refractory focal status epilepticus (SE). Both patients had encephalitis and seizures which responded only when acyclovir was added to the treatment with antiepileptic medication. Herpes encephalitis should be considered as a possible diagnosis in new-onset focal seizures or focal SE in patients with chronic liver disease with or without hepatic encephalopathy, pending further investigations.
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