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Nesrin Senbil

Publications and source records attributed to Nesrin Senbil.

At least 19 recordsLinked to original sources
PubMed2007

Progressive vacuolar myelopathy and leukoencephalopathy in childhood acute lymphoblastic leukemia and transient improvement with vitamin B12.

Vacuolar myelopathy (VM) in leukemia is rare. We report a boy with leukemia who developed isolated central nervous system (CNS) relapse during reinduction therapy. 5 months after cranial radiotherapy, he gradually developed quadriparesis. Magnetic resonance imaging revealed an intramedullary lesion which extended through the cervical spine. Serum vitamin B12, folic acid, cerebrospinal fluid methyl malonic acid were normal. Viral screening by ELISA was negative. He had lymphopenia, and reduced immunoglobulins, from a cardiac arrest. Biopsy revealed VM. He responded to weekly vitamin B12 treatment but on the 6th week of the therapy he died after developing periventricular, gliotic, hyperintense lesions in the brain.

Central Nervous System Neoplasms↗
PubMed2006

Subacute sclerosing panencephalitis: is there something different in the younger children?

Subacute sclerosing panencephalitis is a rare, slow viral infection caused by a defective measles virus. Although it is a rare disease, it is still important in developing countries. The onset is generally between the ages of 5-15 years. We reported the clinical and laboratory profile and nature of 9 patients under the age of 4 years with SSPE. Although it is known that a few patients with SSPE have an acute and rapidly fulminating course, in this study rate of progression was rapidly progressive in 6 patients and progressive in 3 of them on admission. Unfortunately, 4 of them were lost to follow up because of address and/or telephone number alterations. Although the number of patients in this study is not sufficient, we suggest that SSPE patients under the age of 4 years have a poor prognosis as a result of progressive or rapidly progressive course despite medical treatment.

Child, Preschool↗
PubMed2006

Fixation-off sensitivity and generalized epileptic EEG induced by eyes closed.

This report describes a female with eyelid fluttering with absence seizures, infrequent generalized tonic-clonic seizures, and mild mental retardation. Interictal and video-electroencephalography evaluations revealed normal activity while eyes were open but continuous generalized discharges with eyes closed (eyes closed induced abnormality), as well as fixation-off sensitivity. This patient is in the group of a pure and distinct clinical form of fixation-off sensitivity cryptogenic generalized epilepsy.

Child↗
PubMed2006

Nonconvulsive status epilepticus on electroencephalography in a case with subacute sclerosing panencephalitis.

Subacute sclerosing panencephalitis is a neurodegenerative disease with a poor prognosis. We report a case of a 5 1/2-year-old boy who had emotional lability, cognitive difficulties, and myoclonia after a mild closed head injury. The magnetic resonance image of the brain and computed tomographic scan of the head were normal. His electroencephalogram (EEG) showed continuous nonconvulsive status epilepticus activity, which could not be suppressed with intravenous diazepam. After treatment with phenytoin for 2 days, an EEG showed periodic high-amplitude sharp-and-slow-wave complexes, which were also not suppressed with intravenous diazepam. Since the patient had measles at 5 months of age, subacute sclerosing panencephalitis was considered, and the diagnosis was confirmed by the presence of measles antibodies in cerebrospinal fluid.

Anticonvulsants↗
PubMed2005

Serological follow-up in juvenile myasthenia: clinical and acetylcholine receptor antibody status of patients followed for at least 2 years.

Thirty cases of juvenile myasthenia with at least 2 years' follow-up and one measurement of acetylcholine receptor antibody titer were evaluated. Symptoms were initially ocular in 15 (50%) cases; only one developed generalized disease and others remained ocular in 2-24 years' follow-up. Antibodies to acetylcholine receptor (AchRAb) were absent in 12/30 (40%) of all patients initially, but became positive in 5/28 (18%) on further testing in 1-5 years. Spontaneous remission was observed in 6 cases (20%) of whom one became seropositive, and late relapses after 5-11 years' remission were observed in 3 patients with ocular myasthenia, of whom only one developed AchRAbs. Our results show ocular JM has an exceptional tendency to remain confined to these muscles, and delaying the classification of the disease as AchRAb-negative for a few years and repeating serological tests is advisable in the follow-up.

Adolescent↗
PubMed2005

Heart rate variability and autonomic dysfunction in SSPE.

Autonomic nervous system involvement in subacute sclerosing panencephalitis was studied in 29 patients by analysis of heart rate variability and compared with a control group which consisted of 20 age- and sex-matched healthy subjects. Holter recordings for 24 hours were obtained, and all recordings were analyzed using time-domain parameters. The patients with subacute sclerosing panencephalitis were found to have significantly lower values of standard deviation of all normal sinus intervals and triangular index when compared with the control group. Of 23 patients who had regular follow-up, 15 died in a period ranging from 1 to 13 months. Twelve of these patients had heart rate variability parameters lower than normal, but no significant difference was observed between the heart rate variability values of patients who survived or died. Also, there was no association between brain magnetic resonance imaging findings and heart rate variability indices. It is concluded that patients with subacute sclerosing panencephalitis have autonomic dysfunction; and this was thought to be mainly due to central involvement.

Autonomic Nervous System↗
PubMed2004

Subacute sclerosing panencephalitis: a cause of acute vision loss.

A 4-year-old male presented with only acute vision loss. His neurologic examination, funduscopic examination, and pupils were normal. Cranial magnetic resonance imaging revealed abnormal hyperintense, bilaterally symmetric lesions (on T(2)-weighted and fluid-attenuated inversion recovery, images) in bilateral optic radiations, pulvinar region in the thalami, crus posterior of internal capsules, periventricular white matter, and unilaterally left anterior pons. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Vision loss improved and cranial magnetic resonance imaging findings regressed,but myoclonic jerks and deterioration began 7 months later. The diagnosis of subacute sclerosing panencephalitis should be considered in cases with acute vision loss resulting from cortical blindness even when classical findings of the central nervous system do not exist.

Acute Disease↗
PubMed2004

Benign neonatal sleep myoclonus mimicking status epilepticus.

Benign neonatal sleep myoclonus is a self-limited movement disorder characterized by neonatal-onset myoclonic jerks only during sleep, abrupt and consistent cessation with arousal, and absence of concomitant electrographic changes suggestive of seizures. It has a good outcome and was included in the differential diagnosis of neonatal seizures. A presumed transient serotonin imbalance and genetic factors may play a role in the pathogenesis of this disorder. We report a case of benign neonatal sleep myoclonus mimicking status epilepticus in an infant with a family history of nocturnal myoclonus, tic disorder, and sleep disturbance. We suggest that this benign entity should be included in the differential diagnosis of status epilepticus during the newborn period.

Anesthesia, General↗
PubMed2004

Brown-Vialetto-van Laere syndrome; the first Turkish case.

We describe a 14-year-old female patient with progressive ponto-bulbar palsy and deafness. The first symptom was present at the age of 9 as a difficulty in walking and then she was stable with mild clumsy walking till 14 year-old. It was noticed that she had rapidly progression gait disorder, hearing loss, difficulty in swallowing and speaking in a period of 2.5 months. Clinically, there were bilateral facial weaknesss, atrophic tongue with fasciculations, poor gag reflex, deafness, axial and appendicular hypotonia, severe muscular weakness involving muscles of neck, shoulder, and upper arms, hands with thenar and hypothenar amyotrophy. Hearing loss was documented by brainstem auditory evoked potentials. Other laboratory investigations, screening tests and imaging studing were normal. These clinical features are consistent with the Brown-Vialetto-van Laere syndrome.

Adolescent↗
PubMed2003

Combined treatment with subcutaneous interferon-alpha, oral isoprinosine, and lamivudine for subacute sclerosing panencephalitis.

We compared patients with subacute sclerosing panencephalitis who received treatment according to our protocol for at least 6 months (19 patients) with the patients who could not receive any treatment (13 patients). The treatment protocol consisted of oral isoprinosine (100 mg/kg/day), subcutaneous interferon alpha-2a (10 mU/m2/three times a week), and oral lamivudine (10 mg/kg/day). There were no statistical differences between the two groups according to Neurological Deficit Index, clinical stage, and average age on admission and also on the final evaluation after treatment. The mortality rates of both groups were similar: 3 (15.7%) for the treatment group and 6 (46%) for controls. The remission rates for the treatment and control groups were 7 of 19 (36.8%) and 0 of 13 (0%), respectively, and the difference was statistically significant (P = .036). The mean survival period of the treatment group was significantly longer than that of the control group (P = .01). In conclusion, this combination treatment protocol resulted in higher remission rates and longer survival periods when compared with controls, as well as a remission rate that was better than the spontaneous remission rate of 5%. For this reason, and as well as because interferon-alpha therapy has an easier route of application and a higher family compliance, we have considered this an alternative protocol for patients with subacute sclerosing panencephalitis.

Administration, Oral↗
PubMed2003

Central pontine and extrapontine myelinolysis owing to disequilibrium syndrome.

Neurologic disorders can be seen in patients with end-stage renal failure owing to complications of hemodialysis or peritoneal dialysis. The disequilibrium syndrome can be seen, usually soon after or toward the end of dialysis. We report a patient with central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. The patient had depressed consciousness, agitation, tremor, stupor and hyperactive deep tendon reflexes toward the end of the second peritoneal dialysis. A brain computed tomographic (CT) scan showed hypodense lesions in pontine and extrapontine locations without radiocontrast medium enhancement After 2 days, the patient had only minimal memory deficits. A control brain CT scan 1 week later showed a decrease of the lesions in central pontine and extrapontine locations. Central pontine and extrapontine myelinolysis should be suspected and investigated in the acute neurologic disorders of dialysis patients.

Child↗
PubMed2002

Epileptic and non-epileptic cerebral palsy: EEG and cranial imaging findings.

The aims of the study were to compare the clinical types, electroencephalogram (EEG) and cranial magnetic resonance imaging/computed tomography findings of epileptic and non-epileptic cerebral palsy (CP) patients. Seventy-four patients with CP were evaluated in 2 years. Tetraplegic CP had a higher incidence of epilepsy (60.5%). EEG was confirmed abnormal in epileptic CP as 90.3%, and in non-epileptic CP as 39.5%. Focal epileptiform activity, generalized slowing, and multifocal epileptiform activity were significantly frequent in epileptic CP. There were cranial imaging abnormalities of 74.2% in epileptic and 48.8% in non-epileptic CP. Although there was not any statistically significant difference between the two groups, epileptic group revealed more structural abnormalities. Further studies concerning a possible risk of epilepsy development and its relations with the EEG and cranial imaging findings are needed in presenting the other risk factors involved and the factors affecting the CP prognosis.

Brain↗
PubMed2002

Cavanagh's syndrome (congenital thenar hypoplasia).

Thenar hypoplasia can be an isolated defect, as in Cavanagh's syndrome, can be present with cardiac (Holt-Oram syndrome) or eye (Okihiro's syndrome) disorders, or can be associated with hand anomaly, as in Haas's malformation. Vascular abnormality may be associated with thenar hypoplasia, which has been demonstrated in Okihiro's syndrome. Cavanagh's syndrome is a rare anomaly of the upper extremities that presents with unilateral or bilateral hypoplasia of the thenar eminence. Typical clinical, radiographic, and electrophysiologic findings emphasize the diagnosis. Differentiation from carpal tunnel syndrome is important to prevent unnecessary intervention. Electrophysiologic and radiographic findings are necessary tools for the physician to establish a correct diagnosis and make an appropriate referral. Because of its rarity, we present the case of an 8-year-old girl with this syndrome.

Child↗
PubMed2002

Effects of intranasal midazolam and rectal diazepam on acute convulsions in children: prospective randomized study.

In this study, the effects and side effects of rectal diazepam and intranasal midazolam were compared in the treatment of acute convulsions in children to develop a practical and safe treatment protocol. In the diazepam group, the seizures of 13 (60%) patients terminated in 10 minutes; however, 9 (40%) patients did not respond. In the midazolam group, 20 (87%) patients responded in 10 minutes, but 3 (13%) patients did not respond. Regarding the anticonvulsant effect, midazolam was found to be more effective than diazepam, and the difference was statistically significant (P < .05). The necessity of a second drug for the seizures that did not stop with the first drug was higher in the diazepam group than the midazolam group, and the difference was statistically significant (P < .05). We conclude that as an antiepileptic agent, intranasal midazolam is more effective than rectal diazepam. After administration, we did not observe any serious complications. Further investigations are necessary; however, intranasal administration is easy, so if the nasal drop and spray forms used in some European countries and the United States are available worldwide, it will be very useful for physicians in the emergency room.

Administration, Intranasal↗
PubMedDate not supplied

Effect of topiramate on enlargement of head in Canavan disease: a new option for treatment of megalencephaly.

Canavan disease (CD) is a rare autosomal recessive genetic disorder characterized by early onset progressive spongy degeneration of the brain involving the axon's myelin sheath. Patients with CD have leukoencephalopathy and megalencephaly; clinically they show a variable course ranging from slow neurodegenerative course to no neurological development or rapid regression. Current treatment is symptomatic including management of seizures and spasticity. Topiramate (TPM) is a novel antiepileptic drug for treatment of a broad spectrum of seizure types in adults and children. We used TPM in two of our patients diagnosed with CD at six months of age. At seven months and 15 months' follow-up, respectively, each patient showed a decrease in head growth velocity. We suggest that TPM can be used in patients with CD and possibly in other childhood neurodegenerative diseases with leukoencephalopathy and megalencephaly. Further studies are required to reveal the underlying mechanisms that lead to decreased head growth velocity, and to conclude whether this ameliorates the clinical course of CD.

Anticonvulsants↗