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ABSTRACT: Objective: Pseudotumor cerebri (PTC) is a clinical condition characterized by signs and symptoms of increased intracranial pressure, such as headache and papilledema. Our aim was to investigate the etiological and clinical features of pseudotumor cerebri (PTC) in children. Materials and method: We performed a comprehensive analysis of epidemiology, diagnostic work-up, therapy, and clinical follow-up in 42 consecutive patients. Results: Totally 42 patients diagnosed with PTC [27 (64.3%) females and 15 (35.7%) males] were included in the study. The average age of the symptoms onset was 10.79±3.43years (range from 12months to 17years). Obesity was found in eleven (26.2%) of them. Two of the patients had familial mediterranean fever, two of them had posttraumatic PTC. The following diseases were one patient, respectively; mycophenolate mofetil-induced PTC, hypervitaminosis A induced PTC, corticosteroid induced withdrawal due to nephritic syndrome, use of oral contraceptives, Guillain-Barre syndrome, urinary tract infection, varicella-zoster virus infection and dural venous sinus thrombosis associated with otitis media. The most common symptom was headache, recorded in 76.2% of the patients. All patients were treated medically. Three patients in our group also required a ventriculoperitoneal shunt. Conclusion: Pseudotumor cerebri is an avoidable cause of visual loss, both in adults and children. Pre-pubertal obese girls are more common. Medical therapy appeared to be successful in treating pediatric PTC in most patients. Nevertheless, despite adequate treatment, children can rarely experience loss of visual field and acuity; thus, prompt diagnosis and management are important.
Brain & development 09/2012; · 1.74 Impact Factor
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ABSTRACT: Deficiency of vitamin K predisposes to early, classic or late hemorrhagic disease of the newborn (HDN); of which late HDN may be associated with serious and life-threatening intracranial hemorrhage. Late HDN is characterized intracranial bleeding in infants aged 1 week to 6 months due to severe vitamin K deficiency. Late HDN is still an important cause of mortality and morbidity in developing countries where vitamin K prophylaxis is not routinely practiced. Children with cholestatic liver disease are at risk for developing secondary vitamin K deficiency because of fat malabsorbtion and inadequate dietary intake. In this study, we described 11 infants with cholestatic liver disease with different etiologies exhibiting intracranial hemorrhage (ICH). Six patients underwent surgical evacuation of ICH, following the administration of vitamin K and/or fresh frozen plasma. The possibility of cholestatic liver disease should be considered in the treatment of ICH due to vitamin K deficiency.
Neurological Sciences 02/2012; · 1.32 Impact Factor
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ABSTRACT: When electroencephalogram (EEG) activity is recorded for diagnostic purposes, the effects of sedative drugs on EEG activity should be minimal. This study compares the sedative efficacy and EEG effects of dexmedetomidine and midazolam.
EEG recordings of 60 pediatric subjects with a history of simple febrile convulsions were performed during physiologic sleep. All of these patients required sedation to obtain follow-up (control) EEGs. Subjects in Group D received 0.5 μg·kg(-1) of dexmedetomidine, and those in Group M received 0.1 mg·kg(-1) of midazolam. For rescue sedation, the same doses were repeated to maintain a Ramsey sedation score level of between 4 and 6.
The mean doses that were required for sedation were 0.76 μg·kg(-1) of dexmedetomidine and 0.38 mg·kg(-1) of midazolam. Diastolic blood pressure and HR were lower in Group D than in Group M (P < 0.05). Hypoxia was observed in 11 (36.7%) subjects in Group M and none in Group D; this was statistically significant (P < 0.001). Frontal and parieto-occipital (PO) EEG frequencies were similar during physiologic sleep and dexmedetomidine sedation. However, EEG frequencies in these areas (P < 0.001) and PO EEG amplitude (P = 0.030) were greater during midazolam sedation than during physiologic sleep.
Dexmedetomidine is a suitable agent to provide sedation for EEG recording in children. There is less change in EEG peak frequency and amplitude after dexmedetomidine than after midazolam sedation.
Pediatric Anesthesia 04/2011; 21(4):373-8. · 2.10 Impact Factor
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ABSTRACT: Molybdenum cofactor (MoCo) deficiency is a rare autosomal recessive inherited metabolic disorder resulting in the combined deficiency of aldehyde oxidase, xanthine dehydrogenase, and sulfite oxidase. We report a male infant with MoCo deficiency whose clinical findings consisted of microcephaly, intractable seizures soon after birth, feeding difficulties, and developmental delay. Sequencing of MOCS1, MOCS2, and GEPH genes, and single nucleotide polymorphism genotyping array analysis showed, to our knowledge, unusual inheritance of MoCo deficiency/maternal uniparental isodisomy for the first time in the literature. At 10 months of age, he now has microcephaly and developmental delay, and his seizures are controlled with phenobarbital, clonozepam, and vigabatrin therapy.
Developmental Medicine & Child Neurology 09/2010; 52(9):868-72. · 2.92 Impact Factor
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ABSTRACT: Reversible posterior leukoencephalopathy syndrome (RPLS) is recently described disorder with typical radiological findings in the posterior regions of the cerebral hemisphere and cerebellum. Its clinical symptoms include headache, decreased alertness, mental abnormalities, such as confusion, diminished spontaneity of speech, and changed behavior ranging from drowsiness to stupor, seizures, vomiting and abnormalities of visual perception like cortical blindness. RPLS is caused by various heterogeneous factors, the commonest being hypertension, followed by non-hypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. We presented nine patients with RPLS who had primary diagnoses such as acute post-streptococcal glomerulonephritis, idiopathic hypertension, the performing of intravenous immunoglobulin for infection with crescentic glomerulonephritis, erythrocyte transfusion for severe iron deficiency, L: -asparaginase treatment for acute lymphoblastic leukemia and performing of granulocyte-colony stimulating factor for ulcerative colitis due to neutropenia. Early recognition of RPLS as complication during different diseases and therapy in childhood may facilitate precise diagnosis and appropriate treatment.
Neurological Sciences 10/2009; 31(2):125-31. · 1.32 Impact Factor
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ABSTRACT: Herpes simplex virus infection of the central nervous system is still a significant cause of morbidity and often mortality. Early diagnosis is critical because treatment with the antiviral drug acyclovir dramatically decreases morbidity and mortality in children. The use of polymerase chain reaction techniques is a diagnostic procedure in herpes simplex virus infection, but negative polymerase chain reaction test results of cerebrospinal fluid herpes simplex virus can occur within the first 72 hours of illness, with subsequent test results becoming positive as in this case. Therefore, diffusion-weighted magnetic resonance may play a major role as a first and early diagnostic step.
Pediatric emergency care 07/2008; 24(6):377-9. · 0.92 Impact Factor
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ABSTRACT: Pasteurella is a Gram-negative coccobacillus found in 70-90% of oral cavities of cats, and as well, is isolated from the digestive systems of dogs, rats, rabbits, monkeys, and other animals. Pasteurella multocida has been known to cause infections in humans, the most familiar being soft tissue infection after animal bites. However, this organism may affect a variety of systems, causing serious disease. Pasteurella multocida can cause septic arthritis, osteomyelitis, pneumonia, endocarditis, meningitis, and septicemia. We report a case of bacterial meningitis, subgaleal, subdural, and epidural empyema due to Pasteurella multocida by a rabbit licking that resulted in neurological complications and a prolonged recovery period.
Journal of Emergency Medicine 06/2008; 39(1):35-8. · 1.31 Impact Factor
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ABSTRACT: Echinococcosis is a parasitic disease caused by infestation of various body tissues by the encysted larvae of Echinococcosis granulosa, the tapeworm. The disease because of the inefficiency of environmental health and protective care and dealing with animals is prevalent in South America, Africa, Australia, the Mediterranean countries and the Middle East. Cerebral hydatid cysts are rare and comprise only 0.5-3% of all reported hydatid cysts. Brain involvement is seen rare and primary form is very rare. We report five cases of primary hydatid disease of the brain. In three cases (60%), brain involvement was solitary, while two cases (40%) had primary multiple intracranial hydatid cysts. This study confirms that hydatid disease is a difficult health problem in Turkey.
Brain & development 06/2008; 31(3):228-33. · 1.74 Impact Factor
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ABSTRACT: Eosinophilic granuloma (EG) is a benign and solitary bony lesion of unknown etiology, which typically affects children: 90% of patients are between the ages of 5 and 15 years. EG, a variant of histiocytosis X, is a rare disease causing destructive bony lesions of the cervical spine in children. This report describes the case of a 5-year-old boy with torticollis who presented to the Emergency Department and was found to have EG of C6. He was successfully treated with anterior cervical corpectomy and fusion by using anterior plate and screws, with resolution of his symptoms.
Journal of Emergency Medicine 04/2008; 35(4):389-92. · 1.31 Impact Factor
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ABSTRACT: The combination of axial spasms in clusters, hypsarrhythmia, and psychomotor delay beginning in the first year of life defines West syndrome. Adrenocorticotrophic hormone remains the choice of treatment for many neurologists. Recent controlled studies support vigabatrin as first-line therapy, and open-label studies suggest that topiramate, lamotrigine, and zonisamide may be useful in treating spasms. Studies regarding the efficacy and safety of such treatments often come from small, uncontrolled trials and are often inconclusive. Levetiracetam is effective for treating localization-related epilepsy, but it is uncertain whether it is effective for treating West syndrome. To evaluate the efficacy of levetiracetam monotherapy in newly diagnosed cryptogenic West syndrome, levetiracetam was used initially in the treatment of five patients with cryptogenic West syndrome. On admission, levetiracetam (30 mg/kg) tablets were crushed and administered via nasogastric tube. Two patients were seizure free, two patients experienced a 50% reduction in seizure frequency, and one patient had no improvement in seizure frequency. There were no relapses in the two patients at 6 months after the cessation of seizures. It appears that levetiracetam may be effective in the initial treatment of selected patients with cryptogenic West syndrome.
Pediatric Neurology 12/2007; 37(5):350-3. · 1.52 Impact Factor
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Pediatrics International 11/2007; 49(5):672-3. · 0.63 Impact Factor
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ABSTRACT: The molybdenum cofactor is essential for the function of sulphite oxidase, xanthine dehydrogenase, and aldehyde oxidase enzymes. Molybdenum cofactor deficiency (MoCD) is a fatal disease resulting in severe neurological damage and death in early childhood. MoCD is an autosomal recessive condition which may mimic ischaemic encephalopathy. Although milder cases with later onset and less severe symptoms have been identified, the classic presentation involves neonatal seizures, progressive encephalopathy and death at an early age. There is currently no effective therapy, and the prognosis is poor. The disorder should be considered in all cases of intractable seizures in the newborn period and infants with clinical and radiological features of ischaemic encephalopathy, especially when no obvious lesion is detected. Blood uric acid measurement should be included in the battery of tests to be performed in all neonates' refractory seizures. We reported here an infant with MoCD who presented with hypoxic ischaemic encephalopathy and identified a novel mutation, c.130C>T in cDNA of the MOCS2 gene from the infant.
Brain and Development 07/2007; 29(6):365-8. · 2.12 Impact Factor
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ABSTRACT: Intraspinal epidermoid tumors can be congenital and acquired. Acquired intraspinal epidermoid tumors are extremely rare. Epidermal elements are implanted into the arachnoid space by trauma, spinal anesthesia, surgery, bullet wounds, myelography, or lumbar puncture. Approximately 40% of acquired epidermoid tumors are considered a late complication of lumbar puncture. The authors report the case of an 8-year-old boy who presented with a 1-year history of back and hip pain and radiating pain to both thighs posterior. Lumbar puncture was performed in the neonatal intensive care unit to rule out meningitis in the patient's past medical history. The patient underwent total surgical excision of the epidermoid tumor. Pathologic examination revealed the diagnosis of epidermoid tumor. Keywords: intraspinal epidermoid tumor; lumbar puncture; children.
Journal of Child Neurology 04/2007; 22(3):332-6. · 1.75 Impact Factor
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ABSTRACT: The fast ripening of fruits means they may contain various harmful properties. A commonly used agent in the ripening process is calcium carbide, a material most commonly used for welding purposes. Calcium carbide treatment of food is extremely hazardous because it contains traces of arsenic and phosphorous. Once dissolved in water, the carbide produces acetylene gas. Acetylene gas may affect the neurological system by inducing prolonged hypoxia. The findings are headache, dizziness, mood disturbances, sleepiness, mental confusion, memory loss, cerebral edema and seizures. We report the case of a previously healthy 5 year-old girl with no chronic disease history who was transferred to our Emergency Department with an 8-h history of coma and delirium. A careful history from her father revealed that the patient ate unripe dates treated with calcium carbide.
Journal of Emergency Medicine 03/2007; 32(2):179-80. · 1.31 Impact Factor
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American Journal of Emergency Medicine 02/2007; 25(1):87-9. · 1.98 Impact Factor
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ABSTRACT: Head trauma is a frequent cause of mortality and morbidity in the pediatric population. Chronic subdural hematoma is the most common traumatic lesion in head injury. Chronic subdural hematomas are rare in children older than 2 years old; they are more frequent during adolescence. Calcified or ossified chronic subdural hematoma is a rare entity that usually presents as a space-occupying lesion over the cerebral convexity. Chronic calcified subdural hematomas are reported less than chronic subdural hematomas. In this article, we report a successfully treated patient with surgical removal case of calcified chronic subdural hematoma mimicking calvarial mass.
Brain and Development 11/2006; 28(9):607-9. · 2.12 Impact Factor
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ABSTRACT: Torticollis is either congenital or acquired in childhood. Acquired torticollis is not a diagnosis but rather a sign of an underlying disorder. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders and may be a symptom of significant abnormalities of the spinal cord and brain, such as spinal syrinx or central nervous system neoplasia. Torticollis is rarely considered to be an initial clinical presentation of posterior fossa and cervical spinal cord tumors. We report five cases of pediatric tumors with torticollis at the onset: an astrocytoma originating from the medulla oblongata, another presumptive astrocytoma of the spinal cord located between C1 and C6 cervical vertebrae (not operated), an ependymoma located throughout the whole cervical spinal cord extending into the bulbomedullary junction, an astrocytoma originating from the bulbus and extending into the posterior fossa, and another case of a eosinophilic granuloma located extradurally through the anterior and posterior portions of the vertebral bodies from C3 to C7 producing the collapse of the sixth cervical vertebra. All five cases were seen in children, aged between 3 and 12 years. All these cases reflect the misinterpretation of this neurological sign and the lack of association with the possibility of spinal or posterior fossa tumor. This delay in the diagnosis of these diseases led to progressive neurological deterioration and to the increase in the tumor size, which made surgical intervention difficult and the prognosis unfavorable. Although torticollis secondary to tumors is rarely seen, it is necessary to be kept in mind in the differential diagnosis.
Neurosurgical Review 11/2006; 29(4):333-8; discussion 338. · 2.04 Impact Factor
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ABSTRACT: Deficiency of vitamin K predisposes to early, classic or late hemorrhagic disease of the newborn (HDN); late HDN may be associated with serious and life-threatening intracranial hemorrhage. Late HDN is characterized by intracranial bleeding in infants aged 1 week to 6 months due to severe vitamin K deficiency, occurring particularly in exclusively breastfed infants. Late HDN is still an important cause of mortality and morbidity in developing countries where vitamin K prophylaxis is not routinely practiced. In this study, we report on two siblings with intracranial bleeding who were fully breastfed without a routine supplementation of vitamin K. Vitamin K should be given to all newborns as a single, intramuscular dose of 1 mg.
Journal of Emergency Medicine 08/2006; 31(1):49-52. · 1.31 Impact Factor
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American Journal of Emergency Medicine 12/2004; 22(7):626-8. · 1.98 Impact Factor
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ABSTRACT: To review the MR imaging findings of multicystic encephalomalacia and to investigate the correlation between MR imaging and clinical findings.
Twenty-one patients who presented with convulsion, mental-motor retardation and microcephaly and had evidence of multicystic encephalomalacia on MR images were included in this study. MR imaging patterns and clinical findings were reviewed. Consequently, we correlated MR imaging findings and clinical outcome.
All patients had cortical thinning, white matter destruction, atrophy and gliosis. Tetraplegia was seen in 17 out of 19 patients with mixed type cerebral palsy in two patients with diffuse or symmetric involvement on MR imaging. Both of the patients with mixed type cerebral palsy had basal ganglia involvement on MR imaging. Hemiplegia was seen in two patients with asymmetric involvement on MR imaging. Microcephaly was seen in 17 patients with diffuse or symmetrical, and in one patient with asymmetrical, involvement. Microcephaly and tetraplegia was seen in all patients with cerebellar and basal ganglion involvement.
Microcephaly and spastic tetraplegia were developed mostly in patients with diffuse involvement, whereas hemiplegia was seen in patients with asymmetric involvement. The clinical outcome was worse in patients with cerebellar and brainstem involvement. Therefore, we supposed that the symmetry of lesions and cerebellar or brainstem involvement might be used as a prognostic indicator.
Tanısal ve girişimsel radyoloji: Tıbbi Görüntüleme ve Girişimsel Radyoloji Derneği yayın organı 04/2004; 10(1):8-13.
