Olcay Ünver

Pallidal deep brain stimulation in a 5-year-old child with dystonic storm: case report.

A 5-year-old child had a medical history of epilepsy and a newly presented mental retardation with a life-threatening dystonic storm. Neuroimagings showed bilateral calcification of the pallidum. Several treatment modalities were performed, but the symptoms showed no significant improvement. The patient was operated on in order to place a deep brain stimulation (DBS) targeting bilateral globus pallidum internus (GPi). The dystonia showed a remarkable improvement after surgery, with 81% reduction of dystonia severity after 15 months. To our best knowledge, this is the youngest patient mentioned in the literature to be treated with DBS, which was also life-saving in this case.

MRI findings of isoniazid-induced central nervous system toxicity in a child

A 12-year-old female patient was admitted to the pedi-atrics clinic of our hospital with the complaint of failure tothrive. A physical examination showed nothing excepta purified protein derivative (PPD) test scar. Two formerPPD tests that were undertaken 2 months apart were bothpositive (20 mm and 23 mm indurations). A computedtomography (CT) examination was undertaken to investi-gate for lung parenchymal infiltration, but showed noabnormal findings. Sputum and gastric lavage specimenswere collected for culture, and although TB culture wasnegative, 10 mg/kg/day INH and 25 mg/day pyridoxinemedication was started as prophylaxis.Three months after starting prophylactic INH treatment,the patient was referred the pediatric neurology clinic ofour hospital with the complaints of hand tremors anddizziness. A neurological examination revealed intentionaltremor, ataxia, dysdiadokynesia, and dysmetria, andapositiveRombergtest.Noabnormalreflexesornystagmuswere observed. Sensory and cranial nerve examinationswere also normal. Deep tendon reflexes and musclestrength of the upper and lower extremities were alsonormal. A complete blood count and biochemical studiesrevealed no abnormal finding. Serum B12 levels, theerythrocyte sedimentation rate, and thyroid function testswere all within the normal range.Cranial MRI showed bilateral and symmetrical increasedsignal intensity at both cerebellar dentate nuclei andbetween the thalamus and mesencephalic areas on T2-weighted and fluid-attenuated inversion recovery (FLAIR)imaging. Abnormal contrast enhancement was not seen inthe described areas. On diffusion-weighted MRI images,diffusion restriction was present in those areas described(Fig 1). MR angiography, MR venography, and MR spec-troscopy did not show any abnormal findings. After findingthat the blood INH level was 16

Evaluation of cases with cerebral thrombosis in children

AIM We aimed to evaluate the patients who were followed up in our clinic with a diagnosis of cerebral sinovenous thrombosis in terms of age, sex, clinical findings, etiology, thrombophilic factors, imaging findings, treatment and prognosis. MATERIAL AND METHODS The files of 11 patients who were followed up in our pediatric neurology clinic with a diagnosis of cerebral thrombosis between 1 December 2010 and 31 December 2014 were retrospectively analyzed. RESULTS Seven of 11 patients were male (63.6%). The median age was 14 years (2-17 years). Six (54%) of the patients presented with a complaint of headache. Other complaints at presentation included diplopia (n:3), weakness and difficulty in speaking (n:1) and seizure (n:1). A diagnosis of pseudotumor cerebri was made in eight of the patients (72.7%). In the etiology, mastoiditis was found in three patients, mastoiditis and meningitis were found in combination in one patient, Behçets disease was found in three patients and head trauma was found in one patient. In 3 patients, only prothrombotic genetic risk factors were present; one patient had deficiency of protein C and S, one patient had deficiency of antithrombin III and one patient had hyperhomosisteinemia in association with vitamin B12 deficiency. 1A homozygous MTFHR A1298C mutation was detected in the patient who had mastoiditis and meningitis and protein S deficiency and lupus anticoagulant were found in another patient who had mastoiditis. All patients received anticoagulant treatment and all patients recovered without neurological sequelae except one. CONCLUSIONS Cerebral sinovenous thrombosis should be considered in patients who present with headache and focal neurological deficits. Appropriate utilization of imaging studies is necessary for the diagnosis. Detailed ear, nose and throat examination should be performed to detect mastoiditis. It is recommended that genetic risk factors should be investigated, because hereditary thrombophilis factors may have a role in children. Behçets disease which is relatively common in our country should be considered in differential diagnosis.

The Epidemiology of Epilepsy in Children A Report From a Turkish Pediatric Neurology Clinic

This retrospective cohort study aims to assess the distribution of seizure types and epileptic syndromes in children with epilepsy who were followed up in a tertiary outpatient pediatric neurology clinic between January 2004 and December 2009. The findings of 533 children aged between 2 months and 16 years were evaluated. The International League Against Epilepsy criteria (of 1981 and 1989) were used for diagnosis and classification. The rate of partial seizures (56.5%) was higher than that of generalized seizures (43.5%). Partial seizures were more common during late childhood (P < .001). Localization-related epilepsies (53.3%) were more frequent than generalized epilepsies (37.1%). Generalized epilepsies were more frequent during the first year of life, whereas localization-related epilepsies were more common at later ages (P < .001). The majority had a symptomatic etiology (47.1%). The increased frequency of symptomatic etiologies attributed to perinatal insults suggests that intractable epilepsies during childhood represent an important health issue for developing countries.

P104 – 2363: Transient splenial lesion of the corpus callosum related to migraine with aura in a pediatric patient

Objective Transient splenial lesions of the corpus callosum are described in association with many diverse clinical conditions including various infections, use or withdrawal of antiepileptic drugs, and hypoglycemia. The lesion appears as a well-defined hyperintense ovoid lesion in the center of the splenium of the corpus callosum best observed in diffusion weighted brain magnetic resonance images. Generally, the condition is associated with a mild clinical course and a favorable prognosis. We present a pediatric case with a transient splenial lesion of the corpus callosum related to migraine with aura. Methods A 13-year old girl was admitted to our emergency room with a severe headache, confusion, violent behavior, slurred speech, and right-sided hemiparesis. She first experienced a similar but milder attack with numbness in the face and lips not complicated with hemiparesis, 3 weeks before. At the time, her brain magnetic resonance imaging (MRI), electroencephalography (EEG) were unremarkable. After 3 more attacks the diagnosis of migraine with aura was made 1 week before. Her brain MRI conducted on admission revealed well-circumscribed hyperintense ovoid lesion in the splenium of corpus callosum in diffusion-weighted images. Her EEG revealed generalized, high amplitude intermittent slow delta waves prominent on frontal areas, which is consistent with encephalopathy. Her follow-up EEG on day 3 showed significant improvement. She was discharged 4 days after admission with a completely normal neurological examination. Her follow-up MRI 3 weeks after the first MRI, revealed a complete disappearance of the lesion. Results To our knowledge our patient is the first pediatric case of transient splenial lesion of the corpus callosum related to migraine with aura presenting with encephalopathy. Conclusions Transient lesion of splenium is caused by a heterogenous etiology and a mild course and a good prognosis might be expected in the presence of a splenial lesion in the corpus callosum.

PP15.9 – 2371: Pseudotumor cerebri: A single center experience in 24 patients

Objective Pseudotumor cerebri is a syndrome characterized by signs and symptoms of high intracranial pressure without radiological evidence of intracranial mass. Our aim is to present data of patients diagnosed with pseudotumor cerebri in our department. Methods We analyzed the epidemiology, etiology and follow-up of 24 consecutive patients. Results Twenty-four patients were included in the study. There were 14 girls (58.3%), with an average age at presentation of 10.2±3.9 years (3–17 years). The major symptom at presentation was headache (84%, n=21). Two patients (8%) presented with focal convulsion only and six patients (25%) had unilateral abducens paralysis at presentation. Five of these patients (20%) had other complaints like blurred vision, vomiting or convulsion in addition to headache. When classified according to etiology, 10 patients (41.7%) were overweighted. Nine of these patients were prepubertal girls. Sinus vein thrombosis was detected in five patients (20.8%) and two of these patients were males diagnosed with Behcets disease. Etiology was unknown in nine patients, one of whom was diagnosed with Coffin Siris Syndrome. The average number of lumber puncture (LP) performed per patient was two (range 1–10). The average time between two LPs was 4.1 months (0.3–10 m). Acetozolamide was used in all of the patients. Four patients (16.7%) underwent ventriculoperitoneal shunt procedure. Mean follow up time was 10.3 months (0.5–36 m). Mean time to resolution of symptoms was 90 days (7–300 d). Patients who had surgical operation had complete recovery. Patients under medical treatment had exacerbations and resolution periods. No patient had permanent visual loss. Conclusion With increased awareness, patients with complaints of headache or the other symptoms of increased intracranial pressure are more commonly referred to pediatric neurology clinics.

PP08.7 – 2364: Atypical Guillain Barré syndrome with preserved deep tendon reflexes: report of three cases

Objective Guillain Barre syndrome is a postinfectious polyneuropathy involving mainly motor but sometimes also sensory and autonomic nerves. Guillain Barre syndrome is now the worlds most common cause of acute neuromuscular paralysis. Methods We present here 3 patients with preserved deep tendon reflexes. Case 1: An 11 year-old girl was admitted with a complaint of weakness confined to the lower extremities and in a lesser extent to the upper extremities over 4 days. On physical examination muscle strength was 2/5 on lower extremities and 4/5 in upper extremities. Case 2: A 17-year-old boy with known spondylarthritis presented with complaints of weakness of lower extremities over 10 days. On physical system examination muscle strength was 4/5 on lower extremities and 5/5 on upper extremities. Case 3: A 9-year-old boy was admitted with weakness and pain in the lower extremities over 10 days. His CK was 2988 (150–499 U/L). He was diagnosed as myositis and painkillers were prescribed. Then the weakness gradually worsened involving upper extremities. On physical system examination muscle strength was 4/5 on lower extremities and 2/5 on upper extremities. Results The deep tendon reflexes were normal in upper and lower extremities on repeated examinations in all of 3 cases. The diagnosis of Guillain Barre Syndrome was made by electrophysiological studies. Conclusion Although hypo- or areflexia is necessary for clinical diagnosis of GBS, preserved deep tendon reflexes do not exclude the diagnosis of Guillain-Barre syndrome. The electrophysiological studies play a very important role in differentiating it from other causes.

P44 – 2863: Clinical and radiological factors associated with later occurence of epilepsy and epileptic encephalopathy after hypoxic ischemic insult

Objective Epileptic encephalopathy is a devastating type of epilepsy in early life causing severe cognitive and behavioral impairments. Epilepsy and epileptic encephalopathies may be complications of hypoxic ischemic encephalopathy during the neonatal period. Methods In this retrospective study 60 patients who suffered hypoxic ischemic encephalopathy in the neonatal period; aged 6.75±5.34 (0.30–12.09) years, 28 girls (46.7%) and 32 boys (53.3%) were invastigated to describe clinical and radiological factors associated with later occurence of epilepsy and epileptic encephalopathy were divided in 3 groups. Group 1: Patients with epileptic encephalopathy (n: 27 (%45) Group 2: Patients with epilepsy without encephalopathy (n: 18 (%30). Group 3: Patient without epilepsy and epileptic encephalopathy (n:15 (%25). Groups were compared for prenatal, natal and postnatal insults, neonatal seizures, cranial USG, electroencephalography (EEG) in neonatal period and cranial Magnetic Resonance Imagination (MRI). Results Gestational ages and birth weights of group 3 were lower then Group 1 and Group 2 (p Conclusion Higher asphyxia grades, neonatal menengitis and sepsis, cortical impairment in cranial MRI, seizures in the neonatal period are high risk factors for evaluation of epilepsy and epileptic encephalopathy in children suffered from HIE in neonatal period.

P19.15 Acute necrotizing encephalopathy of childhood: a turkish case

Acute necrotizing encephalopathy of childhood is a rare form of acute encephalopathy of unknown etiology characterized by typical symmetrical lesions in the thalami, with variable involvement of the white matter, brainstem and cerebellum. Clinically there is a rapid neurologic deterioration after a short period of a nonspecific viral-like illness associated with gastrointestinal or respiratory signs. Asian children are especially affected. Here we present a 3-yearold boy admitted to our hospital with fever and deterioration of consciousness. His nasopharyngeal swab for polymerase chain reaction revealed positive results for human parainfluenza 2 virüs. The diagnosis of acute necrotizing encephalopathy was made by cranial magnetic resonance imaging findings with symmetrical expansile hyperintense signals in bilateral thalami.