Neuro-Behçet is a rare disease but should be considered in all kinds of neurological findings, even in childhood.

OBJECTIVES: Behçet's disease (BD) is a vasculitis characterised by eye, musculoskeletal, neurological and gastrointestinal involvement, in addition to recurrent oral ulcers. Neuro-Behçet is the term used to define the nervous system involvement in BD and is very rarely seen in childhood. This study aims to show that neuro-Behçet can manifest a clinical course involving all kinds of neurologic findings in the paediatric population. METHODS: The Clinic of Paediatric Neurology at Uludag University provides tertiary treatment for children up to eighteen years of age in Bursa, Turkey. Five patients who were clinically diagnosed with Neuro-Behçet in the last 5 years were included in the study. RESULTS: Seizure, myopathy, transverse myelitis, polyneuropathy, venous thrombosis and facial nerve paralysis were respectively seen in the patients. CONCLUSIONS: Neuro-Behçet is rare in children, but it is important to know that it can cause various neurological findings, and also systemic findings should be taken into consideration in the diagnosis of neurological diseases. Studies on the neurological involvement of BD in children are inadequate. We believe that paediatric neurologists should be more aware of the neuro-Behçet condition.

Facial colliculus syndrome due to a Herpes simplex virus infection following Herpes labialis.

BACKGROUND: The facial colliculus is an elevated area that is formed by fibers from the motor nucleus of the 7`th cranial nerve as they loop over the abducens nucleus. Clinical signs and symptoms of facial colliculus lesions occur primarily due to injury to the abducens nerve nucleus, the facial nerve fibers around the abducens nucleus, paramedian pontine reticular formation, and the medial longitudinal fasciculus. The etiology of facial colliculus lesions varies by age. While tumors, demyelinating lesions, and viral infections can be involved in young individuals` etiology, vascular ischemia is a common causative factor in older people. CASE: In this paper, we present a case of facial colliculus syndrome due to its rare occurrence in a young patient; who developed the signs and symptoms after a herpes infection. CONCLUSION: Facial colliculus syndrome is rare and the treatment is based on etiology.

A rare case of juvenile amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a chronic motor neuron disease characterised by progressive weakness in striated muscles resulting from the destruction of neuronal cells. The term juvenile ALS (JALS) is used for patients whose symptoms start before 25 years of age. JALS may be sporadic or familial. CASE: Here, we present a sporadic case of JALS because of its rarity in children. The heterozygous p.Pro525Leu (c.1574C > T) variation was identified in the fused in sarcoma (FUS) gene. CONCLUSION: The p.Pro525Leu mutation in the FUS gene has been detected in patients with ALS, characterised by early onset and a severely progressive course.

Evaluation of Magnetic Resonance (MR) Findings in Patients with Refractory Epilepsy.

OBJECTIVES: Epilepsy is characterized as a tendency towards recurrent seizures and it is a significant health problem in the world and one of the most common severe neurologic disorders among children. This study aims to evaluate the outcome of magnetic resonance imaging in determining the etiology in patients with refractory epilepsy and to reveal pathologies that may have the potential to be treated with methods, such as epileptic surgery. METHODS: Data were obtained from the patient files of the patients diagnosed with epilepsy and monitored for at least two years between 01.01.2009-12.31.2012 in the Uludag Faculty of Medicine, the Division of the Pediatric Neurology. File records of the patients, age, sex and MRI findings of the patients were recorded. RESULTS: One hundred twenty were girls (49%) and 125 were male (51%) of the cases. The age range ranged from 1 to 18 years and the median value was 8.3 (1-18) years. One hundred twenty of the 245 patients who met the diagnostic criteria for resistant epilepsy was found as well controlled. In patients with resistant epilepsy, the findings of these two groups of patients were compared concerning MR findings. Among all patients, 154 (62.8%) patients were found to have MR pathology. Of these patients, 83 (53.9%) were in the resistant group and 71 (46.1%) were in the well-controlled group. There was no significant difference in the presence of MR findings between the two groups (p=0.354). The highest incidence (24.8%) of the encephalomalacia in patients in the resistant group may explain the association of perinatal hypoxia with resistance development. CONCLUSION: If patients with epilepsy can be predicted early in the disease, which group of the patients will not respond well to medical treatment; unlike other patients, different treatment modalities, such as antiepileptic use, vagal nerve stimulation, ketogenic diet and epilepsy surgery, can be applied to this group of the patients. We think that clinicians can guide the planning of treatment of the MR findings.