Fatih Bayrakli

Gamma-knife radiosurgery in the treatment of trigeminal schwannomas

SummaryBackground. Trigeminal nerve schwannomas account for 0.07%–0.28% of all intracranial tumours. Advances in skull base surgery have led to more aggressive resection of these tumours, but surgery may associated with development of new neurological deficits.n Methods. In this report, we analyse the long-term results 15 patients with newly diagnosed or residual/recurrent trigeminal schwannoma who underwent gamma-knife treatment.n Findings. During a mean 61 months of follow-up, MRI revealed reduction of tumour size in 13 and no size change in 2 patients. The tumour growth control rate was 100% and only 1 patient had transient facial numbness and diplopia.n Conclusions. For patients with small to moderate size trigeminal schwannomas, gamma-knife radiosurgery is associated with good tumour control and a minimal risk of adverse radiation effects.

Surgical treatment of trigeminal schwannomas

Schwannomas that arise from the trigeminal nerve are rare, but this nerve is the second most frequent intracranial site of schwannoma occurrence next to the vestibular nerve. The advent of microsurgical techniques and skull-base approaches has greatly enhanced the surgical management of these tumors, and outcomes have improved markedly. This report documents 18 cases of histologically verified schwannomas that arose from the trigeminal nerve and were treated surgically in our clinic between January 1992 and July 2005. The patients were ten women and eight men of age 39.7xa0years (range, 22–62xa0years). The tumor was located in the middle fossa (type A) in five cases, in the middle and posterior fossae (type C) in nine cases, in the posterior fossa (type B) in two cases, and in the branches of the trigeminal nerve (type D) in two cases. Total excision was achieved in 17 cases, and there was no mortality in the series. Our results indicate that trigeminal schwannomas, regardless of type, can be removed via skull-base approaches. We present an algorithm for surgical management of trigeminal schwannomas based on our experience and information from the literature.

Deep brain stimulation as treatment for dystonic storm in pantothenate kinase-associated neurodegeneration syndrome: case report of a patient with homozygous C.628 2 T > G mutation of the PANK2 gene

Pantothenate kinase-associated neurodegeneration (PKAN) syndrome is an autosomal-recessive neurodegenerative disease that causes progressive generalized dystonia. Currently, the disorder remains pharmacologically intractable. Herein we report the first case in which deep brain stimulation helped to relieve dystonic storm in a patient with PKAN syndrome who had homozygous c.628 2xa0Tu2009>u2009G mutation of the PANK2 gene. A 10-year-old boy with PKAN disease presented with dystonic storm and was admitted to the emergency department. Examination revealed generalized dystonia and impaired breathing due to involvement of the respiratory muscles. The patient underwent surgery for bilateral globus pallidus internus deep brain stimulation. The patient showed marked response to treatment.

Neural tube defect family with recessive trait linked to chromosome 9q21.12-21.31.

PurposeMeningomyelocele is one of the most common and socioeconomically, psychologically, and physically debilitating neurodevelopmental diseases. A few chromosomal locus and genes have been identified as responsible for the disease; however, clear evidence still needs to be produced. This study aimed to show evidence of a strong genetic linkage in a novel chromosomal locus in a family with this neural tube defect.MethodsWe identified a neural tube defect family in eastern Turkey, where two of six offspring had operations due to thoracolumbar meningomyelocele. The parents were of a consanguineous marriage. We collected venous blood from six offspring of the family. Whole genome linkage analysis was performed in all offspring.ResultsA theoretical maximum logarithm of an odds score of 3.16 was identified on chromosome 9q21.12-21.31. This result shows a strong genetic linkage to this locus.ConclusionsOur results identified a novel chromosomal locus related to meningomyelocele and provide a base for further investigations toward the discovery of a new causative gene.

Intractable yawning caused by foramen magnum meningioma in a patient with neurofibromatosis type 2.

Sir, Yawning is a stereotyped event seen in all vertebrates. Neural networks in the brainstem, autonomic nervous system, hypothalamus, and limbic system may be involved in the physiology of yawning. Tumor-related yawning has rarely been reported.[1,2] We present a rare case of a patient with a meningioma at foramen magnum who presented with intractable yawning. After total removal of the tumor, the recurrent yawning resolved completely. Letters to Editor