Neurodegeneration with brain iron accumulation (NBIA) comprises a clinically and genetically heterogeneous group of neurodegenerative diseases characterized by progressive degeneration of the central nervous system and high basal ganglia iron deposition. The list of identified causative genes for NBIA syndromes continues to expand and includes one autosomal dominant, one X-linked, and a number of recessive forms. Mitochondrial membrane protein-associated neurodegeneration is a recently described NBIA syndrome caused by C19orf12 mutations. In this study, we report two consanguineous families with a homozygous C19orf12 p.Thr11Met mutation. Our patients presented at a later age and had more rapid disease progression, leading to early death in two, than those previously reported. We conclude that C19orf12 mutation is associated with wide phenotypic heterogeneity, and that further research is needed to examine the role of C19orf12 in NBIA and related diseases and to elucidate its protein function as well as other factors that may affect disease progression and expression.

Although the International Classification of Headache Disorders, 2nd version, does not specify, atopic disorders should be suspected in all migraine patients and their relatives, not only for accurate diagnosis but also for planning prophylactic medications, such as β-blockers.

Chronic migraine is an infrequent type of migraine and shows age-related changes in some phenotypic characteristics, such as severity of attacks, especially in women aged older than 50 years. Furthermore, positive family history of headaches and history of motion sickness increase the likelihood of developing chronic migraine in older women, indicating involvement of some gender-related, but as-yet unknown, genetic factors.

Purpose: To reveal the presence and nature of exiting points of supraorbital region neurovascular structures and determine the distances of those structures to midline with computed tomography images by taking into account gender and sides in patients with migraine. Methods: The study was conducted retrospectively on computed tomography images of 70 migraine and 70 control patients with a mean age of 39.5 ± 13.8 years (range: 18–80). Presence and nature (foramen or notch) of exiting points of neurovascular structures in terms of side and gender in both groups, and the distances of these structures to the midline of the face were evaluated. Results: In migraine and control groups, the most commonly seen structure was single notch. Coexistence of foramen and notch was statistically significant in migraine and female migraine groups than control and female control groups (P < 0.05). Bilateral presence of supraorbital structure was 51.4% in migraine group and 64.3% in control group patients. In all cases, foramen-midline distance was statistically significant longer than the notch-midline distance (P < 0.05). In migraine patients, no statistically significant difference was detected regarding distances of foramen and notch to midline in terms of side and gender. Conclusion: Consideration of variable presence and location of the supraorbital notch and foramen, analysis of computed tomography scan might be beneficial in preoperative planning of foraminotomy and fascial band release in adult migraine patients to prevent intraoperative complications. Also, coexistence is more frequent on left side in migraine patients that might cause overlooking those structures during surgery.

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Background Richardson’s syndrome (RS) is considered the most symmetric phenotype of progressive supranuclear palsy (PSP) as opposed to PSP with predominant corticobasal syndrome (PSP-CBS) or parkinsonism (PSP-P). Objectives Evaluate asymmetrical motor and higher cortical features in probable PSP-RS and compare the degree of asymmetry of cortical lobes and hemispheres between PSP-RS, PSP-CBS, PSP-P, and age-matched healthy controls (HC). Methods Asymmetry of motor and higher cortical features evaluated with an extensive videotaped neurologic examination was investigated in 28 PSP-RS, 8 PSP-CBS, and 14 PSP-P. Brain MRI to compute the laterality index (LI) was performed in 36 patients as well as in 56 HC. Results In PSP-RS, parkinsonism was the most common asymmetric motor feature (53.6%), followed by dystonia and myoclonus (21.4% and 17.9%, respectively). Among higher cortical features, limb apraxia was found asymmetric in about one-third of patients. PSP-RS disclosed higher LI for hemispheres compared to HC, indicating a greater degree of asymmetry (p = 0.003). The degree of asymmetry of clinical features was not different between PSP-RS and those qualifying for PSP-CBS or PSP-P. As for imaging, LI was not different between PSP-RS, PSP-CBS, and PSP-P in any cortical region. Conclusions Motor and higher cortical features are asymmetric in up to 50% of PSP-RS who also present a greater degree of asymmetry in hemispheres compared to age-matched HC. Lateralization of clinical features should be annotated in PSP.

Childhood and adolescent headaches somehow look like adulthood headaches but not known as much as them. In this paper eight rare known primary headache disorders of children and adolescents have been given with practical clues for physicians. All of the cases have been selected from Mersin University Faculty of Medicine, Childhood and Adolescent Headache Outpatient Department database and discussed with literature. This paper mainly based on increasing physician avareness about unusual primary headache disorders of children and adolescents.

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