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1.  Eye-of-the-Tiger sign is not Pathognomonic of Pantothenate Kinase-Associated Neurodegeneration in Adult Cases 
Brain and Behavior  2011;1(1):55-56.
An eye-of-the-tiger sign is previously known to have one-to-one correlation with pantothenate kinase-associated neurodegeneration (PKAN). Reviewing the literature on this subject, the correlation between eye-of-the-tiger sign and PKAN seems to show an interesting hypothesis that differs from conventional conclusion. We analyze the published papers in an attempt to reflect this trend and illustrate our points with findings in a 39-year-old man. His brain magnetic resonance imaging study shows typical eye-of-the-tiger sign suggestive of PKAN. Genetic analyses revealed no mutations in pantothenate kinase 2.
doi:10.1002/brb3.8
PMCID: PMC3217674  PMID: 22398981
Neurodegeneration with brain iron accumulation; pantothenate kinase-associated neurodegeneration; pantothenate kinase 2; eye-of-the-tiger sign; PKAN; NBIA; Hallervorden-Spatz syndrome
2.  Challenges in diagnosis of isolated central nervous system vasculitis 
Brain and Behavior  2011;1(1):57-61.
Isolated central nervous system (CNS) vasculitis is a rare and complicated disorder. Patients typically present with nonspecific neurologic symptoms such as headache and encephalopathy, and have variable progression and severity of the disease. Challenges to definitive diagnosis include the limitations of currently available diagnostic modalities with high likelihood of false-positive or false-negative findings. Imaging, serologic, and cerebrospinal fluid (CSF) evaluation, and even angiography can fail to establish the diagnosis. Often, brain biopsy is required. In order to illustrate these challenges, we report the case of a patient who presented with subacute cognitive decline and was ultimately diagnosed with isolated CNS eosinophilic vasculitis. Initial work-up included CSF and serologic analyses, magnetic resonance imaging (MRI), and cerebral angiography, but definitive diagnosis required brain biopsy. Immunosuppressive therapy resulted in clinical improvement and stabilization. To our knowledge, only one other case of isolated CNS eosinophilic vasculitis has been reported in the literature. We discuss the importance of a high index of clinical suspicion in cases of progressive nonspecific neurologic symptoms.
doi:10.1002/brb3.12
PMCID: PMC3217675  PMID: 22398982
Vasculitis; hypereosinophilic syndrome; primary angiitis of the central nervous system; memory; aphasia

Results 1-2 (2)