“Eye of the Tiger” in a Non-Responsive Neuropsychiatric Patient: A Case Report
Hallervorden-Spatz syndrome is a rare neurodegenerative disorder with hereditary properties. It usually occurs in young adolescents with extrapyramidal symptoms besides disturbed mental function. In this study, we present a 23-year-old neuropsychiatric patient who primarily misdiagnosed to have conversion disorder. She had 5-year history of progressive dysarthria and generalized abnormal movements. After detecting the pathognomonic sign of “eye of the tiger” diagnosis was confirmed. The patient was discharged. She had satisfactory condition in her follow-up. Such a rare syndrome should be considered in patients with similar presentation, and upon the diagnosis, PKAN2 gene study should be done to detect possible new mutations.
Haraguchi T, Terada S, Ishizu H, Yokota O, Yoshida H, Takeda N, et al. Coexistence of TDP‐43 and tau pathology in neurodegeneration with brain iron accumulation type 1 (NBIA‐1, formerly Hallervorden‐Spatz syndrome). Neuropathology. 2011;31(5):531-9.
Hayflick SJ, Westaway SK, Levinson B, Zhou B, Johnson MA, Ching KH, et al. Genetic, clinical, and radiographic delineation of Hallervorden–Spatz syndrome. New England Journal of Medicine. 2003;348(1):33-40.
Craenenbroeck AV, Gebruers M, Martin JJ, Cras P. Hallervorden‐Spatz disease: Historical case presentation in the spotlight of nosological evolution. Movement Disorders. 2010;25(15):2486-92.
Molinuevo JL, Martí MJ, Blesa R, Tolosa E. Pure akinesia: An unusual phenotype of Hallervorden‐Spatz syndrome. Movement disorders. 2003;18(11):1351-3.
Synofzik M, Asmus F, Reimold M, Schöls L, Berg D. Sustained dopaminergic response of parkinsonism and depression in PoLG‐associated parkinsonism. Movement Disorders. 2010;25(2):243-5.
Neumann M, Adler S, Schlüter O, Kremmer E, Benecke R, Kretzschmar HA. α-Synuclein accumulation in a case of neurodegeneration with brain iron accumulation type 1 (NBIA-1, formerly Hallervorden-Spatz syndrome) with widespread cortical and brainstem-type Lewy bodies. Acta neuropathologica. 2000;100(5):568-74.
Hayflick SJ. Unraveling the Hallervorden-Spatz syndrome: pantothenate kinase–associated neurodegeneration is the name. Current opinion in pediatrics. 2003;15(6):572-7.
Vasconcelos O, Harter D, Duffy C, McDonough B, Seidman J, Seidman C, et al. Adult Hallervorden–Spatz syndrome simulating amyotrophic lateral sclerosis. Muscle & nerve. 2003;28(1):118-22.
Grimes D, Lang A, Bergeron C. Late adult onset chorea with typical pathology of Hallervorden-Spatz syndrome. Journal of Neurology, Neurosurgery & Psychiatry. 2000;69(3):392-5.