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Hypomelanosis of Ito

¹ Department of Neurology, NIMHANS, Bangalore, India
² Department of Neuroradiology, NIMHANS, Bangalore, India

Correspondence to:
Correspondence to:
Professor A B Taly
Department of Neurology, NIMHANS, Bangalore: 560 029, Karnataka, India; abtaly@yahoo.com

Abbreviations: HI, Hypomelanosis of Ito; MR, mental retardation; MRI, magnetic resonance imaging

Keywords: Epilepsy; hemimegalencephaly; hemihypertrophy; hypomelonosis of Ito; MRI

The first 150 words of the full text of this article appear below.

Hypomelanosis of Ito (HI) is characterised by hypochromic unilateral skin lesions, hemi-hypertrophy, mental retardation (MR) and seizures.¹ We describe a young woman with HI with interesting and hitherto unreported magnetic resonance imaging (MRI) changes.

A 22 year old woman presented with a history of uncontrolled generalised tonic clonic seizures and myoclonic jerks since the age of 2 years. She had hypopigmented macular lesions in streaks and whorls on her right side in a dermatomal distribution (fig 1 A–D). There was hemihypertrophy of the right upper extremity, and it measured 15 mm longer than the left (fig 1C).

View larger version (135K): [in this window] [in a new window]   Figure 1  Phenotypic features of hypomelanosis of Ito. (A) Hypopigmented macular lesions in streaks and patches over the right side of trunk; (B) striking whorl over the right half of the abdomen; (C, D) hemihypertrophy of the right upper and lower extremities with hypopigmented macular lesions. Informed consent was obtained for publication of this . . . [Full text of this article]