Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology A 3-year-old boy with silvery gray hair and leukodystrophy.

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Presentation transcript:

Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology A 3-year-old boy with silvery gray hair and leukodystrophy

Vignette A three-year-old boy presented with fever, altered sensorium, focal seizures and regression General examination revealed silvery-gray hair over the scalp and eyebrows and hepatosplenomegaly – Skin, iris and retina were normally pigmented Neurological examination reveled bilateral papilloedema, spastic-quadriparesis, brisk muscle-stretch reflexes and extensor plantars Hepatosplenomegaly Saini et al. © 2013 American Academy of Neurology

Saini et al. Facial and scalp hair characteristics © 2013 American Academy of Neurology

Saini et al. Neuroimaging © 2013 American Academy of Neurology

Griscelli syndrome and CNS lymphohistiocytosis GS is an autosomal-recessive disorder of melanosome transport characterized by hypopigmentation, immunodeficiency and usually results in death in childhood Silvery-gray hair is an important clinical clue Hair-microscopy is diagnostic Fatal complications include the “accelerated phase,” characterized by lymphohistiocytic proliferation in various organs, including brain Saini et al. © 2013 American Academy of Neurology