title: Is congenital melanocytic naevus a link between Hirayama disease and moyamoya pattern: a new syndrome or a co-incidence?
abstract: A 22-year-old man presented with a history of progressive weakness and wasting of the right hand and forearm for 12 months followed by similar symptoms in the left upper limb for the past 5 months. He also gave a history of episodes of loss of consciousness for the past 5 years with a frequency of one per 3 months. On examination, there were melanocytic naevi-one large lesion in the nape of the neck and multiple satellite lesions. On investigation, the cervical cord MRI was normal. The brain MRI and angiography showed a moyamoya pattern. Thus, this patient had congenital melanocytic naevi with Hirayama disease and moyamoya pattern. He was treated with extracranial-intracranial bypass for moyamoya disease. During 6-month follow-up, he has been stable. Although moyamoya syndrome has been associated with several systemic diseases and conditions, the coexistence of a moyamoya pattern with Hirayama disease and melanocytic naevi has not been described so far.
authors.affiliation: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Inder
authors.initials: I
authors.lastname: Puri
authors.affiliation: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Deepti
authors.initials: D
authors.lastname: Vibha
authors.affiliation: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Kameshwar
authors.initials: K
authors.lastname: Prasad
authors.affiliation: Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Rohit
authors.initials: R
authors.lastname: Bhatia
mesh_terms: D001132:Arm; D006801:Humans; D008297:Male; D009072:Moyamoya Disease / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D018908:Muscle Weakness / Q000209:etiology; D009333:Neck / Q000473:pathology; D009508:Nevus, Pigmented; D012878:Skin Neoplasms / Q000473:pathology; D014897:Spinal Muscular Atrophies of Childhood / Q000150:complications / Q000175:diagnosis*; D013577:Syndrome; D014474:Unconsciousness / Q000209:etiology; D055815:Young Adult