LAUSR

Neuronal intranuclear inclusion disease (NIID) is the pathological hallmark of a heterogeneous neurodegenerative disease span-ning dementia, neuropathy, parkinsonism, encephalitic episodes, and seizures. 1 Onset may be in infancy, childhood, or adulthood and be sporadic or familial. Adult-onset disease is divided into dementia- and limb-weakness dominant subtypes. Recent novel presentations include isolated tremor, 2 migraine, 3 recurrent vomiting, 4 and oromandibular dystonia. 5 Identi fi cation of GGC expansions in the 5 ’ UTR of the Notch-2 N-terminal-like-C ( NOTCH2NLC ) gene have enhanced diagnosis. 1 We report a patient with an 11-year history of adult-onset cervical dystonia (CD), without other symptoms, in association with imaging and biopsy appearances of NIID and a monoallelic “ GGC ” repeat expansion in NOTCH2NLC (genetic fi ndings reported elsewhere). 6 Our patient expands the spectrum of NOTCH2NLC and highlights the importance of considering NIID in familial movement disorders with leukoencephalopathy. A 59-year-old Chinese woman was referred with gradually progressive head tremor and neck discomfort. There has been no symptom evolution over 7 years of follow-up. Her father and sister experience upper limb tremor, but were not available for