Familial neuromyelitis optica (Devic’s syndrome) with late onset in Japan

Neuromyelitis optica (NMO), or Devic’s syndrome, is characterized by the selective involvement of the optic nerves and spinal cord with normal brain MRI, in distinction to typical MS. The majority of NMO cases are sporadic, and the disease usually affects young adults: familial NMO has never previously been reported in Japan, where the nonfamilial form of NMO is relatively common, and none of the 71 cases of NMO seen at the Mayo Clinic was familial, although four patients reported a family history of MS.1 Only two familial NMO cases have been reported in the literature, and the ages at onset in those cases were 2 and 3 years in one family and 24 and 26 years in the other.2,3 We herein report NMO in two elderly Japanese sisters in whom the onset of disease occurred at age 59 and age 62 years. Patient 1. Patient 1 was a 67-year-old woman. When she was 62, right-sided blindness developed over 4 days, but the vision was restored in a year. At age 65, she became quadriplegic and felt numb below the neck. After 3 months of rehabilitation, she could walk using a cane. At age 67, she developed bilateral blurred vision, paraplegia, and dysuria subacutely, …