Table 1:

Summary of the clinical features of patients with NIID

Patient No.
Age at onset (yr)6452626267676764
Disease duration at MRI (yr)29835615
Clinical manifestations
    Muscle weakness
    TremorPosturalPosturalPosturalResting, postural
    Hyporeflexia or areflexia in DTR++++++++
    Sensory disturbance
    Urinary incontinence++
    Transient visual field abnormality+++
    Transient hemiparesis+
    Transient abnormal behavior+++
    Generalized convulsion
    Disturbance of consciousness+++
Cognitive screening test
Frontal lobe function test
    Cell (mm3)324116NENE
    Protein (mg/dL)6655100475248NENE
  • Note:—DTR indicates deep tendon reflex; –, absence of the manifestation; +, presence of the manifestation; NE, not examined.

  • a Decreased MMSE score below a cutoff score of 24.

  • b Decreased FAB score below the published age-matched average.