Table 3. Quantitative severity scale for major NKH disease domains.

Domain and Associated Scoring Scale
Cognitive Disorders
0 –No disease
1 –Behavioral issues, learning disabilities, speech delay
2 –Mental disability, some words, global delay in developmental markers
3 –Severe mental disability, no cognitive abilities
Seizures
0 –No disease
1 –Hiccups (infants), Abnormal EEG, Seizures controlled by medication
3 –Intractable seizures (> 2 AEDs)
Muscle/Movement Control
0 –No disease
1 –Assisted locomotion
2 –Hypotonia, able to roll over or lift head, low muscle tone
3 –Severe hypotonia, unable to roll over or lift head or severe global developmental delay
Brain malformation
0 –No disease
3 –Present

EEG = electroencephalogram

AEDs = Anti-epileptic drugs