Measure | Descriptor |
---|---|
Jabbour Stages [15] | |
Stage IA | Behavioral, cognitive and personality change only. |
Stage IB | Myoclonic Spasms: focal and not periodic. |
Stage IIA | Further mental deterioration. Myoclonic spasms: periodic, generalized, frequently causing drop spells precluding ambulation |
Stage IIB | Apraxias, agnosias, language difficulties. Motor signs: spasticity, ataxia. Ambulation with assistance only. |
Stage IIIA | Speaking less, visual difficulties. Sits up. Myoclonic spasms frequent (every 3–5 seconds). May have seizures. |
Stage IIIB | No spontaneous speech, poor comprehension, blind. Myoclonic spasms. Bedridden, dysphagia. EEG background delta. Other abnormal movements: chorea, ballismus. |
Stage IV | No myoclonic spasms. EEG low voltage with no periodic slow wave complexes. Patient in a vegetative state. |
Neurologic Disability Index (NDI) [23] | |
Behavioral/Mental | Irritability, Personality, Withdrawal, Intelligence and Higher cortical function |
Myoclonia/Seizures | Location, Repetition, Frequency, Seizures (major), Synchrony |
Motor/Sensory | Reflexes and tone, Strength and bulk, Abnormal postures and incoordination, Movements, Sensory modalities |
Vegetative/Systemic | Vision, Hearing, Verbalizations, Autonomic, Nutritional |
For each characteristic a score is given from 0 to 4 indicating 'No abnormality' to 'Profound abnormality'. Total Score is 80 and converted to a percentage. A higher score indicates greater impairment. | |
Brief Assessment Examination (BAE). [22] | |
Alertness | No response = 8. Minimum general response = 6, Specific response (35% level) = 4. Specific response (70% level) = 2. Normal = 0. |
Receptive Speech/Language and Cognitive | Follows commands 0–3. Points at objects 0–3. Points at colours 0–3. Points at shapes 0–3. |
Visual Digit Span | Zero digits = 8. One digit = 6. Two digits = 4. Three digits = 2. Four digits = 0. |
Speech | No response= 8. Severe dysarthria = 6. Moderate dysarthria = 4. Mild dysarthria = 2. Normal = 0. |
Mathematics | No skills = 8. Counts to five = 6. Counts to ten = 4. Single digit addition = 2. Single digit subtraction = 0. |
Pictorial Memory | Zero objects = 8. One objects = 6. Two objects = 4. Three objects = 2. Four objects = 0. |
Total Score is out of 60 with greater scores reflecting greater impairment. Modifications are outlined for children less than 6 years of age. |