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Table 3 Clinical outcome measures used in SSPE.

From: Subacute Sclerosing Panencephalitis: Results of the Canadian Paediatric Surveillance Program and review of the literature

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.