Table 3

Clinical outcome measures used in SSPE.

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.


Campbell et al. BMC Pediatrics 2005 5:47   doi:10.1186/1471-2431-5-47

Open Data