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