Staging System of Escolar et al for Infantile Krabbe Disease (Globoid Cell Leukodystrophy)

Clinical Finding

Group

mild thumb clasp, not fixed

A

hypotonia of shoulder girdle

A

weak feeding

A

gastroesophageal reflux

A

thumb clasp, fixed

B

spasticity in extremities

B

trunk abnormality (extensor tone at any age, or hypotonia > 4 months of age) AND abnormal feeding (difficulty latching to breast; decreased rate of nutritive suck; abnormal tongue, lip or chin movements; uncoordinated suck and swallow)

B

clinical seizures

C

absent deep tendon reflexes; abnormal reflexes

C

exaggerated startle

C

difficulty in visual tracking

C

jerky eye movements

C

abnormal pupillary responses

C

severe weakness AND unresponsive to stimuli AND loss of primitive reflexes

D

blindness or deafness

D

Clinical Findings

Stage

<= 2 Group A; no Group B, C or D

1

all Group A; no Group C or D

2

any group B; no Group C or D

2

spastic extremities AND trunk extensor tone or hypotonia (from Group B) AND >= 1 group C; no group D

3

any group D

4

Clinical Findings

Stage

<= 3 Group A; no Group B, C or D

1

3 or 4 from Group A AND > 2 Group B; no Group C or D

2

spastic extremities AND trunk hypotonia (from Group B) AND >= 1 group C; no group D

3

any group D

4