CNS findings in long-term survivors
UPN . | Current age . | Major findings . | CNS imaging after HSCT (age) . | Abnormal findings . | Special school . | |||||
---|---|---|---|---|---|---|---|---|---|---|
Hyperactivity . | Motor dysfunction . | Verbal expression . | Hearing deficit . | Seizures . | Learning disability . | |||||
U466 | 4 y | Normal | ND | − | − | − | − | − | — | − |
U396 | 6 y | Muscular hypotonia (4 mo); sitting without support (18 mo); walking (24 mo); current: gross motor coordination disorder with tiptoe walking, reduced verbal expression (5-10 words), severe learning disability, hyperactivity, continuous special care | US: normal (6 mo) | + | + | + | − | − | Severe | + |
U301 | 9 y | Pendular nystagmus (5 mo); sitting without support (16 mo); walking (24 mo); current: gross motor coordination disorder with tiptoe walking, pendular nystagmus, reduced verbal expression, sensorineural hearing deficit, severe learning disability, hyperactivity, continuous special care | US: normal (3 mo) | + | + | + | + | − | Severe | + |
U175 | 14 y | Mild learning disability | ND | − | + | − | + | − | Mild | + |
U118 | 17 y | Normal, finished regular school | CT scan: normal (6 mo) | − | − | − | − | − | — | − |
M1052 | 9 y | Developmental delay (4 mo); sitting without support (11 mo); walking (18 mo); current: fine motor and coordination deficit, slurred speech, hyperactivity, mild learning disability | ND | + | + | + | + | − | Mild | + |
M1053 | 9 y | Muscular hypotonia (4 mo); sitting without support (18 mo); walking (24 mo); seizures (35 y); current: spastic diplegia, slurred speech, reduced verbal expression (< 10 words), severe learning disability, seizures, continuous special care | MRI: mild expansion of external CSF space (6 y) | − | + | + | − | + | Severe | + |
U442 | 5 y | Normal | ND | − | − | − | − | − | — | − |
U444 | 5 y | Normal | US: normal (3 mo) | − | − | − | − | − | — | − |
U51 | 21 y | Hearing deficit (12 mo); sitting without support (12 mo); walking (23 mo); current: reduce expressive speech, attention deficit, moderate to severe learning disability, hyperactivity, needs support in everyday life | CT-scan: normal (8 mo+5 y) | + | + | + | + | − | Moderate/severe | + |
U36 | 22 y | Normal, finished regular school | ND | − | − | − | − | − | — | − |
U26 | 22 y | Generalized muscular hypertonia (11 mo); sitting without support (15 mo); walking (20 mo); current: fine motor and coordination deficit, slurred speech, attention deficit, moderate learning disability, needs support in everyday life | ND | − | + | + | + | − | Moderate | + |
UPN . | Current age . | Major findings . | CNS imaging after HSCT (age) . | Abnormal findings . | Special school . | |||||
---|---|---|---|---|---|---|---|---|---|---|
Hyperactivity . | Motor dysfunction . | Verbal expression . | Hearing deficit . | Seizures . | Learning disability . | |||||
U466 | 4 y | Normal | ND | − | − | − | − | − | — | − |
U396 | 6 y | Muscular hypotonia (4 mo); sitting without support (18 mo); walking (24 mo); current: gross motor coordination disorder with tiptoe walking, reduced verbal expression (5-10 words), severe learning disability, hyperactivity, continuous special care | US: normal (6 mo) | + | + | + | − | − | Severe | + |
U301 | 9 y | Pendular nystagmus (5 mo); sitting without support (16 mo); walking (24 mo); current: gross motor coordination disorder with tiptoe walking, pendular nystagmus, reduced verbal expression, sensorineural hearing deficit, severe learning disability, hyperactivity, continuous special care | US: normal (3 mo) | + | + | + | + | − | Severe | + |
U175 | 14 y | Mild learning disability | ND | − | + | − | + | − | Mild | + |
U118 | 17 y | Normal, finished regular school | CT scan: normal (6 mo) | − | − | − | − | − | — | − |
M1052 | 9 y | Developmental delay (4 mo); sitting without support (11 mo); walking (18 mo); current: fine motor and coordination deficit, slurred speech, hyperactivity, mild learning disability | ND | + | + | + | + | − | Mild | + |
M1053 | 9 y | Muscular hypotonia (4 mo); sitting without support (18 mo); walking (24 mo); seizures (35 y); current: spastic diplegia, slurred speech, reduced verbal expression (< 10 words), severe learning disability, seizures, continuous special care | MRI: mild expansion of external CSF space (6 y) | − | + | + | − | + | Severe | + |
U442 | 5 y | Normal | ND | − | − | − | − | − | — | − |
U444 | 5 y | Normal | US: normal (3 mo) | − | − | − | − | − | — | − |
U51 | 21 y | Hearing deficit (12 mo); sitting without support (12 mo); walking (23 mo); current: reduce expressive speech, attention deficit, moderate to severe learning disability, hyperactivity, needs support in everyday life | CT-scan: normal (8 mo+5 y) | + | + | + | + | − | Moderate/severe | + |
U36 | 22 y | Normal, finished regular school | ND | − | − | − | − | − | — | − |
U26 | 22 y | Generalized muscular hypertonia (11 mo); sitting without support (15 mo); walking (20 mo); current: fine motor and coordination deficit, slurred speech, attention deficit, moderate learning disability, needs support in everyday life | ND | − | + | + | + | − | Moderate | + |
US indicates ultrasound; CSF, cerebrospinal fluid; +, present finding; and −, absent finding.