
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
| 0000016801 |
juvenile MLD, diagnosed clinically and biochemically; GS 280 nmoles/mg lipid (normal=2+/-1) |
- |
- |
Familial, autosomal recessive |
- |
- |
03y |
- |
9 nmol/mg/hr (normal=137+/-53) |
Martina Cesani |
00018449 |
| 0000016902 |
juvenile, MRI brain pathological; cognitive and motor decline as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
02y08m |
- |
27 nmol/mg/h (normal= 100-260) |
Martina Cesani |
00018555 |
| 0000016904 |
juvenile, MRI brain pathological; cognitive/behavioral regression as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
04y02m |
- |
16 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00018557 |
| 0000016905 |
juvenile, MRI brain pathological; cognitive and motor decline as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
03y11m |
- |
15.8 nmol/mg/h (normal= 78-180) |
Martina Cesani |
00018559 |
| 0000016906 |
juvenile, MRI brain pathological; cognitive/behavioral regression as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
03y09m |
- |
0.04 microkat/kg |
Martina Cesani |
00018560 |
| 0000017297 |
juvenile, MRI brain pathological; cognitive/behavioral regression as first symptom; no peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
11y |
- |
28 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00019495 |
| 0000017336 |
adult, MRI brain pathological; cognitive/behavioral regression as first symptom; no peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
31y |
- |
11.5 nmol/mg/h (normal= 78-180) |
Martina Cesani |
00019539 |
| 0000017337 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y04m |
- |
14 nmol/mg/h (normal= 71) |
Martina Cesani |
00019540 |
| 0000017338 |
late infantile, MRI brain pathological; cognitive/behavioral regression as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
02y06m |
- |
18 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00019542 |
| 0000017339 |
juvenile, MRI brain pathological; cognitive/behavioral regression as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
05y |
- |
6.8 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00019543 |
| 0000017340 |
Juvenile; no peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
06y |
- |
4.5%; 6% |
Martina Cesani |
00019544 |
| 0000017341 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y04m |
- |
8.5 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00019545 |
| 0000017342 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y |
- |
4.88 nmol/mg/h (normal= 63.32) |
Martina Cesani |
00019546 |
| 0000017343 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y04m |
- |
18 nmol/mg/h |
Martina Cesani |
00019547 |
| 0000017344 |
Late Infantile; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y |
- |
22.1 nmol/mg/h (normal:121±30 nmol/mg/h) |
Martina Cesani |
00019548 |
| 0000017345 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y02m |
- |
11% of control |
Martina Cesani |
00019549 |
| 0000017346 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y09m |
- |
14.6 nmol/mg/h (normal= 121+/-30) |
Martina Cesani |
00019550 |
| 0000017381 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
02y |
- |
58 nmol/mg/h (normal= 80-200) |
Martina Cesani |
00019591 |
| 0000017382 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y03m |
- |
3.24 nmol/mg/h (normal= 63.32) |
Martina Cesani |
00019592 |
| 0000017383 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y04m |
- |
0.5 nmol/mg/h (normal= 10-22) |
Martina Cesani |
00019593 |
| 0000017384 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y06m |
- |
5 nmol/mg/h (normal= 121+/-30) |
Martina Cesani |
00019594 |
| 0000017385 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
01y09m |
- |
5.5-13.5nmol/mg/h |
Martina Cesani |
00019595 |
| 0000017386 |
late infantile, MRI brain pathological; gross motor impairment as first symptom; peripheral neuropathy (HP:0009830) |
- |
- |
Familial, autosomal recessive |
- |
- |
02y |
- |
4 nmol/mg/h (normal= 40-300) |
Martina Cesani |
00019596 |
| 0000027470 |
9.5y-neurological examination minimal signs of diffuse encephalopathy, minimal distally accentuated polyneuropathy; 12.5y-neurological findings entirely normal, excellent school reports, computerized tomography/nuclear magnetic resonance/nerve conduction velocity normal (11y and 12.2y); GS 137 nmoles/mg lipid (normal=2+/-1) |
- |
- |
Isolated (sporadic) |
05y |
- |
- |
- |
- |
Johan den Dunnen |
00019502 |
| 0000027471 |
late infantile; MRI brain pathological, spastic paraparesis, ataxia, mental deterioration, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
02y |
ataxia |
- |
Alessandra Biffi |
00034075 |
| 0000027472 |
late infantile, spastic tetraparesis, mental deterioration, slowed motor NCV |
- |
- |
Familial, autosomal recessive |
- |
- |
01y |
psychomotor deterioration |
- |
Alessandra Biffi |
00034076 |
| 0000027473 |
late infantile; spastic tetraparesis, mental deterioration, slowed motor and sensory NCVs, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
01y05m |
gait disturbances, strabismus |
47 nmol/mg/h (normal= 520+/-149) |
SIB - Livia Famiglietti |
00034077 |
| 0000027474 |
spastic tetraparesis, mental deterioration, slowed motor and sensory NCVs, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
01y02m |
psychomotor regression |
- |
SIB - Livia Famiglietti |
00034078 |
| 0000027475 |
late infantile; spastic paraparesis, mental deterioration, slowed motor NCV, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
01y10m |
gait disturbances, mental deterioration |
- |
SIB - Livia Famiglietti |
00034079 |
| 0000027477 |
late infantile; spastic paraparesis, dysarthria, slowed motor and sensory NCVs, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
01y02m |
gait disturbances |
26.4 nmol/mg/h |
SIB - Livia Famiglietti |
00034081 |
| 0000027479 |
spastic paraparesis, mental deterioration, slowed motor and sensory NCVs, symmetrical deep WM abnormalities |
- |
- |
Familial, autosomal recessive |
- |
- |
01y09m |
gait disturbances |
- |
SIB - Livia Famiglietti |
00034083 |
| 0000027480 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
06y-08y |
- |
- |
Alessandra Biffi |
00034084 |
| 0000027481 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
01y03m |
gait disturbances |
5 nmol/mg/h (normal= 65.8+/-13.7) |
Alessandra Biffi |
00034085 |
| 0000027482 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034086 |
| 0000027483 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
11y |
mental deterioration |
4.2 nmol/mg/h (normal= 65.8+/-13.7) |
Alessandra Biffi |
00034087 |
| 0000027484 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
16y |
behvioral changes |
8.5 nmol/mg/h (normal= 65.8+/-13.7) |
Alessandra Biffi |
00034088 |
| 0000027485 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.44 nmol/mg/h |
Alessandra Biffi |
00034089 |
| 0000027486 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
22y |
behvioral changes |
7.5 nmol/mg/h (normal= 65.8+/-13.7) |
Alessandra Biffi |
00034090 |
| 0000027487 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034091 |
| 0000027489 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034093 |
| 0000027490 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.62 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034094 |
| 0000027491 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.45 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034095 |
| 0000027492 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
10.9 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034096 |
| 0000027493 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
2.3 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034097 |
| 0000027494 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.42 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034098 |
| 0000027495 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.91 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034099 |
| 0000027496 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034100 |
| 0000027497 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034101 |
| 0000027498 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0.19 nmol/mg/h (normal= 51+/-14) |
Alessandra Biffi |
00034102 |
| 0000027499 |
adult, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
26y |
- |
14.1 nmol/mg/h (normal= 109.0-217.2) |
Alessandra Biffi |
00034103 |
| 0000027501 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034105 |
| 0000027502 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034106 |
| 0000027503 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034107 |
| 0000027504 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034108 |
| 0000027505 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034109 |
| 0000027506 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034110 |
| 0000027507 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034111 |
| 0000027508 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
SIB - Livia Famiglietti |
00034112 |
| 0000027509 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034113 |
| 0000027510 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034114 |
| 0000027511 |
juvenile, MRI brain pathological, behavioral changes, epilepsy |
- |
- |
Familial, autosomal recessive |
- |
- |
07y |
- |
5% of control |
Alessandra Biffi |
00034115 |
| 0000027512 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034116 |
| 0000027513 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034117 |
| 0000027514 |
late infantile, developmental delay, spina bifida, unusual physical features |
- |
- |
Familial, autosomal recessive |
- |
- |
01y |
- |
0% of control |
Alessandra Biffi |
00034118 |
| 0000027515 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034119 |
| 0000027516 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034120 |
| 0000027517 |
late infantile, MRI-brain apthological |
- |
- |
Familial, autosomal recessive |
- |
- |
01y |
- |
low |
Alessandra Biffi |
00034121 |
| 0000027519 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034123 |
| 0000027520 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034124 |
| 0000027521 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034125 |
| 0000027522 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034126 |
| 0000027523 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034127 |
| 0000027524 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
09y |
- |
- |
Alessandra Biffi |
00034128 |
| 0000027525 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
08y |
- |
- |
Alessandra Biffi |
00034129 |
| 0000027526 |
adult, MRI brain pathological, cognitive decline, no motor impairment |
- |
- |
Familial, autosomal recessive |
- |
37y |
- |
- |
0.01 nmol/mg/h |
Alessandra Biffi |
00034130 |
| 0000027527 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034131 |
| 0000027528 |
juvenile, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
07y |
- |
3 nmol/mg/h (normal= 118.5) |
Alessandra Biffi |
00034132 |
| 0000027529 |
late infantile, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
00y06m |
- |
15 nmol/mg/h (normal= 118.5) |
Alessandra Biffi |
00034133 |
| 0000027530 |
juvenile, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
05y |
- |
25 nmol/mg/h (normal= 118.5) |
Alessandra Biffi |
00034134 |
| 0000027531 |
adult |
- |
- |
Familial, autosomal recessive |
- |
- |
18y |
- |
2 nmol/mg/h (normal= 34-117) |
Alessandra Biffi |
00034135 |
| 0000027532 |
adult, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
42y |
- |
11.3% of control |
Alessandra Biffi |
00034136 |
| 0000027534 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034138 |
| 0000027535 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
0% of control |
Alessandra Biffi |
00034139 |
| 0000027536 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
13y |
- |
- |
Alessandra Biffi |
00034140 |
| 0000027537 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
08y |
- |
5.9% of control |
Alessandra Biffi |
00034141 |
| 0000027538 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Alessandra Biffi |
00034142 |
| 0000027539 |
late infantile |
- |
- |
Familial, autosomal recessive |
- |
- |
<01y |
- |
- |
Alessandra Biffi |
00034143 |
| 0000027540 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034144 |
| 0000027541 |
late infantile, MRI brain pathological |
- |
- |
Familial, autosomal recessive |
- |
- |
02y05m |
- |
2.43 nmol/mg/h |
Alessandra Biffi |
00034145 |
| 0000027542 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034146 |
| 0000027543 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034147 |
| 0000027544 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034148 |
| 0000027545 |
Juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034149 |
| 0000027546 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034150 |
| 0000027548 |
adult, MRI brain pathological, cognitive decline, no motor impairment |
- |
- |
Familial, autosomal recessive |
- |
- |
33y |
- |
15 nmol/mg/h (normal= 109-217) |
Alessandra Biffi |
00034152 |
| 0000027549 |
late infantile, MRI brain pathological, gross motor impairment as first symptom |
- |
- |
Familial, autosomal recessive |
- |
- |
01y08m |
- |
9 nmol/mg/h (normal= 93-145) |
Johan den Dunnen |
00034153 |
| 0000027550 |
late Infantile, peripheral neuropathy as first symptom, normal cognitive performance |
- |
- |
Familial, autosomal recessive |
- |
- |
50y |
- |
0.03 nmol/mg/min (normal= 0.77-1) |
Alessandra Biffi |
00034154 |
| 0000027551 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034155 |
| 0000027552 |
juvenile |
- |
- |
Familial, autosomal recessive |
- |
- |
05y |
- |
5 nmol/mg/h (normal= 43-234) |
Johan den Dunnen |
00034156 |
| 0000027553 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00034157 |