Phenotypes for disease #00448 (MLD (Metachromatic leukodystrophy), OMIM:250100)

458 entries on 5 pages. Showing entries 1 - 100.
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AscendingPhenotype 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
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