
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
0000029693 |
pure HSP, pes cavus (HP:0001761), no urinary symptoms, no LL sensory disturbance, no europathy, no cognitive
impairment, no thinning corpus callosum, no white matter lesions, no cerebellar signs |
- |
- |
Familial, autosomal dominant |
- |
- |
22y |
- |
- |
David Lynch |
00039355 |
0000069500 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
15y? |
- |
- |
Christian Beetz |
00090126 |
0000072414 |
lower limb weakness (HP:0007340), spastic gait (HP:0002064), mild distal atrophy (HP:0003693), mild scoliosis (HP:0002650), positive Babinski sign (HP:0003487), normal cranial MRI |
- |
- |
Familial, autosomal dominant |
? |
- |
18y |
- |
- |
Christian Beetz |
00094022 |
0000072415 |
mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
18y |
- |
- |
Christian Beetz |
00094023 |
0000072419 |
lower limb weakness (HP:0007340), spastic gait (HP:0002064), ankle clonus (HP:0011448), mild scoliosis (HP:0002650), positive Babinski sign (HP:0003487), normal spinal MRI; age at onset 5-18y |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094027 |
0000072420 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094028 |
0000072421 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094029 |
0000072422 |
age onset 5-18y; mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094030 |
0000072423 |
mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094031 |
0000072424 |
mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094032 |
0000072425 |
mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094033 |
0000072426 |
mild scoliosis |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094034 |
0000072427 |
lower limb weakness (HP:0007340), spastic gait (HP:0002064), no scoliosis (-HP:0002650), positive Babinski sign (HP:0003487), normal spinal MRI, learning difficulties (2 affecteds); age at onset 3-62y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094035 |
0000072428 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094036 |
0000072429 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094037 |
0000072430 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094038 |
0000072431 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094039 |
0000072432 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094040 |
0000072433 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094041 |
0000072434 |
age onset 3-62y; maybe learning difficulties |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094042 |
0000072435 |
lower limb weakness (HP:0007340), spastic gait (HP:0002064), ankle clonus (HP:0011448), no scoliosis (-HP:0002650), positive Babinski sign (HP:0003487), normal spinal MRI; age at onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094043 |
0000072436 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094044 |
0000072437 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094045 |
0000072438 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094046 |
0000072439 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094047 |
0000072440 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094048 |
0000072441 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094049 |
0000072442 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094050 |
0000072443 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094051 |
0000072444 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094052 |
0000072445 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094053 |
0000072446 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094054 |
0000072447 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094055 |
0000072448 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094056 |
0000072449 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094057 |
0000072450 |
age onset 3-60y |
- |
- |
Familial, autosomal dominant |
? |
- |
- |
- |
- |
Christian Beetz |
00094058 |
0000072451 |
none |
- |
- |
Familial, autosomal dominant |
76y |
- |
? |
- |
- |
Christian Beetz |
00094059 |
0000072452 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
<76y |
- |
- |
Christian Beetz |
00094060 |
0000072453 |
right cerebellar venous angioma |
- |
- |
Familial, autosomal dominant |
<51y |
- |
32y |
- |
- |
Christian Beetz |
00094061 |
0000072454 |
none |
- |
- |
Familial, autosomal dominant |
<23y |
- |
4y |
- |
- |
Christian Beetz |
00094062 |
0000072455 |
none |
- |
- |
Familial, autosomal dominant |
<60y |
- |
<10y |
- |
- |
Christian Beetz |
00094063 |
0000072456 |
none |
- |
- |
Familial, autosomal dominant |
<60y |
- |
<10y |
- |
- |
Christian Beetz |
00094064 |
0000072457 |
none |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094065 |
0000072458 |
none |
- |
- |
Familial, autosomal dominant |
<60y |
- |
<10y |
- |
- |
Christian Beetz |
00094066 |
0000072459 |
none |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094067 |
0000072460 |
none |
- |
- |
Familial, autosomal dominant |
5y |
- |
? |
- |
- |
Christian Beetz |
00094068 |
0000072461 |
none |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094069 |
0000072462 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094070 |
0000072463 |
none |
- |
- |
Familial, autosomal dominant |
24y |
- |
3y |
- |
- |
Christian Beetz |
00094071 |
0000072464 |
none |
- |
- |
Familial, autosomal dominant |
24y |
- |
3y |
- |
- |
Christian Beetz |
00094072 |
0000072467 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094075 |
0000072468 |
none |
- |
- |
Familial, autosomal dominant |
>50y |
- |
<10y |
- |
- |
Christian Beetz |
00094076 |
0000072469 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
<45y |
- |
- |
Christian Beetz |
00094077 |
0000072470 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
<45y |
- |
- |
Christian Beetz |
00094078 |
0000072471 |
white matter lesion above trigonum, central motor conduction time increased latency |
- |
- |
Isolated (sporadic) |
54y |
- |
42y |
- |
- |
Christian Beetz |
00094079 |
0000072472 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094080 |
0000072473 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094081 |
0000072474 |
none |
- |
- |
Familial, autosomal dominant |
? |
- |
38y |
- |
- |
Christian Beetz |
00094082 |
0000072475 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094083 |
0000072476 |
none |
- |
- |
Familial, autosomal dominant |
? |
- |
2y |
- |
- |
Christian Beetz |
00094084 |
0000072477 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094085 |
0000072478 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094086 |
0000072479 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094087 |
0000072480 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094088 |
0000072481 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094089 |
0000072482 |
none |
- |
- |
Familial, autosomal dominant |
<46y |
- |
<15y |
- |
- |
Christian Beetz |
00094090 |
0000072483 |
none |
- |
- |
Familial, autosomal dominant |
<46y |
- |
<15y |
- |
- |
Christian Beetz |
00094091 |
0000072484 |
none |
- |
- |
Familial, autosomal dominant |
<34y |
- |
<15y |
- |
- |
Christian Beetz |
00094092 |
0000072485 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094093 |
0000072486 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094094 |
0000072487 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094095 |
0000072488 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094096 |
0000072489 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094097 |
0000072490 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094098 |
0000072491 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094099 |
0000072492 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094100 |
0000072493 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094101 |
0000072494 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094102 |
0000072495 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094103 |
0000072496 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094104 |
0000072497 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094105 |
0000072498 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094106 |
0000072499 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094107 |
0000072500 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094108 |
0000072501 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094109 |
0000072502 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094110 |
0000072503 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094111 |
0000072504 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094112 |
0000072505 |
age analysis 2-76y, age onset 1-75y; some muscle weakness of hands |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Christian Beetz |
00094113 |
0000072506 |
distal amyotrophy of upper limbs, scoliosis, small unspecific white matter lesions in frontal lobe, distal sensory and motor neuropathy |
- |
- |
Familial, autosomal dominant |
35y |
- |
5y |
- |
- |
Christian Beetz |
00094114 |
0000072507 |
none |
- |
- |
Familial, autosomal dominant |
7y |
- |
4y |
- |
- |
Christian Beetz |
00094115 |
0000072509 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094117 |
0000072510 |
none |
- |
- |
Familial, autosomal dominant |
59y |
- |
50y |
- |
- |
Christian Beetz |
00094118 |
0000072511 |
none |
- |
- |
Familial, autosomal dominant |
56y |
- |
? |
- |
- |
Christian Beetz |
00094119 |
0000072512 |
early upper limb involvement |
- |
- |
Familial, autosomal dominant |
95y |
- |
90y |
- |
- |
Christian Beetz |
00094120 |
0000072513 |
early upper limb involvement |
- |
- |
Familial, autosomal dominant |
47y |
- |
42y |
- |
- |
Christian Beetz |
00094121 |
0000072514 |
none |
- |
- |
Familial, autosomal dominant |
53y |
- |
? |
- |
- |
Christian Beetz |
00094122 |
0000072515 |
scoliosis |
- |
- |
Familial, autosomal dominant |
29y |
- |
4y |
- |
- |
Christian Beetz |
00094123 |
0000072516 |
unknown |
- |
- |
Familial, autosomal dominant |
? |
- |
? |
- |
- |
Christian Beetz |
00094124 |
0000072517 |
decreased vibration sense, distal hypoesthesia, pain, dysphagia |
- |
- |
Familial, autosomal dominant |
19y |
- |
3y |
- |
- |
Christian Beetz |
00094125 |