
 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
| 0000050877 |
HP:0007503 (Generalized Ichthyosis); HP:0001257 (Spasticity);
HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
03y |
- |
- |
- |
- |
Johan den Dunnen |
00064676 |
| 0000054366 |
HP:0012758 (neurodevelopmental delay); HP:0001622 (premature birth, 31 weeks, complications by infections at 6/7 months: malaria, varicella);
HP:0007503 (generalized ichtyosis,neck, trunk, extremities);
HP:0001250 (seizures; brief and isolated, remission without treatment);
HP:0000962 (hyperkeratosis);
HP:0000989 (pruritus);
HP:0007894 (Fundus hypopigmentation, mild);
HP:0007034 (generalized hyperreflexia);
HP:0001264 (spastic diplegia, lower ex more than upper ex) |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Maximilian Weustenfeld |
00074600 |
| 0000058854 |
HP:0007503 (generalized Ichthyosis, entire body); HP:0012758 (neurodevelopmental delay, movement, speech); HP:0002510 (spastic quadriplegia, partial contractures in all extremeties);
HP:0011400 (abnormal CNS myelination); HP:0001250 (seizures); HP:0001336 (nocturnal myoclonus); HP:0000297 (facial hypotonia); HP:0000670 (dental caries); HP:0000028 (Cryptorchidism, maldescensus of right testicle); HP:0012203 (onychomycosis, fingernails); HP:0007957 (corneal opacity); HP:0000613 (photophobia, severe) |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079126 |
| 0000059089 |
HP:0007503 (Generalized Ichthyosis);
HP:0000989 (Pruritus);
HP:0012758 (Neurodevelopmental delay); HP:0001258 (Spastic paraplegia); HP:0007702 (Pigmentary retinal deposits, "white glistening dots"); HP:0007266 (Cerebral dysmyelination); |
- |
- |
Familial, autosomal recessive |
07y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079361 |
| 0000059293 |
HP:0007503 (Generalized Ichthyosis);
HP:0000989 (Pruritus);
HP:0012758 (Neurodevelopmental delay); HP:0001258 (Spastic paraplegia); HP:0007702 (Pigmentary retinal deposits, "white glistening dots"); HP:0007266 (Cerebral dysmyelination) |
- |
- |
Familial, autosomal recessive |
14y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079569 |
| 0000059294 |
HP:0007503 (Generalized Ichthyosis);
HP:0000989 (Pruritus);
HP:0012758 (Neurodevelopmental delay); HP:0001258 (Spastic paraplegia); HP:0007702 (Pigmentary retinal deposits, "white glistening dots"); HP:0007266 (Cerebral dysmyelination) |
- |
- |
Familial, autosomal recessive |
18y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079570 |
| 0000059357 |
HP:0007503 (Generalized Ichthyosis);
HP:0000989 (Pruritus);
HP:0012758 (Neurodevelopmental delay); HP:0001258 (Spastic paraplegia); HP:0007266 (Cerebral dysmyelination) |
- |
- |
Familial, autosomal recessive |
02y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079633 |
| 0000059358 |
HP:0007503 (Generalized Ichthyosis);
HP:0000989 (Pruritus);
HP:0012758 (Neurodevelopmental delay); HP:0001258 (Spastic paraplegia); HP:0007266 (Cerebral dysmyelination) |
- |
- |
Familial, autosomal recessive |
01y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079634 |
| 0000059587 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, severe); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 35 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079874 |
| 0000059612 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability, severe); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
07y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079916 |
| 0000059613 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability, severe); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 36 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
09y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079917 |
| 0000059614 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability, IQ 55); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 38 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
09y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079918 |
| 0000059615 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, IQ 56); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 36,5 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
17y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079919 |
| 0000059616 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, IQ 54); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 37,5 weeks); HP:0002188 (Delayed CNS myelination |
- |
- |
Familial, autosomal recessive |
18y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00079920 |
| 0000060302 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability, IQ 50); HP:0030507 (retinal crystals); HP:0001622 (premature birth, 28 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
21y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080727 |
| 0000060333 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 34,5 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
02y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080754 |
| 0000060334 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, IQ 61); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 36,5 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
08y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080755 |
| 0000060335 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 35 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
11y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080756 |
| 0000060336 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, IQ 49); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 34 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
09y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080757 |
| 0000060337 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability, IQ 48); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 33 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
16y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00080758 |
| 0000060800 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 36 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
13y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081222 |
| 0000060801 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, moderate); HP:0001249 (intellectual disability, IQ 48); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, 35 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
14y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081223 |
| 0000060802 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
45y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081224 |
| 0000060803 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001257 (Spasticity, severe); HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
46y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081225 |
| 0000060804 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0000989 (Pruritus, moderate); HP:0001257 (Spasticity, mild); HP:0001249 (intellectual disability, IQ 83, mild); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
42y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081226 |
| 0000060805 |
HP:0007503 (Generalized Ichthyosis, mild); HP:0000989 (Pruritus, mild); HP:0001249 (intellectual disability, IQ 84, mild); HP:0030507 (retinal crystals); HP:0000613 (photophobia); HP:0001622 (premature birth, <37 weeks); HP:0002188 (Delayed CNS myelination) |
- |
- |
Familial, autosomal recessive |
46y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081227 |
| 0000060806 |
HP:0007503 (Generalized Ichthyosis); HP:0001249 (intellectual disability); HP:0001250 (seizures); HP:0001264 (spastic diplegia); HP:0007663 (reduced visual acuity, 0,5 in both eyes); HP:0030507 (retinal crystals); HP:0007305 (CNS demyelination, periventricular white matter) |
- |
- |
Familial, autosomal recessive |
30y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081228 |
| 0000060807 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001257 (Spasticity, unable to walk); HP:0001249 (intellectual disability, moderate); HP:0001250 (seizures); HP:0007305 (CNS demyelination, periventricular); HP:0001622 (premature birth, 32 weeks);
HP:0006801 (hyperact. deep tendon refl.) |
- |
- |
Familial, autosomal recessive |
12y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081229 |
| 0000060808 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus); HP:0001257 (Spasticity); HP:0002510 (spastic tetraplegia); HP:0001249 (intellectual disability);HP:0007305 (CNS demyelination, periventricular); HP:0030507 (retinal crystals);
HP:0006801 (hyperact. deep tendon refl.) |
- |
- |
Familial, autosomal recessive |
20y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081230 |
| 0000060809 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0001622 (premature birth, 34 weeks); HP:0001257 (Spasticity, more prominent in lower extremities); HP:0001249 (intellectual disability); HP:0002188 (Delayed CNS myelination, periventricular);
HP:0006801 (hyperactive deep tendon refl.) |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081231 |
| 0000060810 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0001250 (seizures); HP:0001249 (intellectual disability); HP:0002188 (Delayed CNS myelination, periventricular); HP:0002510 (spastic tetraplegia);
HP:0006801 (hyperactive deep tendon refl.) |
- |
- |
Familial, autosomal recessive |
06y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081232 |
| 0000060811 |
HP:0007503 (Generalized Ichthyosis); HP:0001257 (Spasticity, more prominent in lower extremities); HP:0007266 (Cerebral dysmyelination, periventricular, very mild); abnormal H-MRS lipid peaks at 1.3 ppm (prominent) and 0.9 ppm (small, narrow);
HP:0006801 (hyperact. deep tendon refl.) |
- |
- |
Familial, autosomal recessive |
05y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081233 |
| 0000060812 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus); HP:0001622 (premature birth, 32 weeks); HP:0001250 (seizures, associated with hypocalcemia); HP:0001270 (motor delay); HP:0000750 (Delayed speech and language development); HP:0002510 (Spastic tetraplegia); HP:0001249 (intellectual disability); HP:0007266 (Cerebral dysmyelination); HP:0005750 (Contractures of the joints of the lower limbs); HP:0002015 (dysphagia); HP:0000498 (Blepharitis); no retinal abnormalities! |
- |
- |
Familial, autosomal recessive |
24y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081234 |
| 0000060813 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus); HP:0009237 (Short 5th finger, bilaterally); HP:0001264 (Spastic diplegia); HP:0008936 (Muscular hypotonia of the trunk, problems with sitting); HP:0001250 (seizures); HP:0001622 (premature birth, 36 weeks); no ocular or visual abnormalities! |
- |
- |
Familial, autosomal recessive |
01y07m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081235 |
| 0000060814 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0002510 (Spastic tetraplegia); HP:0001249 (intellectual disability); HP:0030507 (retinal crystals, "white dots") |
- |
- |
Familial, autosomal recessive |
01y06m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081236 |
| 0000060815 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0002510 (Spastic tetraplegia, severe); HP:0001263 (Global developmental delay); HP:0001249 (intellectual disability, severe); HP:0001250 (seizures); HP:0030507 (retinal crystals, "white dots"); HP:0000613 (photophobia, severe); HP:0000316 (hypertelorism); HP:0000268 (Dolichocephaly); HP:0000527 (Long eyelashes); HP:0010743 (short metatarsal, D3,D4 and D5); HP:0002267 (Exaggerated startle response); HP:0007266 (Cerebral dysmyelination) |
- |
- |
Familial, autosomal recessive |
>01y06m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081237 |
| 0000060816 |
HP:0007503 (generalized ichthyosis); "neurologic symptoms typical of SLS"; HP:0001264 (spastic diplegia);
HP:0001249 (intellectual disability, severe); more phenotype data available: PMID 1836061 |
- |
- |
Familial, autosomal recessive |
32y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081238 |
| 0000060817 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
34y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081239 |
| 0000060818 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081240 |
| 0000060819 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
03y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081241 |
| 0000060820 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
01y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081243 |
| 0000060821 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
21y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081242 |
| 0000060822 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
19y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081244 |
| 0000060823 |
HP:0007503 (Generalized Ichthyosis); "neurologic symptoms typical of SLS" |
- |
- |
Familial, autosomal recessive |
15y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081245 |
| 0000060824 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity);
HP:0001249 (intellectual disability, with severe speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
76y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081246 |
| 0000060825 |
HP:0007503 (Generalized Ichthyosis); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (moderate);
HP:0005656 (Positional foot deformity, severe);
HP:0001249 (intellectual disability, with severe speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
70y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081247 |
| 0000060826 |
HP:0007503 (Generalized Ichthyosis); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe);
HP:0001249 (intellectual disability, with moderate speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
66y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081248 |
| 0000060827 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (moderate);
HP:0005656 (Positional foot deformity, severe);
HP:0001249 (intellectual disability, with severe speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
63y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081249 |
| 0000060828 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (moderate);
HP:0005656 (Positional foot deformity, severe);
HP:0001249 (intellectual disability, with mild speech disorder); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
56y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081250 |
| 0000060829 |
HP:0007503 (Generalized Ichthyosis); HP:0000982 (Palmoplantar keratoderma); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, moderate); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate);
HP:0001249 (intellectual disability, with mild speech disorder); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
53y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081251 |
| 0000060830 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe); Motor disability in arms (mild); HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
51y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081252 |
| 0000060831 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair-bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with severe speech disorder); HP:0001250 (seizures); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
47y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081253 |
| 0000060832 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
46y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081254 |
| 0000060833 |
HP:0007503 (Generalized Ichthyosis); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with severe speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
45y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081255 |
| 0000060834 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
45y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081256 |
| 0000060835 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with severe speech disorder); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
43y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081257 |
| 0000060836 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with severe speech disorder); HP:0001250 (seizures); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
43y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081258 |
| 0000060837 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with moderate speech disorder); HP:0001250 (seizures); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
43y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081259 |
| 0000060838 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with moderate speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
40y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081260 |
| 0000060839 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with severe speech disorder); HP:0001250 (seizures); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
38y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081261 |
| 0000060840 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, moderate); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, mild); HP:0001249 (intellectual disability, with moderate speech disorder); HP:0001250 (seizures); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
34y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081262 |
| 0000060841 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe); Motor disability in arms (mild); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
30y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081263 |
| 0000060842 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
29y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081264 |
| 0000060843 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
28y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081266 |
| 0000060844 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with severe speech disorder); HP:0001250 (seizures); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
26y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081267 |
| 0000060845 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, moderate); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, mild); HP:0001249 (intellectual disability, mild, no speech disorder!); HP:0001250 (seizures); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
23y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081268 |
| 0000060846 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0001264 (Spastic diplegia, severe, wheelchair bound); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, severe); HP:0001249 (intellectual disability, with moderate speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
17y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081269 |
| 0000060847 |
HP:0007503 (Generalized Ichthyosis); HP:0000989 (Pruritus); HP:0000982 (Palmoplantar keratoderma); HP:0001264 (Spastic diplegia, severe); Motor disability in arms (mild);
HP:0005656 (Positional foot deformity, moderate); HP:0001249 (intellectual disability, with mild speech disorder); HP:0000966 (Hypohidrosis); HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
08y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081270 |
| 0000060849 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, severe);
HP:0001249 (intellectual disability, mild) |
- |
- |
Familial, autosomal recessive |
12y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081274 |
| 0000060850 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, mild);
HP:0001249 (intellectual disability, mild) |
- |
- |
Familial, autosomal recessive |
05y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081275 |
| 0000060854 |
HP:0007503 (Generalized Ichthyosis); HP:0002510 (Spastic tetraplegia); HP:0001622 (premature birth, 34 weeks);
HP:0001249 (intellectual disability); HP:0001250 (seizures); HP:0030507 (retinal crystals);HP:0007266 (Cerebral dysmyelination, periventricular) |
- |
- |
Familial, autosomal recessive |
02y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081279 |
| 0000060855 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001249 (intellectual disability, severe); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0003781 (Excessive salivation) |
- |
- |
Familial, autosomal recessive |
08y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081280 |
| 0000060856 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus, severe); HP:0001249 (intellectual disability, severe); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0003781 (Excessive salivation) |
- |
- |
Familial, autosomal recessive |
06y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081281 |
| 0000060857 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0000989 (Pruritus, moderate); HP:0001249 (intellectual disability, moderate); HP:0002385 (paraparesis, ambulatory with support); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia) |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081282 |
| 0000060858 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0001249 (intellectual disability, severe); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
25y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081283 |
| 0000060859 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0001249 (intellectual disability, moderate); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
10y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081284 |
| 0000060860 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0000989 (Pruritus); HP:0001249 (intellectual disability, severe); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
14y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081285 |
| 0000060861 |
HP:0007503 (Generalized Ichthyosis, severe); HP:0000989 (Pruritus); HP:0001249 (intellectual disability, moderate); HP:0002385 (paraparesis, ambulatory with support); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia); HP:0000613 (photophobia); HP:0007663 (Reduced visual acuity); HP:0000821 (Hypothyroidism) |
- |
- |
Familial, autosomal recessive |
30y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081286 |
| 0000060862 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0001249 (intellectual disability, severe); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia); HP:0000613 (photophobia); HP:0007663 (Reduced visual acuity); HP:0000821 (Hypothyroidism) |
- |
- |
Familial, autosomal recessive |
28y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081287 |
| 0000060863 |
HP:0007503 (Generalized Ichthyosis, moderate); HP:0000989 (Pruritus); HP:0001249 (intellectual disability, moderate); HP:0001264 (Spastic diplegia, severe, wheelchair bound); HP:0006801 (Hyperactive deep tendon reflexes); HP:0001276 (Hypertonia);
HP:0030507 (retinal crystals); HP:0000613 (photophobia) |
- |
- |
Familial, autosomal recessive |
21y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081288 |
| 0000060864 |
HP:0007503 (Generalized Ichthyosis,
Extremities: severe,
Trunk: severe
Face: mild);
HP:0002313 (Spastic paraparesis, moderate, moderate gait abnormality);
HP:0005750 (Contractures of the joints of the lower limbs, mild)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, right: 6/12, left: 6/18);
HP:0030507 (retinal crystals);
HP:0000551 (Abnormality of color vision, mild);
HP:0007266 (Cerebral dysmyelination, moderate, frontal, parietal and periventricular);
H-MRS lipid peak at 1.3 ppm (Lipid/Creatine-Ratio: 1.3) |
- |
- |
Familial, autosomal recessive |
16y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081292 |
| 0000060865 |
HP:0007503 (Generalized Ichthyosis,
Extremities: severe,
Trunk: severe);
HP:0002313 (Spastic paraparesis, moderate, moderate gait abnormality);
HP:0005750 (Contractures of the joints of the lower limbs, moderate)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, right: 6/36, left: 6/18);
HP:0030507 (retinal crystals);
HP:0000551 (Abnormality of color vision, moderate) |
- |
- |
Familial, autosomal recessive |
19y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081293 |
| 0000060866 |
HP:0007503 (Generalized Ichthyosis,
Extremities: moderate,
Trunk: moderate,
Face: mild);
HP:0002313 (Spastic paraparesis, severe, marked gait disturbance);
HP:0005750 (Contractures of the joints of the lower limbs, moderate)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, right: 6/36, left: 6/36);
HP:0030507 (retinal crystals);
HP:0007266 (Cerebral dysmyelination, mild, frontal, occipital and periventricular);
H-MRS lipid peak at 1.3 ppm (Lipid/Creatine-Ratio: 1.2) |
- |
- |
Familial, autosomal recessive |
24y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081294 |
| 0000060867 |
HP:0007503 (Generalized Ichthyosis,
Extremities: moderate,
Trunk: severe
Face: mild);
HP:0002313 (Spastic paraparesis, severe, marked gait disturbance, wheelchair);
HP:0005750 (Contractures of the joints of the lower limbs, moderate)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, right: 6/18, left: 6/24);
HP:0030507 (retinal crystals);
HP:0000551 (Abnormality of color vision, mild);
HP:0007266 (Cerebral dysmyelination, severe, frontal, parietal, temporal, occipital and periventricular);
H-MRS lipid peak at 1.3 ppm (Lipid/Creatine-Ratio: 0.7) |
- |
- |
Familial, autosomal recessive |
33y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081295 |
| 0000060868 |
HP:0007503 (Generalized Ichthyosis,
Extremities: severe,
Trunk: severe);
HP:0002313 (Spastic paraparesis, severe, marked gait disturbance);
HP:0005750 (Contractures of the joints of the lower limbs, moderate)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, right: 6/18, left: 6/36);
HP:0030507 (retinal crystals) |
- |
- |
Familial, autosomal recessive |
34y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081296 |
| 0000060869 |
HP:0007503 (Generalized Ichthyosis,
Extremities: moderate,
Trunk: mild,
Face: mild);
HP:0002313 (Spastic paraparesis, moderate, moderate gait abnormality);
HP:0005750 (Contractures of the joints of the lower limbs, moderate)
HP:0001249 (intellectual disability, mild); HP:0001260 (Dysarthria, mild);
HP:0007663 (Reduced visual acuity, left: 6/36);
HP:0030507 (retinal crystals, severe);
HP:0000551 (Abnormality of color vision, moderate);
HP:0007266 (Cerebral dysmyelination, moderate, frontal, occipital and periventricular);
H-MRS lipid peak at 1.3 ppm (Lipid/Creatine-Ratio: 0.5) |
- |
- |
Familial, autosomal recessive |
36y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081297 |
| 0000060871 |
HP:0007503 (Generalized Ichthyosis); HP:0001249 (intellectual disability); HP:0002313 (Spastic paraparesis); HP:0001276 (Hypertonia, lower limbs);
HP:0007266 (Cerebral dysmyelination, post angular area of left parietal lobe) |
- |
- |
Familial, autosomal recessive |
06y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081298 |
| 0000060872 |
HP:0007503 (Generalized Ichthyosis, brown-colored pigmentation); HP:0001249 (intellectual disability); HP:0002313 (Spastic paraparesis) |
- |
- |
Familial, autosomal recessive |
01y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081299 |
| 0000060873 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, ambulatory);
HP:0001249 (intellectual disability, mild) |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081300 |
| 0000060874 |
HP:0007503 (Generalized Ichthyosis, collodion membrane at birth);
HP:0001264 (Spastic diplegia, non-ambulatory);
HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
01y03m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081301 |
| 0000060875 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia);
HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
02y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081302 |
| 0000060876 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, non-ambulatory);
HP:0001249 (intellectual disability, moderate) |
- |
- |
Familial, autosomal recessive |
02y06m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081303 |
| 0000060877 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, non-ambulatory);
HP:0001249 (intellectual disability, mild) |
- |
- |
Familial, autosomal recessive |
02y06m |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081304 |
| 0000060878 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, ambulatory);
HP:0001249 (intellectual disability, moderate) |
- |
- |
Familial, autosomal recessive |
05y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081305 |
| 0000060879 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia, ambulatory);
HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
28y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081306 |
| 0000060880 |
fetus!
no phenotype information available |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081307 |
| 0000060881 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia);
HP:0001249 (intellectual disability) |
- |
- |
Familial, autosomal recessive |
04y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081309 |
| 0000060882 |
fetus!
no phenotype information available |
- |
- |
Familial, autosomal recessive |
- |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081310 |
| 0000060883 |
HP:0007503 (Generalized Ichthyosis); HP:0001264 (Spastic diplegia);
HP:0001249 (intellectual disability, moderate) |
- |
- |
Familial, autosomal recessive |
14y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081311 |
| 0000060885 |
HP:0007503 (Generalized Ichthyosis, severe);
HP:0007325 (Generalized dystonia, severe); HP:0001257 (Spasticity, severe, with "twisting spasms");
HP:0008936 (Muscular hypotonia of the trunk, severe);
HP:0001347 (Hyperreflexia, both lower limbs and right arm);
HP:0002312 (Clumsiness);
HP:0000369 (low-set ears);
HP:0012758 (Neurodevelopmental delay, severe);
HP:0001249 (intellectual disability, severe); HP:0001622 (premature birth, 36 weeks); HP:0007305 (CNS demyelination, frontal, parietal, occipital, periventricular) |
- |
- |
Familial, autosomal recessive |
08y |
- |
- |
- |
- |
Maximilian Weustenfeld |
00081313 |