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Phenotypes for disease #00244 (MYOP (myopathy (MYOP)))
Legend
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Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
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878 entries on 9 pages. Showing entries 1 - 100.
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Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000002972
-
-
-
Unknown
-
-
-
-
-
Nyamkhishig Sambuughin
00004155
0000003237
mtDNA depletion myopathy
-
-
Familial, autosomal recessive
-
-
-
-
-
Robert McFarland
00004540
0000041561
myotonic dystrophie like
-
-
Unknown
-
-
-
-
-
Mirjam Larsen
00054897
0000041562
myotonic dystrophy like
-
-
Unknown
-
-
-
-
-
Mirjam Larsen
00054896
0000041563
myotonic dystrophy like
-
-
Isolated (sporadic)
-
-
-
-
-
Mirjam Larsen
00054898
0000042780
myopathy, proximal dominant; CPK 811
-
-
Isolated (sporadic)
-
-
32y
-
-
Johan den Dunnen
00056142
0000042901
myopathy
-
-
Unknown
-
34y
22y
leg weakness
-
Frank Celeste
00056263
0000042902
myopathy
-
-
Unknown
-
30y
27y
left hand weakness
-
Frank Celeste
00056264
0000042903
myopathy
-
-
Unknown
-
28y
25y
foot drop
-
Frank Celeste
00056265
0000042904
myopathy
-
-
Unknown
-
50y
23y
-
-
Frank Celeste
00056266
0000042905
myopathy
-
-
Unknown
-
30y
19y
left hand weakness
-
Frank Celeste
00056267
0000042906
myopathy
-
-
Unknown
-
24y
23y
trouble with stairs
-
Frank Celeste
00056268
0000042907
myopathy
-
-
Unknown
-
25y
30y
-
-
Frank Celeste
00056269
0000042908
myopathy
-
-
Unknown
-
39y
23y
foot drop when exercising
-
Frank Celeste
00056270
0000042986
mild, adult onset
-
-
Unknown
-
-
-
-
-
Olivera Casar-Borota
00056373
0000050846
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Carola Hedberg-Oldfors
00063263
0000053372
Muscular hypotrophy, cognitive and psychomotor delay, epilepsy, congenital cataract, scoliosis, lactic acidosis and deficiency of the mitochondrial respiratory chain
-
-
Familial, autosomal recessive
-
-
00y09m
-
-
Sophie Nambot
00073635
0000053375
muscular hypotrophy and hypotonia, psychomotor delay, ID, congenital cataract,lactic acidosis, mitochondrial disorders
-
-
Familial, autosomal recessive
-
-
-
-
-
Sophie Nambot
00073639
0000058300
Laing distal myopathy-like phenotypes with variable expressivity and neck extensor contracture; myopathy, scapuloperoneal, MYH7-related (SPMM)
-
-
Familial, autosomal recessive
-
-
-
-
-
Yonatan Perez
00078525
0000058654
myopathy, congenital; increasing falls and decreasing exercise tolerance (wheelchair for long distances); weakness distal>proximal, hand intrinsics most involved; no eye muscle involvement, no respiratory involvement, no cardiac involvement; normal school performance; prominent myalgias; normal EMG; biopsy type I fiber predominance, core-like areas, central nuclei >25% fibers; normal; normal CPK (-HP:0003236); areas DES/actin immunopositivity
-
-
Familial, autosomal dominant
-
-
-
neonatal hypotonia, delayed motor development
-
Johan den Dunnen
00078898
0000058655
myopathy, congenital; no progression; diffuse weakness; no eye muscle involvement, no respiratory involvement, no cardiac involvement; delayed cognitive development; prominent myalgias; no EMG; type I fiber predominance, core-like areas, central nuclei 10% fibers; mild MR; normal CPK (-HP:0003236); DES diffusely positive cytoplasmic staining
-
-
Familial, autosomal dominant
-
-
-
neonatal hypotonia, delayed motor development
-
Johan den Dunnen
00078899
0000058656
myopathy, congenital; worsening exercise tolerance (ambulation independent); mild distal only weakness; ; no eye muscle involvement, no respiratory involvement, no cardiac involvement; required special education; myalgias and muscle cramping; normal EMG; fiber atrophy (type I and II), core-like areas; mild MR; normal CPK (-HP:0003236); DES positive cytoplasmic staining
-
-
Familial, autosomal dominant
-
-
-
increased falls, abnormal gait
-
Johan den Dunnen
00078900
0000058657
myopathy, congenital; progressive gait difficulties (requires assistance with prolonged ambulation); weakness distal>proximal, LE>UE; no eye muscle involvement, no respiratory involvement, no cardiac involvement; required special education; ankylosing spondylitis; epilepsy; mild MR; increased CPK (HP:0003236) 129-355 IU/L
-
-
Familial, autosomal dominant
-
-
-
developmental delay
-
Johan den Dunnen
00078901
0000058658
myopathy, congenital; worsening exercise intolerance; weakness mild distal only in UE, mild diffuse weakness in LE; no eye muscle involvement, no respiratory involvement, no cardiac involvement; mild cognitive difficulties; mild MR; normal CPK (-HP:0003236)
-
-
Familial, autosomal dominant
-
-
-
poor athletic skills, frequent falls
-
Johan den Dunnen
00078902
0000078456
suspected mitochondrial myopathy manifested as progressive muscle weakness, hypotonia, seizures, poor weight gain, lactic acidosis
-
-
Familial, autosomal recessive
07y
-
02y
toe-walking, Gowers sign
-
Johan den Dunnen
00024128
0000079414
myopathy
-
-
Unknown
-
-
-
-
-
Arnold Reuser
00101193
0000082241
myopathy, desmin-related (DRM)
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104300
0000082243
myopathy, desmin-related, Mallory body-like inclusions
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00104302
0000082256
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104324
0000082261
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104329
0000082262
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104330
0000082264
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00104332
0000082305
myopathy, scoliosis, reduced muscle mass; biopsy with myopathic and dystrophic changes
-
-
Unknown
-
-
-
-
-
Tom Winder
00104373
0000082392
core myopathy
-
-
Unknown
-
-
-
-
-
Tom Winder
00104460
0000082422
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Tom Winder
00104490
0000082426
congenital hypotonia and weakness with sparing of ocular muscles, prominent neck weakness, scoliosis, nocturnal BiPAP support.
-
-
Familial, autosomal recessive
-
-
-
-
-
Tom Winder
00104494
0000083982
dominant congenital myopthy; patients show generalized proximal weakness in early childhood; non-progressive
-
-
Familial, autosomal dominant
-
-
-
-
-
Kristen Nowak
00106167
0000083988
AM; Patient # 2 in Goebel et al., 1997
-
-
Isolated (sporadic)
3m
-
-
-
-
Kristen Nowak
00106173
0000083989
AM; 2.5y-floppy, very limited movement, dependent on a respirator
-
-
Isolated (sporadic)
-
-
-
-
-
Kristen Nowak
00106174
0000084037
intranuclear rod myopathy
-
-
Isolated (sporadic)
-
-
-
-
-
Kristen Nowak
00106222
0000084041
AM
-
-
Unknown
5m
-
-
-
-
Kristen Nowak
00106226
0000084042
AM, severe
-
-
Isolated (sporadic)
6m
-
-
-
-
Alan Beggs
00106227
0000084043
AM, severe; deceased when ventilator withdrawn
-
-
Unknown
2m15d
-
-
-
-
Kristen Nowak
00106228
0000084044
AM
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106229
0000084046
intranuclear rod myopathy
-
-
Isolated (sporadic)
-
-
-
-
-
Kristen Nowak
00106231
0000084048
intranuclear rod myopathy
-
-
Unknown
-
-
-
-
-
Alan Beggs
00106233
0000084049
AM; IRM; Patient 1 in Goebel et al., 1997
-
-
Isolated (sporadic)
7y6m
-
-
-
-
Kristen Nowak
00106234
0000084050
AM; IRM
-
-
Unknown
4m
-
-
-
-
Kristen Nowak
00106235
0000084051
intranuclear rod myopathy
-
-
Familial, autosomal dominant
-
-
-
-
-
Kristen Nowak
00106236
0000084134
intranuclear rod myopathy
-
-
Unknown
2y6m
-
-
-
-
Kristen Nowak
00106319
0000084136
AM
-
-
Isolated (sporadic)
10y
-
-
-
-
Kristen Nowak
00106321
0000084147
intranuclear rod myopathy
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106332
0000084226
intranuclear rod myopathy; deceased; Email from Francesco 6.1.09
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106422
0000084242
cap myopathy; pregnancy reduced fetal movements; birth weight 3160g, 39w, caesarian section; respiratory efforts (1h intubated), weak cry, low hairline, micrognathia, high arched palate, single palmar crease, long fingers, undescended testes; GJ tube and tracheostomy; 4y11m-hypotonic, generalized muscle atrophy, muscle strength MRC2/5 (upper)/1/5 (lower extremities), absent deep tendon reflexes; died cardiac arrest
-
-
Isolated (sporadic)
5y
-
-
-
-
Johan den Dunnen
00106438
0000084252
myopathy, congenital
-
-
Isolated (sporadic)
-
-
-
-
-
Tom Winder
00106448
0000084253
myopathy, actin; akinesia, foetal
-
-
Isolated (sporadic)
1d
-
-
-
-
Kristen Nowak
00106449
0000084256
myopathy; cardiomyopathy
-
-
Unknown
-
-
-
-
-
Tom Winder
00106452
0000084259
myopathy
-
-
Unknown
-
-
-
-
-
Tom Winder
00106455
0000084260
myopathy, congenital
-
-
Unknown
-
-
-
-
-
Tom Winder
00106456
0000084263
congenital myopathy with fibre type disproportion, normal CK values; CPK: normal
-
-
Isolated (sporadic)
-
-
<0d
-
-
Wolfram Kress
00106459
0000084269
Congenital myopathy; Hypotonic from birth. Knee flexion and hip flexion contractures, predominantly proximal weakness.; CPK: 38
-
-
Isolated (sporadic)
-
-
1d
-
-
Thomas Cullup
00106465
0000084284
congenital myopathy with CFTD; decreased fetal movements, hypotonia, delayed motor milestones, joint laxity; muscle weakness: facial, axial, proximal mostly; CPK: normal
-
-
Familial, autosomal dominant
-
-
>1m
-
-
Thomas Cullup
00106480
0000084285
myopathy
-
-
Familial, autosomal dominant
-
-
-
-
-
Tom Winder
00106481
0000084294
myopathy, congenital; died at 4m
-
-
Unknown
4m
-
-
-
-
Kristen Nowak
00106490
0000084295
myopathy, congenital; facial diplegia, poor gag & swallowing, marked truncal and neck hypotonia, reflexes present but diminished
-
-
Isolated (sporadic)
-
-
-
-
-
Kristen Nowak
00106491
0000084297
myopathy, congenital; dilated cardiomyopathy (severe)
-
-
Unknown
-
-
-
-
-
Tom Winder
00106493
0000084308
intranuclear rod myopathy,u atonomic dysfunction
-
-
Isolated (sporadic)
-
-
1d
-
-
Kristen Nowak
00106504
0000084313
congenital myopathy; Muscle biopsy with core myopathy, increased internal nuclei, type 1 predominance. Other family members not yet analysed
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106509
0000084314
congenital myopathy
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106510
0000084315
myotubular myopathy;e tsting other familiy members not done yet
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106511
0000084478
Congenital myopathy - no rods
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106681
0000084480
Profoundly hypotonic at birth with no perceivable movements, other than eye movements. Discharged at 4 months of age on mechanical ventilation. Last examination described at age 5: some flicker movements of fingers and toes
-
-
Unknown
00y00m00d
00y00m00d
-
-
-
Kristen Nowak
00106683
0000084481
Fraternal twins described by Donkervoort et al 2017 with cytoplasmic body myopathy. Both twins had evidence of facial weakness, generalized hypotonia with marked head lag, and severe weakness with only minimal movements of the fingers and toes. Extraocular movements were normal
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106684
0000084536
Described as congenital myopathy
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106739
0000084537
Described as congenital myopathy with de novo ACTA1 mutation
-
-
Unknown
-
-
-
-
-
Kristen Nowak
00106740
0000085333
Patient diagnosed with congenital myopathy. First muscle biopsy stated to contain zebra-body like structures, thickened Z lines myofibrillar disorganisation. Second biopsy did not show zebra bodies. Totally asymptomatic father showed same ACTA1 c.529A>G (p.Ile177Val) variant with no sign of mosaicism from Sanger sequencing
-
-
Familial
-
-
-
-
-
Kristen Nowak
00107542
0000085788
idiopathic hyperCKemia (CK 2500-4000)
idiopathic hyperCKemia
-
Isolated (sporadic)
23y
-
-
-
-
Mireille Cossee
00108193
0000086353
-
-
-
Unknown
-
-
-
-
-
Tom Winder
00108880
0000086355
congenital myopathy
-
-
Unknown
-
-
-
-
-
Tom Winder
00108882
0000087158
diagnosed with zebra body myopathy, has affected daughter
-
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00106658
0000092790
dystrophic myopathy
myopathy
dystrophy, muscular, Duchenne type (DMD)
Unknown
-
-
-
-
-
Johan den Dunnen
00034402
0000109715
no loss ability to walk (-HP:0006957)
myopathy, quadriceps
dystrophy, muscular, Becker type (BMD)
Unknown
-
-
7y
large claves
WB 50% DMD
Johan den Dunnen
00136966
0000110082
-
myopathy
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00137334
0000110083
-
myopathy
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00137335
0000110381
no loss ability to walk (-HP:0006957)
myopathy, quadriceps
dystrophy, muscular, Becker type (BMD)
Unknown
-
-
8y
poor running
WB 60% DMD
Johan den Dunnen
00137633
0000110479
elevated serum CPK (HP:0003236); no loss ability to walk (-HP:0006957)
myopathy, quadriceps
dystrophy, muscular, Becker type (BMD)
Unknown
-
-
-
-
WB 20% DMD
Johan den Dunnen
00137731
0000111347
-
myopathy, quadriceps
dystrophy, muscular, Becker type (BMD)
Unknown
-
43y
-
-
-
Johan den Dunnen
00138599
0000118848
-
-
-
Unknown
-
-
-
-
-
Aparna Ganapathy
00144663
0000119203
congenital autophagic vacuolar myopathy; see paper; ..., 5/7 died soon after birth due to inability to breathe and suckle
-
-
Familial, X-linked recessive
-
-
-
-
-
Johan den Dunnen
00146462
0000124996
myopathy
myopathy
-
Unknown
-
-
-
-
IHC no LAMA2
Tom Winder
00102323
0000124997
myopathy; sarcotubular proliferation
myopathy
-
Unknown
-
-
-
-
-
Johan den Dunnen
00102518
0000124998
Elevated serum creatine phosphokinase (HP:0003236), proximal muscle weakness in lower limbs (HP:0008994) and upper limbs (HP:0008997), joint contracture (HP:0001371), calf muscle hypertrophy (HP:0008981), dilated cardiomyopathy (HP:0001644), hyperintensity of cerebral white matter on MRI (HP:0030890)
myopathy
-
Familial, autosomal recessive
49y
-
06y
Stiff elbow (HP:0025259) and spinal rigidity (HP:0003306)
muscle, IHC for LAMA2, residual expression
Jorge Oliveira
00103327
0000124999
Elevated serum creatine phosphokinase (HP:0003236), proximal muscle weakness in lower limbs (HP:0008994) and upper limbs (HP:0008997), joint contracture (HP:0001371), hyperintensity of cerebral white matter on MRI (HP:0030890)
myopathy
-
Familial, autosomal recessive
-
-
02y06m
Frequent falls (HP:0002359), delayed gross motor development (HP:0002194)
-
Jorge Oliveira
00103461
0000125746
see paper; ..., atypical mild clinical feature and slow progression; 46y-left foot drop, EMG and muscle biopsy left tibialis anterior muscle compatible with myopathy
myopathy
-
Familial, autosomal recessive
48y
-
-
-
-
Johan den Dunnen
00153018
0000130169
congenital myopathy, severe
-
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00165290
0000130170
congenital myopathy, severe
-
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00165291
0000130171
predominant axial and limb girdle weakness; 13y-severe kyphoscoliosis; CPK 134 U/l; 6m-sit, 11m-crawl, 2y6m-walk; 21y-loss ambulation
myopathy, congenital
-
Isolated (sporadic)
-
-
15y
keeping head straight
-
Johan den Dunnen
00165292
0000130172
predominant axial and limb girdle weakness; 2y7m-hypotonia, hyperextension knees/elbows, waddling gait, Gowers’ sign; >5y-loss ambulation
myopathy, congenital
-
Isolated (sporadic)
-
-
2y7m
delayed motor development
-
Johan den Dunnen
00165293
0000130183
The patient presented with severe hypotonia, arthrogryposis, and femoral and humeral fractures, required cardiopulmonary resuscitation, and deceased at 13 days.
-
-
Familial, autosomal recessive
-
-
-
-
-
Johann Böhm
00165319
0000130184
Patients presented with profound neonatal hypotonia, minimal respiratory effort, arthrogryposis, and multiple bone fractures, and deceased shortly after birth.
-
-
Familial, autosomal recessive
-
-
-
-
-
Johann Böhm
00165320
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