Le Diko - en:symmetrical, proximal limb muscle atrophy (pelvic, scapular, trunk muscles)

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Le Diko - en:symmetrical, proximal limb muscle atrophy (pelvic, scapular, trunk muscles)

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en:symmetrical, proximal limb muscle atrophy (pelvic, scapular, trunk muscles)

Nom

Informations lexicales

Langue:anglais

Associations d'idées

1000

en:mild muscle atrophy in forearm muscles

•

en:impaired toe-walking

•

en:loss of truncal subcutaneous adipose tissue

•

•

en:axial hypotonia (in some patients)

•

en:calf hypertrophy (less common)

•

en:difficulty walking, running, climbing stairs

•

en:fatty replacement in hip muscles and proximal muscles of the lower limb seen on mri

•

en:complex v deficiency

•

en:muscle biopsy shows decreased glycosylation of alpha-dystroglycan

•

en:vacuolar membranes immunostain with sarcolemmal proteins

•

en:muscle weakness, proximal, limb-girdle

•

en:exercise-induced muscle stiffness

•

•

en:polymyositis

•

en:normal dystrophin immunostaining

•

en:muscle weakness after cooling or on warming affected muscle

•

en:some patients become wheelchair-dependent

•

en:autophagic rimmed vacuoles

•

en:cores or minicores

•

en:dystrophic features

•

en:generalized amyoplasia

•

•

en:normal sensation

•

en:sarcoplasmic accumulation of electron-dense granulofilamentous material

•

en:skeletal muscle weakness

•

en:electron microscopy shows dense material emanating from the z-disk

•

en:swelling of the affected areas

•

en:large lipid droplets

•

en:normal alpha-glucosidase or acid maltase activity (gaa, 606800)

•

en:absence of laminin alpha-2 chain in muscle

•

en:muscle weakness is predominantly of extremities and tongue

•

en:myopathy (rare)

•

en:impaired mitochondrial respiration

•

•

en:dystrophic changes

•

en:delayed motor development (cvs+)

•

en:distal limb muscle weakness, lower more than upper

•

en:decreased lipoic acid

•

en:fibrous dysplasia (rare)

•

en:lower limb muscle atrophy (in 2 of 3 families)

•

en:muscle weakness, symmetric, proximal, lower limbs

•

en:nemaline bodies (rods) on gomori trichrome staining seen on muscle biopsy

•

en:soft tissue or tendinous calcinosis may occur

•

en:distal leg muscle atrophy

•

en:inclusion body myopathy

•

en:distal muscles may be affected

•

en:myogenic changes seen on emg

•

en:inclusions are dnajb6-immunoreactive

•

en:necrotic and regenerating fibers

•

•

en:necrotic process

•

en:muscle coenzyme q deficiency

•

en:muscle biopsy shows multiple mitochondrial dna (mtdna) deletions

•

en:lack of integrin alpha-7 staining

•

en:increased intramuscular fat

•

en:predominance of oxidative fibers

•

en:emg shows polyphasic continuous motor unit discharges

•

en:lipomas or lipomatous masses (most cases)

•

en:muscle biopsy may show mild kinetic abnormalities of the achr

•

en:muscle hypertrophy (meb)

•

en:muscle weakness (cvs+)

•

•

en:neck and proximal limb muscle myopathy

•

en:bulbar weakness

•

en:shoulder muscle weakness

•

en:variation of fiber size and shape

•

en:casq1-immunopositive inclusions

•

en:deficiency of mitochondrial complexes i, iii, and iv

•

en:degenerate mitochondrial on em

•

en:muscle biopsy shows decreased activity of cytochrome c oxidase

•

en:normal or increased adipose tissue around the neck

•

en:secondary loss of merosin and desmin

•

en:upper limb muscle hypoplasia

•

en:rhabdomyolysis with exercise (in older patients)

•

en:congophilic staining

•

en:distal muscle wasting, mild

•

en:generalized muscle weakness also seen in symptomatic carrier females

•

en:myotonia is most pronounced in the extremities

•

en:preserved heel standing

•

en:neuropathic process seen on muscle biopsy

•

en:slim, muscular build (in some patients)

•

en:electromyogram abnormal

•

en:lower limb muscle atrophy

•

en:skeletal muscle biopsy shows minor variation in fiber size

•

en:walking delay

•

en:type ii fiber atrophy seen on muscle biopsy

•

en:increased mitochondria with rare paracrystalline inclusions

•

en:decreased collagen vi

•

en:muscle biopsy shows neurogenic atrophy

•

en:small or absent axon terminals

•

en:inability to sit or hold head up

•

en:muscle biopsy shows myofiber degeneration

•

en:muscle weakness (type i)

•

en:nemaline bodies (rods) on gomori trichrome staining

•

en:proximal lower limb muscle weakness, upper and lower (later onset)

•

en:subsarcolemmal lipofuscin-positive deposits

•

en:ubiquinone decreased

•

en:emg shows myopathy

•

en:disruption of sarcomeres with disorganization of the z band

•

en:inability to stand on tiptoes

•

en:loss of subcutaneous fat, particularly affecting the limbs

•

en:muscle fatigue

•

en:muscle weakness, distal, particularly affecting the hands

•

en:muscle weakness, predominantly proximal (in some patients)

•

en:muscle weakness increases with age

•

en:myopathic pattern seen on emg

•

en:minicores (in some)

•

en:emg shows myotonic discharges in the absence of clinical myotonia

•

en:fat accumulation in the suprabuttock area

•

en:tubulofilamentous inclusions

•

en:thigh hypertrophy

•

en:muscle biopsy shows neurogenic atrophy affecting both fibers types

•

en:no dystrophic changes

•

en:minicore regions are poorly defined and do not extend through entire fiber length

•

en:marked loss of subcutaneous fat in the limbs, face, and sometimes chest

•

en:increased numbers of abnormally-shaped mitochondria in subsarcolemmal areas

•

•

en:generalized muscle hypertrophy

•

en:shoulder weakness and atrophy

•

en:amyotrophy, distal, upper and lower limbs

•

en:decreased or absent ankle reflexes

•

en:depletion of mitochondrial dna

•

•

en:myopathy, mild to severe

•

en:red-ragged fibers (2p21del)

•

en:shortening of various muscle-tendon groups in legs

•

•

en:deposits immunoreactive to beta-amyloid protein

•

en:muscle weakness, distal, restricted to the lower limbs

•

en:dystrophic features and atrophic fibers seen on muscle biopsy

•

en:reduced muscle mass

•

en:no staining for oxidative enzymes

•

en:trunk muscle atrophy

•

en:muscle fatigability

•

en:mitochondrial dna depletion, severe

•

en:emg shows fibrillation potentials

•

en:muscle biopsy shows small rounded fibers

•

en:lipid and glycogen accumulation

•

en:muscle biopsy shows necrotic and regenerating fibers

•

en:muscle biopsy shows myopathic or dystrophic changes

•

en:muscle biopsy shows lipid deposition

•

en:muscle biopsy shows glycogen storage

•

en:muscle biopsy shows endplate myopathy (see details under neurologic heading)

•

en:muscle atrophy affecting the upper back and neck muscles

•

en:muscle atrophy (type i)

•

en:minimal subcutaneous fat

•

en:muscle atrophy (1 patient)

•

en:muscle amyotrophy

•

en:mitochondrial vacuolization seen on muscle biopsy

•

en:mitochondrial myopathy

•

en:mild variability in muscle fiber size

•

en:mild atrophy of the proximal lower limbs

•

en:marked lack of adipose tissue

•

en:mallory-like bodies

•

en:loss of facial adipose tissue

•

en:muscle pain after exercise

•

en:muscle biopsy shows type 2 fiber atrophy

•

en:proximal muscle weakness (occasional)

•

en:muscle paralysis, episodic

•

en:proximal amyotrophy

•

en:severe muscle hypotonia at birth

•

en:rhabdomyolysis (in some patients) endocrine : hypogonadotropic hypogonadism (rare)

•

en:rhabdomyolysis

•

en:retain some mechanical adipose tissue (joints, orbits, palms, soles)

•

en:rectus fascia separation

•

en:recovery between attacks

•

en:ragged-red muscle fibers

•

en:quadriceps atrophy

•

en:limb-girdle myopathy

•

en:premature exertional muscle weakness

•

en:muscle weakness, mild, proximal and distal

•

en:poor muscle mass

•

en:poor head control

•

en:no facial muscle involvement

•

en:normal mitochondrial morphology (1 patient)

•

en:neonatal hypertonias

•

en:muscle weakness and atrophy may be asymmetric

•

en:muscle weakness and atrophy, distal

•

en:muscle weakness, proximal, pelvic girdle (in severe cases)

•

en:muscle weakness, proximal, distal, and axial, severe

•

en:limb muscle weakness, proximal, upper and lower

•

en:fiber-type grouping pattern

•

en:indentations or folds of the sarcolemma are connected to the membranes enclosing the vacuoles

•

en:calf muscle enlargement

•

en:deficiency of mitochondrial respiratory enzymes seen on muscle biopsy

•

en:decreased respiratory complex i+iii and ii+iii activity

•

en:decreased postsynaptic folding at the neuromuscular junction

•

en:decreased muscle mass (especially of limbs)

•

en:decreased muscle mass, especially distally

•

en:decreased mitochondrial respiratory chain complex activity

•

en:decreased hand grip strength

•

en:combined mitochondrial respiratory chain deficiency (in most patients)

•

en:chronic denervation seen on emg

•

en:calf hypertrophy (uncommon)

•

en:disorganized myofilaments seen on muscle biopsy

•

en:calf hypertrophy (early in the disease)

•

en:biopsy shows rimmed vacuoles, central nuclei, and variation in fiber size

•

en:benign soft tissue developmental anomalies

•

en:attacks may present during or after sleep

•

en:atrophy of upper girdle muscles

•

en:ascites (in some patients)

•

en:absence of pectoralis minor muscle

•

en:abnormal mitochondria

•

en:symmetrical muscle wasting

•

en:difficulty rising from chair

•

en:distal muscle weakness, lower limbs more severely affected than upper limbs

•

en:increased variability of fiber size seen on muscle biopsy

•

en:fiber type variation seen on biopsy

•

en:increased muscle glycogen content

•

en:increased intraabdominal fat

•

en:increased glycogen in muscle

•

en:increased echo intensity in affected muscles

•

en:hypotonia, severe, neonatal

•

•

en:hematomas, recurrent large subcutaneous

•

en:generalized muscle wasting

•

en:gait abnormalities (occurs later)

•

en:fiber size variation seen on muscle biopsy

•

en:distal muscle weakness and atrophy of upper and lower limbs due to peripheral neuropathy

•

en:fiber necrosis

•

en:fat pad between achilles tendon and medial malleolus

•

en:fat pads on the hands and feet

•

en:emg shows myotonic changes

•

en:emg - repetitive muscle discharges

•

en:edema is present at birth and then slowly decreases

•

en:easy fatigability with exercise

•

en:easy bruisability of lower extremities

•

en:dystrophic features seen on muscle biopsy

•

en:subsarcolemmal accumulation of normal mitochondria (in some patients)

•

en:hip girdle muscle weakness (usually presenting symptom)

•

en:type 1 fiber atrophy seen on muscle biopsy

•

en:muscle biopsy shows nonspecific myopathic changes

•

en:myotonia seen on emg

•

en:myopathy, mild

•

en:myopathic pattern seen on emg (in some patients)

•

en:muscle weakness, distal more than proximal

•

en:muscle wasting (especially legs and arms)

•

en:muscle pain with exercise (in older patients)

•

en:muscle mounding

•

en:muscle biopsy shows small, membrane-bound vacuoles predominantly in type 2 fibers

•

en:muscle biopsy shows no dystrophic changes

•

en:muscle biopsy shows centralized nuclei

•

en:necrotic change

•

en:mri shows increased connective tissue and fat

•

en:mitochondrial swelling

•

en:lymphedema (lower limb)

•

en:weakness of long finger extensor muscles (occurs later)

•

en:lymphedema, hands (rare)

•

en:lower limbs more often affected

•

en:loss of ambulation (in some patients)

•

en:limb weakness, proximal followed by distal

•

en:limb-girdle muscular dystrophy

•

en:myxedematous appearance

•

en:nemaline rod formation

•

en:independent walking may not be achieved

•

en:short and thickened z-discs

•

en:upper and/or lower limbs may be affected

•

en:vacuolar myopathy

•

en:vacuolar degeneration

•

en:type 1 fiber atrophy

•

en:tubular aggregates mainly in type 1 fibers

•

en:triceps aplasia

•

•

en:soft fleshy palms (50%)

•

en:slowly-progressive muscle wasting and weakness with humeroperoneal and scapuloperoneal distribution

•

en:redundancy of basal lamina with accumulated debris

•

en:neurogenic changes later in disease seen on emg

•

en:reduced activity of mitochondrial respiratory chains (1 family)

•

en:rectus abdominis muscle separation

•

en:progressive proximal muscle weakness

•

en:progressive distal muscle weakness

•

en:poor exercise tolerance

•

en:pectoralis hypoplasia

•

en:onset of lymphedema around puberty

•

en:no lipodystrophy in face and neck

•

en:normal muscle fiber (hcs)

•

en:inguinal hernia

•

en:tremor at birth, involving most skeletal muscle groups, especially jaw muscles and lower limb muscles

•

en:inclusions stain positively with nitroblue tetrazolium (nbt)

•

en:ambulation difficulty

•

en:decreased muscle mass (predominantly in lower extremities)

•

en:decreased density of calcium release units

•

en:decreased cytochrome c oxidase activity

•

en:decreased activity of mitochondrial complex i

•

en:congophilic inclusions

•

en:chronic denervation seen on skeletal muscle biopsy

•

en:calf hypertrophy (in some patients)

•

en:atrophy, mild (in some cases)

•

en:angular atrophic fibers

•

en:acquire ability to walk and jump in childhood, but motor regression occurs later

•

en:deltoid muscle hypoplasia

•

en:absence of subcutaneous fat over entire body except buttocks, hips, and thighs

•

en:abnormal mitochondria with paracrystalline inclusions seen on muscle biopsy

•

en:abnormal mitochondrial morphology

•

en:abnormal fiber size variation seen on muscle biopsy

•

en:'hanging' big toe

•

en:variability in fiber size

•

en:vacuoles predominantly in type 2 fibers seen on muscle biopsy

•

en:vacuoles appear to be exocytosing material at the cell surface

•

en:inclusions form a lobulated structure seen on electron microscopy

•

en:degrading mitochondria

•

en:weakness of the intrinsic hand muscles (in some patients)

•

en:diastasis recti

•

en:firm muscles

•

en:disorganization of the neuromuscular junction

•

en:hypoplastic biceps

•

en:hypoplasia of infraspinatus muscle

•

en:hang weakness

•

en:granular deposits in the sarcomeres

•

en:glycogen accumulation seen on biopsy

•

en:gait disturbance

•

en:gait, drop foot

•

en:hip girdle muscle atrophy

•

en:fibromyalgia

•

en:emg shows myopathic or neurogenic changes

•

en:distal muscle atrophy due to peripheral neuropathy

•

en:fibrillation

•

en:edema at birth

•

•

en:emg shows reduction of voluntary recruitment

•

en:fatty deposition

•

en:fiber size variation

•

en:imaging shows muscle atrophy and fatty replacement

•

en:exercise intolerance (also seen in some heterozygotes)

•

en:myosin storage myopathy (in some patients)

•

en:proximal muscles may be mildly affected

•

en:proliferation of fatty tissue in muscle (in some patients)

•

en:peroneal muscle atrophy

•

en:paracrystalline inclusions

•

en:painful adipose tissue

•

en:nemaline bodies are usually subsarcolemmal or sarcoplasmic

•

en:myotonic discharges

•

en:muscle biopsy shows type i fiber predominance

•

en:muscle fiber necrosis

•

en:myopathy in approximately 45% of patients

•

en:muscle biopsy shows preserved junctional folds

•

en:myopathic changes on pathology

•

en:muscular hypotonia of the trunk

•

en:proximal myopathy

•

en:muscle weakness, proximal greater than distal

•

en:muscle wasting, severe, primarily in the legs

•

en:muscle hyperirritability

•

en:myopathic findings on biopsy have been occasionally reported

•

en:subsarcolemmal accumulations of abnormally shaped mitochondria (on electron microscopy)

•

en:pyruvate oxidation defect

•

en:tubular aggregates mainly in type 2 fibers and are subsarcolemmal or intermyofibrillar and stain for nadh-tetrazolium reductase

•

en:amyotrophy, neurogenic

•

en:muscle biopsy shows myofibrillar changes

•

en:adhalin deficiency seen on muscle biopsy

•

en:abnormal mitochondria in skeletal muscle biopsy

•

en:weakness of the anterior tibial muscle and toe extensors

•

en:weakness of neck extensors (rare)

•

en:tubulofilamentous nuclear or cytoplasmic inclusions (15-18 nm)

•

en:thenar amyotrophy (rare)

•

en:reduced muscle bulk

•

en:subsarcolemmal accumulations of abnormally shaped mitochondria seen with electronmicroscopy

•

en:spheroid bodies are composed of fine filamentous (myofibrillar) material

•

en:skeletal muscle tissue shows 14 to 45% depletion of mitochondrial dna (mtdna)

•

en:skeletal muscle biopsy shows dystrophic changes

•

en:scapuloperoneal amyotrophy

•

en:rhabdomyolysis, episodic and recurrent

•

en:replacement of affected muscle tissue with fatty tissue

•

en:reduced subcutaneous adipose tissue

•

en:muscle biopsy shows pleomorphic hyaline, granular, and amorphous deposits that stain with gomori trichrome

•

en:electron microscopy shows subsarcolemmal accumulations of abnormally shaped mitochondria

•

en:muscle biopsy shows myodegenerative changes with beta-amyloid and phosphorylated tau deposits

•

en:distal muscle weakness, predominantly lower limbs

•

en:electromyogram normal

•

en:edema of dorsum of hands and feet

•

•

en:dystrophic changes seen on muscle biopsy

•

en:dystrophic changes occur later

•

en:dystrophic changes, mild, seen on muscle biopsy

•

en:distal muscle weakness may occur

•

en:distal limb muscle weakness due to peripheral neuropathy

•

en:electron microscopy shows abnormal mitochondria with disorganized and vacuolated cristae

•

en:difficulty walking on heels occurs prior to difficulty walking on toes

•

en:difficulty running and jumping

•

en:decreased activities of mitochondrial complexes i, iii, and iv

•

en:congophilic amyloid material

•

en:buffalo hump (rare)

•

en:biopsy shows myopathy without vacuoles

•

en:atrophy of the muscles in the anterior compartment of the lower leg

•

en:asymmetric muscle involvement

•

en:electromyography shows myotonic discharges

•

en:excess fat, symmetrically located in buttocks and lower extremities to ankles

•

en:muscle biopsy shows decreased muscle-specific phosphorylase kinase activity

•

en:internal nuclei seen on biopsy (in some patients)

•

en:muscle biopsy shows cytochrome c oxidase-negative fibers

•

en:muscle biopsy shows chronic and active denervation

•

en:mild muscle weakness

•

en:mildly decreased laminin alpha-2 expression (lama2, 156225)

•

en:lymphedema, of upper and/or lower extremity (rare)

•

en:lymphangitis, episodic

•

en:large hypertrophied fibers

•

en:knee and hip flexor weakness due to peripheral neuropathy

•

en:interfibrillar and subsarcolemmal vacuoles

•

en:generalized hypotonia

•

en:inflammatory cells absent

•

en:increased mitochondria with abnormal cristae

•

en:increased focal mitochondrial density

•

en:increased central nucleation

•

en:hypotonia, mild (1 patient)

•

en:hypotonia, infantile, severe

•

en:hip flexor weakness

•

en:giant granules in muscle cells

•

en:angulated atrophic muscle fibers

•

en:calf pseudohypertrophy rare

•

en:atrophy of ankle and toe extensor (dorsiflexor) muscles

•

en:muscle biopsy shows neurogenic angulated fibers

•

en:muscle weakness and atrophy, progressive

•

en:muscle weakness (in 90% of patients)

•

en:muscle weakness, hypokalemia-related

•

en:muscle cramps affect the upper and lower limbs

•

en:muscle biopsy shows type 2 fiber atrophy (see details under neurologic heading)

•

en:muscle biopsy shows type 1 fiber predominance and hypertrophy

•

en:muscle biopsy shows subsarcolemmal nemaline bodies (rods) on gomori trichrome staining

•

en:muscle biopsy shows increased variation in fiber size

•

en:partial lipodystrophy (abnormal distribution of adipose tissue)

•

en:muscle biopsy shows hypoglycosylation of alpha-dystroglycan

•

en:muscle biopsy shows grouped atrophy

•

en:muscle biopsy shows decreased postsynaptic areas of clefts and folds

•

en:muscle biopsy shows core-like regions devoid of oxidative activity

•

en:muscle biopsy shows atrophic fibers

•

en:muscle biopsies show variation in fibrodiameter, internal nuclei, atrophy of type i fibers, focal loss of cross- striations, and cores of myofibrillar disruption with associated absence of mitochondria

•

en:muscle atrophy appears later in disease course

•

en:multiple mitochondrial respiratory enzymes deficiencies seen on biopsy

•

en:nuclear clumps

•

en:pelvic girdle muscle weakness (occurs earlier)

•

en:mri shows fatty infiltration of affected muscles

•

en:symptoms are progressive for a few years, and then become stationary

•

en:limb-girdle muscular dystrophy type 2a

•

en:atrophy of neck muscles may occur later

•

en:umbilical depression

•

en:type 2 muscle fiber predominance

•

en:type 1 fiber predominance

•

en:tubulofilamentous nuclear or cytoplasmic inclusions on biopsy

•

en:tubular aggregates seen on biopsy

•

en:skeletal myopathy

•

en:proximal muscles more often affected

•

en:shortening of various muscle-tendon groups in feet

•

en:secondary decrease of calpain-3 (capn3, 114240) protein expression in muscle

•

•

en:rhabdomyolysis, episodic

•

en:reduced o-mannosyl glycans on alpha-dystroglycan

•

en:ragged-red fibers seen on muscle biopsy

•

en:proximal upper limb amyotrophy

•

en:proximal muscle weakness, lower limbs, mild

•

en:mtdna deletions seen on muscle biopsy

•

en:upper arm and pelvic muscle weakness and atrophy (later onset)

•

en:minipolymyoclonus

•

en:decreased muscle mass

•

en:distal muscle weakness, affecting the lower limbs

•

en:distal limb muscular atrophy due to peripheral neuropathy

•

en:distal limb muscle atrophy, lower more than upper

•

en:dense membranous structure surrounding nuclei on electron microscopy

•

en:defective mitochondrial oxidative phosphorylation of multiple enzymes

•

en:decreased sarcolemmal immunostaining for ptrf

•

en:decreased muscle mitochondrial aconitase

•

en:decreased coenzyme q10 levels in muscle

•

en:distal muscle weakness, upper and lower limbs

•

en:decreased activity of cytochrome c oxidase (in most cases)

•

en:contractures of distal lower limbs

•

•

en:calf muscle pseudohypertrophy

•

en:calf muscle hypertrophy

•

en:biceps brachii atrophy

•

en:axial weakness

•

en:lymphangiectasia

•

en:distal muscle weakness, mild

•

en:attacks of weakness

•

en:duchenne muscular dystrophy (dmd, 310200) in 'complex' form

•

en:histochemically normal type-ii muscle fibers

•

en:lower and upper limbs affected

•

en:increased visceral fat

•

en:increased internal nuclei in skeletal muscle fibers

•

en:increased fibrosis

•

en:increased endo- and perimysial connective tissue

•

en:inability to jump or squat

•

en:emg may showed myotonic discharges

•

en:hyperactive deep tendon reflexes

•

•

en:global muscle weakness

•

en:generalized weakness (adult-onset)

•

en:fragmentation of the neuromuscular junction with pre- and postsynaptic defects

•

•

en:fiber-type variability

•

•

en:facial muscle atrophy of involved side

•

en:extensor muscle weakness more severe than flexor muscle weakness

•

en:emg shows fasciculations

•

en:neurogenic changes seen on emg

•

en:myofibrillar myopathy

•

en:myoclonic spasms

•

en:muscular weakness, improving in infancy (medical research council muscle score of 3 to 4)

•

en:muscle weakness is not a feature

•

en:muscle weakness, severe

•

en:muscle weakness, limb-girdle, mild

•

en:lymphedema of the extremities

•

en:muscle weakness, episodic

•

en:muscle spasm

•

en:muscle biopsy shows pas-positive glycogen-containing vacuoles

•

en:muscle biopsy shows fibrosis and increased connective tissue

•

en:muscle biopsy shows decreased secondary clefts

•

en:muscle biopsy may show deficiency of multiple mitochondrial respiratory complexes

•

en:muscle atrophy restricted to the upper limb

•

en:motor clumsiness

•

en:lymphography shows hypoplasia of lymphatic vessels

•

en:nonspecific muscle fiber atrophy

•

en:small, angulated muscle fibers seen on muscle biopsy

•

en:no muscle fiber necrosis

•

en:swelling of the hands and feet, episodic

•

en:loss of subcutaneous fat

•

en:accumulated lipid droplets seen on muscle biopsy

•

en:abnormal changes in the organization of the sarcolemma and the subsarcolemmal membrane cytoskeleton

•

en:muscle soft, tissue

•

en:z-disk streaming

•

en:type i fibers with large, irregularly polygonal congophilic hyaline masses and nemaline rods consistent with nonlysosomal protein degradation

•

en:thick palmar fascia

•

en:subsarcolemmal mitochondrial accumulation

•

•

en:sparing of small hand and finger muscles

•

en:skeletal muscle shows decreased sarcoglycan proteins

•

en:skeletal muscle biopsy shows impaired respiratory oxidation

•

en:scapular muscle atrophy

•

en:ragged-red fibers on muscle biopsy

•

en:proximal muscle weakness more severe than distal muscle weakness

•

en:postexercise cmap amplitude facilitation

•

en:pectoralis major hypoplasia

•

en:lower limbs more affected than upper limbs

•

en:delayed ambulation

•

en:loss of subcutaneous adipose tissue primarily in gluteal region and lower limbs

•

en:cardiomyopathy is not a feature

•

en:decreased activity of respiratory complex ii+iii

•

en:decreased activities of mitochondrial-encoded respiratory chain complexes

•

en:cytoplasmic inclusion bodies (finding)

•

en:centralized nuclei may be seen

•

en:centralized myonuclei

•

en:attacks precipitated by potassium

•

en:atrophy of the thenar and hypothenar muscles

•

en:atrophy of shins

•

en:decrement of the compound muscle action potential on repetitive stimulation

•

en:atrophy of anterior compartment tibial muscles

•

en:amyotrophy affects c5 to t1 myotomes

•

en:affected muscles show atrophy

•

en:acquired partial lipodystrophy

•

en:abnormal subcutaneous fat tissue distribution

•

en:abnormal mitochondrial proliferation

•

en:abnormal irregular myofibrillar network

•

en:decreased shank muscle mass

•

en:defective glycosylation of alpha-dystroglycan seen on biopsy

•

en:loss of independent ambulation around age 12 years

•

en:glycogenosis of skeletal muscle, mild (in some patients)

•

en:loss of ambulation between third and sixth decade

•

en:loose connective tissue

•

en:lipoatrophy, generalized

•

en:levator palpebrae superioris atrophy

•

en:hypotonia (2 patients)

•

en:hypotonia, fixed

•

en:hypoplasia of latissimus dorsi muscle

•

en:gluteal hypoplasia

•

en:denervation of skeletal muscles

•

en:generalized muscle weakness, proximal and distal

•

en:generalized muscle contractions, episodic

•

en:focal areas of z-band disarray devoid of mitochondria seen on electron microscopy

•

en:finger and wrist extensor weakness

•

en:emg shows high-frequency spontaneous discharges

•

en:emg may show myopathic features

•

en:distal muscle weakness

•

en:distal limb atrophy (upper and lower)

•

en:biopsy shows degenerative changes consistent with myopathy

•

en:swelling of the hands and feet (in 1 patient)

•

en:changes in sarcoplasmic reticulum and t-tubules

•

en:muscle hypertrophy of the lower extremities

•

en:nemaline bodies occur in type 1 fibers

•

en:necrotic and atrophic fibers with centralized nuclei

•

en:myopathic features seen on biopsy

•

en:myofibrillar disintegration

•

en:muscular dystrophy

•

en:muscle weakness may affect upper limbs

•

en:muscle weakness, limb-girdle (lower and upper limbs may be affected)

•

en:muscle fiber atrophy, profound (rare)

•

en:positive staining for atpase activity at ph of 4.3

•

en:muscle biopsy shows necrotic and atrophic fibers with centralized nuclei

•

en:muscle biopsy shows glycogen accumulation in myofibrils and lysosomes

•

en:muscle biopsy shows fiber type variation (in some patients)

•

en:muscle atrophy later in the disease

•

en:muscle atrophy (in some patients)

•

en:mitochondrial proliferation

•

en:mild proximal muscle weakness (<10% of patients)

•

en:painful swelling in fasciae

•

en:progressive muscle weakness

•

en:loss of subcutaneous adipose tissue in limbs

•

en:tendon tears

•

en:cold sensitivity has been reported

•

en:z-line streaming see on electron microscopy

•

en:z-band streaming

•

en:upper limb weakness

•

en:type 2 fiber atrophy see on biopsy

•

en:tubular aggregates in muscle fibers may occur

•

en:thenar eminence hypoplasia

•

en:subcutaneous edema

•

en:proximal limb muscle weakness (occurs later)

•

en:soft tissue vascular malformations

•

en:skeletal muscle tissue shows depletion of mitochondrial dna (mtdna)

•

en:skeletal muscle biopsy shows hypertrophy of type 1 and 2 muscle fibers

•

en:severely decreased or absent lamp2 protein

•

en:sarcomere disappearance seen on muscle biopsy

•

en:quadriceps aplasia

•

en:proximal weakness of the lower limbs with longer disease duration

•

en:proximal muscle weakness occurs later

•

en:loss of subcutaneous and visceral adipose tissue, mild

•

en:winged scapula

•

en:localized cellulitis

•

en:defects in mitochondria respiratory activities, mainly complexes i, ii, and iv

•

en:distal muscle weakness occurs later

•

en:distal muscle weakness due to neuropathy

•

en:distal muscle weakness and atrophy

•

en:distal lower limb amyotrophy

•

en:distal limb muscle weakness due to peripheral neuropathy (lower limbs are more affected than upper limbs)

•

en:dependent edema after excess fat has been present for several years

•

en:degenerating nerve terminals

•

en:decreased or patchy amounts of alpha-dystrobrevin and beta-2-syntrophin at the sarcolemma

•

en:electromyographic decrements in some muscles

•

en:decreased expression of the vdac1 protein

•

en:decreased ant1 protein (103220) in muscle tissue

•

en:decreased activity of respiratory complex i+iii

•

en:decreased activity of cytochrome c oxidase seen on muscle biopsy

•

en:cores with lack of oxidative activity and mitochondrial depletion may also be found and extend along length of fiber

•

en:congenital fiber-type disproportion

•

en:lipid accumulation in muscle tissue

•

en:congenital, nonprogressive myopathy

•

en:edema of lower extremity

•

en:cores have abrupt borders within normal regions of the muscle fiber

•

en:emg shows increased insertion activity and fibrillation

•

en:hyporeflexia of lower limbs

•

en:limb lymphedema (in some patients)

•

en:infantile muscular hypotonia

•

en:intrasarcoplasmic dense granulofilamentous aggregates that are immunoreactive with desmin

•

en:increased variation in fiber size seen on muscle biopsy

•

en:increased subcutaneous truncal adipose tissue

•

en:increased lipid droplets and abnormal mitochondria

•

en:hypotonia, truncal and appendicular

•

en:hypotonia (45%)

•

en:hypertonia at birth

•

en:gluteus maximus and thigh adductors most affected

•

en:filamentous inclusions in muscle fibers

•

en:fat pad anterior to malleolus

•

en:fatty replacement

•

en:fatty infiltration of affected muscles

•

en:endomysial fibrosis

•

en:emg shows neurogenic changes consistent with denervation

•

en:emg shows myopathic changes

•

en:loss of subcutaneous adipose tissue from upper limbs and trunk

•

en:mri shows increased signal intensity in affected muscles consistent with fatty infiltration

•

en:mitochondrial dna depletion (20 to 35% of normal) seen on skeletal muscle biopsy

•

en:mild distal muscle atrophy

•

en:marked variability of fiber size

•

en:lower limb atrophy

•

en:hypotonia at birth

•

en:loss of adipose tissue occurs around puberty

•

en:limb-girdle muscle weakness (2 families)

•

en:increased muscle tone (early in life)

•

en:increased lipid

•

en:increased fatty infiltration

•

en:inclusions are tdp43-immunoreactive

•

en:impaired heel-walking

•

en:hypotonia (patient a)

•

en:muscle atrophy, proximal and distal (predominant lower limb involvement)

•

en:myopathic changes seen on emg and muscle biopsy

•

en:muscle biopsy shows abnormal mitochondria

•

en:nemaline bodies

•

en:proximal limb muscle weakness initially

•

en:hypoplastic deltoids

•

en:poor sarcomeric structure (in some patients)

•

en:pelvic girdle muscle atrophy

•

en:paucity or absence of lymph nodes in the axillae and above the inguinal ligaments seen on scintilymphangiography

•

en:painful swelling in aponeuroses

•

en:neurogenic changes

•

en:nearly complete absence of mechanical adipose tissue (joints, orbits, palms, soles)

•

en:muscle biopsy shows fiber type disproportion

•

en:myopathic episodes triggered by exercise, fasting, infection, cold temperatures

•

en:myofibrillar disorganization

•

en:muscle weakness

•

en:muscle strength and bulk is preserved

•

en:muscle cramps (also seen in some heterozygotes)

•

en:muscle biopsy shows type 1 fiber predominance

•

en:muscle biopsy shows neurogenic changes

•

•

en:distal amyotrophy

•

en:hypoplasia of the hand muscles (29%)

•

en:centrally nucleated skeletal muscle fibers

•

en:decreased cytochrome c oxidase (cox) activity and protein

•

en:decreased coenzyme q10 content

•

en:decreased activity of complex i and complex iv

•

en:decreased ability to run

•

en:cranial muscles spared

•

en:contractures of upper limbs (in some patients)

•

en:calf muscle weakness

•

en:decreased exercise tolerance

•

en:biopsy shows dystrophic changes

•

en:ankle edema (finding)

•

en:alimentary edema

•

en:absence of telethonin protein

•

en:absence of gamma-sarcoglycan protein

•

en:abnormal fat accumulation in axillae (in some patients)

•

en:secondary loss of sarcolemmal caveolin-3

•

en:decreased distal reflexes

•

en:disruption of the sarcolemmal membrane seen on muscle biopsy

•

en:hypoplasia of serratus anterior muscle

•

en:fasciculation

•

en:hypertonicity of the lower limbs

•

en:hypertonia late

•

en:hangrip myotonia

•

en:glycogen deficiency in muscle fibers

•

en:foot dorsiflexor weakness

•

en:fibrillations in affected muscles

•

en:fasciculations (less common)

•

en:facial muscle weakness

•

en:distal muscle atrophy due to neuropathy

•

en:exertional fatigue

•

en:evidence of reinnervation seen on emg (1 family)

•

en:episodic, nonerythematous, nonpruritic, nontender edema, episodic

•

en:enteroviral dermatomyositis syndrome

•

en:emg shows myopathic pattern

•

en:emg shows myopathic features

•

en:distal skeletal muscle atrophy (after long disease duration)

•

en:proximal muscle weakness (85% of patients)

•

en:emg shows chronic denervation

•

en:secondary myopathic features

•

en:myalgia (in some patients)

•

en:nonspecific myopathic changes

•

en:neck flexor weakness

•

en:myotonia (delayed muscle relaxation after contraction)

•

en:myopathy with abnormal lipid metabolism

•

en:myopathic changes seen muscle biopsy

•

en:myopathic changes early in disease seen on emg

•

en:muscle weakness and atrophy, upper and lower limbs

•

en:percussion myotonias

•

en:muscle weakness, primarily proximal

•

en:muscle fiber splitting (in some patients)

•

en:muscle cramps during exercise

•

en:muscle biopsy shows myopathic changes

•

en:muscle biopsy shows enlargement of type i muscle fibers, consistent with denervation

•

en:muscle biopsy shows dystrophic features

•

en:muscle biopsy shows abnormal fiber size and variation

•

en:patients may have distal or proximal muscle weakness, or both

•

en:poor muscle bulk

•

en:minicores extend full fiber diameter

•

en:superior rectus atrophy

•

en:shortening of the fascial structures of the palm and fingers

•

•

en:wasted lower leg muscles

•

en:variation of myofiber diameter

•

en:type 2 fiber predominance

•

en:type 1 muscle fibers with clearly demarcated central 'core' regions of sarcomeric disorganization, lack of oxidative activity, and absent mitochondria

•

en:toe and foot dorsiflexor weakness

•

en:subsarcolemmal mitochondrial aggregates

•

en:proximal muscle weakness (120270.0002)

•

en:sparing of anterior tibialis muscle

•

en:skeletal muscle shows dystrophic changes

•

en:shoulder girdle muscle weakness and atrophy, progressive

•

en:scapular muscle weakness

•

en:respiratory muscles may be involved (more common in juvenile-onset)

•

en:relative muscle weakness, especially in pelvic girdle

•

en:quadriceps weakness

•

en:muscles become hard and tender during exercise

•

en:variable deficiencies of mitochondrial respiratory chain complexes i, iii, and iv

•

en:mild weakness

•

en:wide variation in fiber size

•

en:attacks usually decrease or disappear after age 40 years

•

en:attacks last 4 to 24 hours

•

en:ascending muscle weakness (beginning with feet and legs)

•

en:absence of necrosis

•

en:absence of collagen vi immunostaining

•

en:abnormal jitter seen on emg

•

en:abnormal iron deposition in mitochondria

•

en:variably decreased activities of mitochondrial respiratory complexes i, ii, and iv

•

en:cytochrome c oxidase-negative fibers

•

en:upper limb muscle weakness may be more severe than lower limb weakness

•

en:triceps weakness

•

en:toe-walking gait

•

en:subcutaneous fat markedly reduced

•

en:small and incompletely fused muscle fibers seen on biopsy

•

en:skeletal muscle biopsy shows misplaced mitochondria

•

•

en:cellulitis or infection may occur

•

en:thenar muscle atrophy

•

en:decreased glycosylation of alpha-dystroglycan

•

en:episodic, nonpruritic, nonurticarial, nonpitting edema

•

en:increased adipose fibrosis

•

en:decreased mitochondria

•

en:hyporeflexia

•

en:intermyofibrillar and subsarcolemmal accumulation of electron-dense material (in some patients)

•

en:hip abductor weakness and atrophy

•

en:glycogen depletion in skeletal muscle

•

en:fetal akinesia deformation sequence

•

en:hypertrophy of calves (in 3 of 6 patients)

•

en:edema, generalized

•

en:deep finger muscle weakness

•

en:distal limb muscle atrophy due to peripheral neuropathy

•

en:disruption plasma membrane (in some patients)

•

en:difficulty walking, running

•

en:difficulty rising from a squatting position

•

en:easily fatigued (finding)

•

en:decreased shoulder muscle mass

•

en:rupture of muscle

•

en:relative sparing of the rectus femoris

•

en:proximal muscle weakness in lower limbs

•

en:proximal muscle weakness, mild (in some patients)

•

en:protein aggregates immunopositive for actin and desmin

•

en:no myofibrillar abnormalities or protein aggregation

•

en:pharyngeal muscle weakness

•

en:percussion-induced rapid rolling muscle contractions (pirc)

•

en:paralysis of the lower limbs (later)

•

en:non-pitting edema

•

en:neonatal hypotonia muscle biopsy shows pas-positive vacuoles

•

en:necrotic and regenerating muscle fibers

•

en:myalgia, episodic

•

en:muscle pain and cramps following exercise

•

en:sparing of the brachioradialis muscle (gives appearance of 'oblique amyotrophy')

•

en:abnormal z bands

•

en:structurally abnormal mitochondria

•

en:biopsy shows nonspecific myopathy without rimmed vacuoles

•

en:decreased coenzyme q10

•

en:muscle biopsy with small a-fibers, increased pas-positive material, and ringbinden (disoriented myofibrils)

•

en:decreased achr at the endplate

•

en:cytochemical and em muscle studies show large, bizarre mitochondria in striated muscle

•

en:centralized nuclei, rare

•

en:calcific tendinopathy, early-onset (in some patients)

•

en:atrophic fibers

•

en:tubular aggregates (in some patients)

•

en:acetazolamide may worsen symptoms

•

en:velopharyngeal muscle weakness

•

en:variable deficiencies of mitochondrial respiratory chain enzymes

•

en:umbilical hernia

•

en:type 2 fiber hypertrophy

•

en:tubular aggregates seen on muscle biopsy

•

en:muscle hypertrophy may occur

•

en:emg shows denervation

•

en:muscle biopsy shows ragged red fibers

•

en:decreased mitochondrial complex iii activity seen on muscle biopsy

•

en:distal muscle weakness and atrophy, upper and lower limbs

•

en:distal lower limb anterior compartment weakness

•

en:distal limb muscle weakness (less common)

•

en:difficulty raising arms

•

en:degenerating fibers

•

en:decreased muscle mass in the thighs

•

en:decreased activity of mitochondrial complexes i and iv seen on skeletal muscle biopsy

•

•

en:congenital muscular dystrophy (disorder)

•

en:broad-based gait

•

en:atrophic calf muscles

•

en:abnormal mitochondria with loss of cristae

•

en:abnormal glycosylation of alpha-dystroglycan seen on muscle biopsy

•

en:decreased glycosylation of alpha-dystroglycan seen on muscle biopsy

•

en:distal muscular atrophy due to peripheral neuropathy

•

en:emg shows myopathic and neurogenic changes

•

en:muscle biopsy shows atrophy

•

en:increased staining for myotilin, dystrophin, desmin

•

en:mri may show cervical spinal cord atrophy

•

en:mitochondria dna deletion

•

en:mitochondrial paracrystalline inclusion bodies

•

en:mild weakness of the small hand muscles

•

en:lower limb muscle weakness (in 2 of 3 families)

•

en:lipid droplet accumulation

•

en:inability to run

•

en:fatty degeneration of muscle seen on mri

•

en:hypotonia (cvs+)

•

en:hypoplasia of cervical paravertebral muscles

•

en:hyaline plaques with absent oxidative activity

•

en:grip strength decreased

•

en:greater sartorius involvement than gracilis involvement seen on mri

•

en:fragmented mitochondrial network

•

en:decreased endplate potential and amplitudes

•

en:hypotonia, generalized (in some patients)

•

en:decreased immunostaining for alpha-sarcoglycan

•

en:phagocytic vacuoles with degraded membranous material

•

en:muscle weakness, severe, proximal and distal

•

en:muscle weakness, upper and lower limbs

•

en:muscle weakness of proximal and distal upper and lower limbs

•

en:muscle weakness restricted to the upper limb

•

en:muscular rigidity

•

en:myofibromatosis

•

en:myopathic pattern on emg

•

en:myopathy, nonprogressive

•

en:nearly complete absence of metabolically active adipose tissue (subcutaneous, intraabdominal, intrathoracic)

•

en:nemaline bodies are rarely intranuclear

•

en:neurogenic changes seen on emg and biopsy

•

en:nonspecific myopathic changes without dystrophic or inflammatory changes seen on muscle biopsy

•

en:other mitochondrial complex enzyme activities may also be decreased

•

en:percussion-induced muscle mounding (muscle rippling)

•

en:poor musculature

•

en:muscle weakness, proximal, slowly progressive

•

en:subsarcolemmal accumulations of abnormally shaped mitochondria seen on electron microscopy

•

•

oedème de la face

•

en:weakness of the muscles in the anterior compartment of the lower leg (particularly the tibialis anterior muscle)

•

en:vacuoles containing neutral lipids seen on muscle biopsy

•

en:upper limb involvement may occur later

•

en:transient neonatal hypotonia (less common)

•

en:difficulty running

•

en:progression to wheelchair in fourth decade

•

en:severe muscle wasting and atrophy, primarily in the legs

•

en:secondary myopathic changes

•

en:sarcoplasmic inclusions

•

en:rimmed vacuoles on biopsy

•

en:pseudohypertrophy of muscle

•

en:prominent muscular appearance

•

en:muscle weakness, proximal and distal (predominant lower limb involvement)

•

en:shoulder muscle hypoplasia

•

en:muscle weakness, congenital

•

en:generalized muscle weakness (less common)

•

en:lack of adipose tissue on limbs and femorogluteal regions

•

en:increased glycogen

•

en:hypotonia (in some patients)

•

en:hypotonia, neonatal and later

•

en:hypotonia, mild

•

en:hypertonicity (uncommon)

•

en:floppy infant

•

en:limb-girdle muscle weakness, proximal, upper greater than lower

•

en:exertional leg pain

•

en:enlarged vesicles of sarcoplasmic reticulum origin

•

en:easy fatigue

•

en:distal weakness, upper and lower limbs (variable)

•

en:distal muscle weakness and atrophy may occur, upper and lower limbs affected

•

en:muscle wasting in lower limbs

•

en:limb-girdle muscle atrophy

•

en:electrophysiologic studies show abnormal inactivation and delayed repolarization of the scn4a sodium channel

•

en:lipodystrophy

•

en:muscle biopsy shows decrease or absence of cytochrome c oxidase

•

en:muscle stiffness or aching

•

•

en:muscle pain and cramping on exertion

•

en:muscle fibers contain internal nuclei

•

en:muscle biopsy shows sarcoplasmic pas-positive vacuoles

•

en:muscle biopsy shows degenerating endplates and junctional folds

•

en:muscle hematoma

•

en:muscle biopsy shows decreased glycosylation of alpha-dystroglycan (dag1, 128239)

•

en:low maximum workload on exercise testing

•

en:muscle aches, cramps

•

en:loss of subcutaneous adipose tissue from gluteal region

•

en:mitochondrial complex iii deficiency (disorder)

•

en:lymphedema of upper and/or lower limbs (in some patients)

•

en:muscle atrophy

•

en:muscle atrophy, distal, particularly affecting the hands muscle biopsy shows variation in fiber size

•

en:muscle biopsy shows 'spheroid bodies' in type 1 muscle fibers

•

en:muscle biopsy shows decreased numbers of acetylcholine receptors (achr) in the postsynaptic membrane (less than 50% of normal)

•

en:muscle biopsy shows dystrophic pattern

•

en:muscle biopsy shows nemaline bodies (rods) on gomori trichrome staining

•

en:muscle biopsy shows increased number of endplate regions distributed over increased span of muscle fiber

•

en:muscle biopsy shows mitochondrial aggregates

•

en:muscle biopsy shows small nerve terminals

•

en:muscle fibers appear as fetal myotubules

•

en:multiple mitochondrial dna (mtdna) deletions seen on muscle biopsy

•

en:muscle fiber regeneration

•

en:muscle activity is electrically silent on emg

•

•

en:mildly decreased cytochrome c oxidase activity

•

en:may have proximal muscle weakness later in the disease

•

en:marked predominance of slow-twitch, oxidative (type 1) muscle fibers

•

en:lymphography shows increased number of dilated lymphatic vessels (hyperplasia)

•

en:lymphedema of the hands (in some patients)

•

•

en:increased muscle tone at birth

•

en:increased endomysial fibrosis

•

en:increased connective tissue

•

en:hypoplasia of muscle

•

en:hyaline inclusion

•

en:muscle weakness following prolonged exercise

•

en:muscle hypertrophy

•

en:retroperitoneal fibrosis (rare)

•

en:muscle weakness in forearm muscles

•

en:rare necrotic fibers

•

faiblesse de muscle

•

en:weakness of ankle plantar flexion

•

en:weakness and wasting of the lower limbs

•

en:variable fiber size

•

en:thickening of the fascial structures of the palm and fingers

•

en:subsarcolemmal accumulation of enlarged mitochondria with variably sized rectangular or elongated rhomboidal paracrystalline inclusions

•

en:sparing of the anterior tibial compartment

•

en:some patients never achieve ambulation

•

en:shortening of flexor profundus muscle-tendon unit

•

en:rhabdomyolysis may occur

•

en:reduced lower limb subcutaneous fat

•

en:quadriceps atrophy (later onset)

•

en:muscular dystrophy with variable age of onset (infancy to young adult)

•

en:proximal muscle weakness, less severe

•

en:preservation of visceral, neck, and axillary fat pads

•

en:potassium sensitivity has been reported

•

en:paradoxical myotonia (myotonia increases with exercise)

•

en:normal or decreased facial and neck adipose tissue

•

en:neurogenic atrophy seen on muscle biopsy

•

en:neonatal hypotonia

•

en:neck muscle weakness

•

en:myotonia improves with continued activity ('warm-up phenomenon')

•

•

en:myofibrillar lesions

•

en:groups of atrophic fibers seen on muscle biopsy (type i fiber predominance)

◀

• Mathieu Lafourcade • LIRMM (données libres en Creative Commons) •

• This page link : https://www.jeuxdemots.org/diko.php?gotermid=7120530 • caches: c1=6 c2=1002 c3=1

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