Phenotypes for disease #00433 (NBIA1 (neurodegeneration, with brain iron accumulation, type 1 (NBIA)), OMIM:234200)

4 entries on 1 page. Showing entries 1 - 4.
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0000053525 birth weight: 3,850 g; No history of perinatal complications;Normal early developmental milestones; 24 months: parents reported gait difficulties and persistent toe walking; 6y: poor academic ability;15y: Normal general physical examination; Mild oro-mandibular dystonia with dysarthria; spastic dystonic paraparesis; still able to walk unaided; IQ ¼ 49; 20y:unable to ambulate independently; 25y: severe spastic bradykinetic-rigid syndrome associated with mild dystonia with distal areflexia in the lower limbs - - Familial, autosomal recessive 25y - - - - Marianne Vos (LOVD-team) 00017613
0000053526 born at term of uneventful pregnancy;1y Normal psychomotor development; walking delayed as a result of instability and toe walking; 3y: spastic tetraparesis; moderate mental and language impairment; Disease was progressive, worsening of motor signs in lower limbs; progressive involvement of upper limbs and oro-mandibular region;15y:No independent ambulation; 17y: mild oro-mandibular dystonia with dysarthria; spastic-dystonic tetraparesis with prevalent involvement of lower limbs; parkinsonian features (rigidity and abnormal postural reflexes); Distal amyotrophia; areflexia with pes cavus; Severe cognitive impairment (total IQ < 40); Obsessive-compulsive behavior; complex motor tics; 19y-unchanged neurological picture; Motor axonal neuropathy more prominent in the lower limbs - - Familial, autosomal recessive 20y - - - - Marianne Vos (LOVD-team) 00017614
0000078710 - - - Unknown - - - - - Bader Alhaddad 00100446
0000301252 Global developmental delay, Gait disturbance, Abnormal cerebral morphology, Gait imbalance, Postural instability, Frequent falls, Falls, Functional motor deficit, Neurodevelopmental delay, Abnormality of movement - - Familial, autosomal recessive 03y - - - - Andreas Laner 00409136
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