Phenotypes for disease #01685 (BVVLS1 (Brown-Vialetto-Van Laere syndrome, type 1 (BVVLS1)), OMIM:211530)

13 entries on 1 page. Showing entries 1 - 13.
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Phenotype details     

Diagnosis/Initial     

Diagnosis/Definite     

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Age/Examination     

Age/Diagnosis     

Age/Onset     

Phenotype/Onset     

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Owner     

Individual ID     
0000152877 Progressive weakness, muscle wasting and truncal ataxia. Initial presentation: Multiple peripheral neuropathy - - - - - - - - LOVD 00204615
0000152878 Anterior horn neuropathy; Intact cognitive development. Initial presentation: Progressive bulbar palsy - - - - - - - - LOVD 00204616
0000152879 Progressive muscle weakness. Initial presntation: VII nerve palsy, deafness - - - - - - - - LOVD 00204617
0000152880 Poor balance, proximal muscle group weakness. Initial presentation: Deafness, Tongue wasting and fasciculations - - - - - - - - LOVD 00204618
0000152881 Multiple cranial nerve involvement. Initial presentation: Breathing problems - - - - - - - - LOVD 00204619
0000152882 Progressive muscle weakness and wasting, external ophthalmoplegia. Initial presentation: Tongue fasciculations, facial palsy - - - - - - - - LOVD 00204620
0000152883 Hypotonia, cerebellar signs, brisk reflexes in lower limbs, persistent ankle clonus, stridor, EMG: bulbar palsy, anterior horn involvement; phrenic nerve denervation, auditory neuropathy. Initial presentation: Sub-acute encephalopathy - - - - - - - - LOVD 00204621
0000152884 Hypotonia, bulbar palsy, respiratory difficulties. Initial Presentation: Hypotonia, bulbar palsy - - - - - - - - LOVD 00204622
0000152885 General weakness, flaccid. Initial presentation: Breathing difficulties, weakness - - - - - - - - LOVD 00204625
0000155279 visual impairment (HP:0000505); progressive hearing impairment (HP:0000365); no abnormality of the liver (-HP:0001392); no hepatic Wilson disease symptoms, liver biopsy normal; visits regular school - BVVLS Familial, autosomal recessive 10y 05y - - - Gunnar Schmidt 00207500
0000155299 Visual impairment (HP:0000505) Hearing impairment (HP:0000365) - BVVLS Familial, autosomal recessive - - - - - Gunnar Schmidt 00207521
0000293117 Abnormal nervous system physiology, Visual impairment, Abnormal cranial nerve morphology, Cranial nerve paralysis, Abnormality of eye movement, Dysphagia, Hearing impairment - - Familial, autosomal recessive 50y - - - - Andreas Laner 00400076
0000301659 best corrected visual acuity: 1; electroretinogram , rod: non- recordable, cone: non- recordable - dystrophy, corneoretinal, crystalline, Bietti (BCD) Familial, autosomal recessive 63y - 45y - - LOVD 00409542
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