The THAP1 mutations database - List of Phenotypic information


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SymptomSeverity
 
Age at last examination
Age of onset
arm
arm+cervical
arm+cranial
arm+head
cranial
face
foot
hand
jaw
larynx
larynx+cervical
leg
neck
speech
unknown
Arm
bilateral
hand tremor
mild
moderate
Asymptomatic
Cranial
Deep Brain Stimulation
Excellent long-term effects
Good improvement of axial and limb dystonia. No improvement of disarthria.
Improvement of axial and limb dystonia. No improvement of speech.
mild improvement
Mild improvement of axial and limb dystonia.
Mild improvement of cervical and limb dystonia. No improvement of dysarthria.
moderate improvement
Dysarthria
mild
moderate
severe
Essential hand tremor
Face
blepharospasm
mild
Foot
Incomplete clinical description
Jaw
severe
Larynx
moderate
severe
Left arm
Left leg
Leg
bilateral
mild
moderate
severe
Neck
mild
moderate
Severe
No clinical data available
Paroxysmal head shake
Right arm
Right leg
Site of onset-Foot
Site of onset-Hand (writer's dystonia)
Site of onset-Orofacial/Oromandibular
Speech
mild
Spontaneous complete remission (honeymoon)from 18 to 22
Tongue
mild
severe
Trunk
mild
moderate