Professor Con Yiannikas
Cervical Dystonia
Head Tremor
Isolated tremor syndrome of bilateral action/postural tremor
At least 3 years duration
May have associated Head or Voice tremor
Absence of other neurological signs
Cervical Dystonia
Isolated head tremor is excluded from ET - need at least a 5 year history of hand tremor
The phenotype of hereditary ET was studied in 20 index patients and their kindred: tremor of the head never occurred in isolation
The relationship between isolated head tremor and focal tremulous cervical dystonia is a topic of ongoing controversy
Head tremor was present in 30-40% of ET cases. Female gender was associated with a fourfold increased risk of head tremor
Head Tremor
Cervical Dystonia
75 year old woman history of tremor since her early 20’s. The tremor is there predominantly when she is using her hands and she has not known a resting tremor. It initially did not affect her head or her voice. There is no family history of tremor.
Over the last 10 years her speech and head have become affected.
Head Tremor
Cervical Dystonia
54 yr old male has had tremor of his arms since he was a young boy probably aged between three and four years.
He has not noticed a tremor in his voice.
Over the last twenty years he has had a tremor affecting his head which is troubling him greatly.
Head Tremor
Cervical Dystonia
FDP
EIP
SCM
L&R
8-9 Hz tremor
Synchronous bursting pattern
Head Tremor
Cervical Dystonia
Head Tremor
Cervical Dystonia
Head Tremor
Cervical Dystonia
Head tremor can be the presenting feature of cervical dystonia and may remain isolated for long periods
The frequency and amplitude of dystonic tremor is often variable, typically irregular
May disappear when the affected body part assumes a dystonic position (the “null point”) and be exacerbated when turning away from dystonic position.
Head Tremor
Cervical Dystonia
CD head tremor can be multidirectional at the start compared to ET which is usually unidirectional (most commonly “no no”).
Head Tremor
Cervical Dystonia
Clinical Assessment
Dystonic posturing of neck, other dystonias affecting face or limbs point towards a dystonic head tremor.
CD head tremor can be multidirectional at the start compared to ET which is usually unidirectional (most commonly “no no”).
Cervical Dystonia
Clinical Assessment
Dystonic tremor often has task or position specificity, persistence at rest, and overflow to neighbouring body segments.
ET head tremor more likely to resolve in the supine position compared to dystonic tremor.
Cervical Dystonia
Clinical Assessment
Rare focal tremors that may occur in the absence of other neurological signs, such as hereditary geniospasm, isolated jaw tremor, isolated tongue tremor, rabbit syndrome, and tremor during smiling.
Cervical Dystonia
Clinical Assessment
Dystonic tremor is often suppressed by sensory tricks (geste antagoniste)
Cervical Dystonia
Clinical Assessment
Tremor may disappear when the affected body part assumes a dystonic position (the “null point”) and be exacerbated when turning away from dystonic position.
Cervical Dystonia
Clinical Assessment
Whats driving tremor extension or flexion?
Cervical Dystonia
Clinical Assessment
Jaw tremor is a type of cranial tremor that is classically associated with PD but can affect lips and tongue.
May reset like arm tremor
Head tremor is rare
Cervical Dystonia
Clinical Assessment
Cervical Dystonia
Clinical Assessment
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Titubation occurs as a component of midline cerebellar ataxia and is accompanied by cerebellar findings
Cervical Dystonia
Clinical Assessment
Tremor occurring to the side of dystonia as it tries to bring back to midline (anti-null).
Splenius capitis (SPL), semispinalis capitis and obliquus capitis inferior muscles (OCI)
Burst-like tremor activity was present in both OCI in 25 and in one in 10 patients. In 18 patients, tremor activity was present in one SPL and in 2 in both SPL.
Null point in action that releases dystonic muscles?
Cervical Dystonia
Clinical Assessment
Null -? Looking left
Yes–Yes in all directions except extension where it converts to No-No
Cervical Dystonia
Clinical Assessment