• 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

• 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

• 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.

• 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.

FDP

EIP

SCM

L&R

8-9 Hz tremor
Synchronous bursting pattern

• 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.

• CD head tremor can be multidirectional at the start compared to ET which is usually unidirectional (most commonly “no no”).

• 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”).

• 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.

• 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.

• Dystonic tremor is often suppressed by sensory tricks (geste antagoniste)

• Tremor may disappear when the affected body part assumes a dystonic position (the “null point”) and be exacerbated when turning away from dystonic position.

Whats driving tremor extension or flexion?

• 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

• Titubation occurs as a component of midline cerebellar ataxia and is accompanied by cerebellar findings

• 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?

• Null -? Looking left

• Yes–Yes in all directions except extension where it converts to No-No