TWMUTWMU

Deep Brain Stimulation for Speech Problems

Takaomi Taira, M.D., Ph.D.

Department of Neurosurgery Tokyo Women’s Medical University Tokyo, Japan

T.Taira,T.Taira, 2009 2009 TWMUTWMU The old Japanese proverbs

• Speech is silver, silence is golden. • Out of the mouth comes evil. • Confine your tongue, lest it confine you. • Action rather than talk.

T.Taira,T.Taira, 2009 2009 TWMUTWMU Speech disturbance and

Necessary anatomical structures for speech Type of dystonia

Diaphragm Laryngeal dystonia (spasmodic dysphonia) Pharyngeal dystonia Pharynx Tongue Orofacial dystonia jaw opening/closing dystonia Mouth Cervical dystonia Face !(exp. antecollic CD)

T.Taira,T.Taira, 2009 2009 TWMUTWMU Spasmodic dysphonia

Traube (1871) die spastische Form der nervosen Heiserkeit (spastic form of neurogenic hoarseness)

Frankel mogiphoni Schnitzler (1885) aphonia spastica Gowers (1899) functional laryngeal Crithcley (1935) inspiratory speech

Aronson (1985) spasmodic dysphonia not psychogenic but neurological abductor type, adductor type

Blitzer & Brin (1984) spasmodic dysphonia as focal dystonia T.Taira,T.Taira, 2009 2009 TWMUTWMU Spasmodic dysphonia isolated laryngeal 70% segmental/generalized 30%

adductor type 82% abductor type 17% adductor breathing type 1% (Blitzer & Brin, 1998)

primary laryngeal type --- > spread to other areas (15%)

T.Taira,T.Taira, 2009 2009 TWMUTWMU Oromandibular dystonia

associated symptoms

cervical dystonia 57% limb dystonia 21% 16% spasmodic dysphonia 10%

(Tan & Jankovic, 1999) T.Taira,T.Taira, 2009 2009 TWMUTWMU

speech --> facial dystonia --> speech disturbance facial dystonia triggered by speaking

T.Taira,T.Taira, 2009 2009 TWMUTWMU

Laterocollic cervical dystonia No speech disturbance

T.Taira,T.Taira, 2009 2009 TWMUTWMU Tongue protrusion dystonia

15-year-old boy, 27-year-old man, neuroacanthocytosis Lesch-Nyhan syndrome

T.Taira,T.Taira, 2009 2009 TWMU TWMU Segmental dystonia (head / neck)

Blephalospasm Meige syndrome Oromandibular dystonia

Spasmodic dysphonia Cranio-cervical dystonia Spasmodic torticollis

SP/SW difficulty laryngo-phryngeal dystonia

Yawning Man or De Gaper. blepharospasm-oromandibular dystonia and suggested the eponym "Brueghel's syndrome" (Marsden CD, JNNP 1976;39:1204-9). T.Taira,T.Taira, 2009 2009 TWMUTWMU Meige syndrome and DBS

Houser M, Waltz T Meige syndrome and pallidal deep brain stimulation. Mov Disord. 2005; 20:1203-5

Foote KD, et al. Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology. Neurosurgery. 2005;56:E415

Krauss JK Deep brain stimulation for dystonia in adults. Overview and developments. Stereotact Funct Neurosurg. 2002;78:168-82

T.Taira,T.Taira, 2009 2009 TWMUTWMU

!"#$%&'%()&*"+'&%(,-./0#1'%!"#$%&'%()&*"+'&%(,-./0#1'%

GPi DBS T.Taira,T.Taira, 2009 2009 TWMUTWMU Bilateral GPi DBS

dysarthric speech most common current spread to corticobulbar tr

“Can we improve dystonic speech problems with GPi DBS ?”

T.Taira,T.Taira, 2009 2009 TWMUTWMU Intention to speak ----> orofacial dystonia

T.Taira,T.Taira, 2009 2009 TWMUTWMU "#$%&'!Spasmodic Dysphonia

Before !!GPi DBS! !After

T.Taira,T.Taira, 2009 2009 TWMUTWMU Severe oral dyskinesia Speech/eating problems

before GPi DBS after

T.Taira,T.Taira, 2009 2009 TWMUTWMU Oropharyngeal swallowing in craniocervical dystonia. Ertekin C, et al: JNNP2002 Oct;73(4):406-11.

25 patients with cranio-cervical dystonia

Dysphagia was suspected in 36% (clinical assessment). 72% on electrophysiological evaluation

Observations supported the neurogenic cause of dysphagia in patients with any kind of cervical dystonia.

T.Taira,T.Taira, 2009 2009 TWMUTWMU Laryngo-pharyngeal dystonia

swallowing difficulty

Before GPi DBS After

T.Taira,T.Taira, 2009 2009 TWMUTWMU Dystonia Movement Scale (Burke et al)

Movement Scale

T.Taira,T.Taira, 2009 2009 TWMUTWMU Burke-Fahn-Marsden scale

provoking severity weight product factor factor

Eyes 0-4 0-4 0.5 0-8

Mouth 0-4 0-4 0.5 0-8

Speech/ 0-4 0-4 1 0-16 Swallowing

T.Taira,T.Taira, 2009 2009 TWMUTWMU

case duration BMF scale (EMS) F/U (yrs) preope postope (mos)

1 39M 1.5 18 0 34 2 49M * 2.5 16 2 25 3 41M 3 24 0 25 4 48M 3 18 4 18 5 63M 4 16 0 16 6 43F* 3 20 2 16 7 42F* 3 16 6 16 8 81M 4 20 20 10 mean 48 2.8 18 4.3 20 * history of antipsychotic medication T.Taira,T.Taira, 2009 2009 TWMUTWMU

81 year-old man orofacial-cervical laryngo-pharyngeal dystonia

GPi DBS not effective T.Taira,T.Taira, 2009 2009 TWMUTWMU Question

Do we have to change the target in GPi to maximize improvement of speech and swallowing function ? Somatotopy of motor GPi

Best stimulation parameters

T.Taira,T.Taira, 2009 2009 TWMUTWMU Deep brain stimulation for dystonia confirming a somatotopic organization in the globus pallidus internus Vayssiere N, et al. J. Neurosurg. 2004, 101(2):181-8.

a location more anterior for the inferior limb and one more posterior for the superior limb for the right side, but not for the left side. more more posterior for head and neck ????

T.Taira,T.Taira, 2009 2009 TWMUTWMU The effect of electrode contact location on clinical efficacy of pallidal deep brain stimulation in primary generalised dystonia. Tisch S, et al J NNP 2007 Apr 18;

greater improvement was for posteroventral than anterodorsal stimulation for the arm and trunk .

For the leg, posteroventral and anterodorsal stimulation were of equivalent efficacy.

Posteroventral GPi stimulation provides the best overall effect and is superior for the arm and trunk.

T.Taira,T.Taira, 2009 2009 TWMUTWMU

Pantaleo Romanelli et al, Somatotopy in the : experimental and clinical evidence for segregated sensorimotor channels Brain Research Reviews 48 (2005) 112– 128 GPi DBS for dystonia with speech problems More ventral, more posterior, and more medial ? T.Taira,T.Taira, 2009 2009 TWMUTWMU !GPi DBS: four electrodes

T.Taira,T.Taira, 2009 2009 TWMUTWMU 85 cases, GPi DBS with four electrodes used not used 56 (66 %) 10 (12 %)

Rt Lt SD Rt Lt

8 (9 8%) 11 (13%)

Rt Lt Rt Lt T.Taira,T.Taira, 2009 2009 TWMUTWMU Speech problem ()*

prevalence 0.8 - 1.2% in adults 5 % in children male dominant (2-5:1)

Lewis Carroll, the well-known author of Alice in Wonderland, was afflicted with a stammer, as were his siblings. T.Taira,T.Taira, 2009 2009 TWMUTWMU Is stuttering a functional dystonia? Le Huche F, Charpy N. Rev Laryngol Otol Rhinol (Bord). 2002;123(5):329-32. [in French]

Stuttering can be legitimately considered as a dystonia affecting the articulation of speech.

Stuttering and the basal ganglia circuits: a critical review of possible relations. Alm PA. , J Commun Disord. 2004 37(4):325-69.

The basal ganglia-thalamocortical motor circuits are likely to play a key role in stuttering. The core dysfunction in stuttering is impaired ability of the basal ganglia to produce timing cues for the initiation of the next motor segment in speech. Similarities between stuttering and dystonia are indicated. T.Taira,T.Taira, 2009 2009 TWMUTWMU

A case control study of idiopathic Fletcher et al: Mov Disord 6:304-309, 1991

Family History of stuttering 8 in 71 persons with torsion dystonia 1 in the control group

Both stuttering and dystonia are: task-specific as in focal dystonia improved by blocking sensory feedback

T.Taira,T.Taira, 2009 2009 TWMUTWMU Stuttering23and Dystonia Stuttering may be a type of action dystonia Kiziltan G, et al: Mov Disord. 1996 ; 11: 278-82

Subcortical infarction resulting in acquired stuttering Ciabarra AM, et al: JNNP 2000 ; 69: 546-9 Acquired and persistent stuttering as the main symptom of striatal infarction Carluer L, et al: Mov Disord. 2000 ; 15: 343-6 Cessation of stuttering after bilateral thalamic infarction Muroi A, et al: . 1999 ; 53: 890-1

The Basal Ganglia's Possible Role in Stuttering: An Examination of Similarities between Stuttering, Tourette Syndrome, Dystonia, and other Neurological-Based Disorders of Movement, Molt P., International Stuttering Awareness Conference , 1999 T.Taira,T.Taira, 2009 2009 TWMUTWMU Acquired stuttering after pallidal deep brain stimulation for dystonia Nebel A, et al J Neural Transm. 2009 Feb;116(2):167-9

We report two patients, in whom stuttering evolved as an adverse effect of pallidal deep brain stimulation for treating dystonia. Speech dysfluency was observed under conditions that optimally suppressed dystonic symptoms without inducing other extrinsic stimulation effects. This emphasizes a role of the sensorimotor part of the internal globus pallidus in regulating speech fluency.

T.Taira,T.Taira, 2009 2009 TWMUTWMU Voice tremor and thalamic DBS

Moringlane JR, et al: Bilateral high-frequency electrical impulses to the thalamus reduce voice tremor A case report. Eur Arch Otorhinolaryngol. 2004 261:334-6

Sataloff RT, et al: Vocal tremor reduction with deep brain stimulation: a preliminary report. J Voice. 2002 16:132

Yoon MS, et al: Vocal tremor reduction with deep brain stimulation. Stereotact Funct Neurosurg. 1999;72:241-4

Taha JM, et al: Thalamic deep brain stimulation for the treatment of head, voice, and bilateral limb tremor. J Neurosurg. 1999 91:68-72 T.Taira,T.Taira, 2009 2009 TWMUTWMU Neurosurgical DBS strategy for speech disturbance

spasmodic dysphonia bilateral GPi DBS segmental dystonia stuttering ???

voice tremor (ET) bilateral Vim DBS

T.Taira,T.Taira, 2009 2009 TWMUTWMU Conclusions

Speech disturbance in dystonic patients is not always due to laryngeal dystonia. Dystonic speech and swallowing dysfunctions may improve dramatically after GPi DBS. GPi DBS for stuttering may become relevant in near future.

T.Taira,T.Taira, 2009 2009