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Conditions Treated
Conditions Treated
Conditions Treated
Acoustic Neuroma
Adult Tethered Cord
Acromegaly
Acute Subdural Hematomas
Anaplastic Astrocytoma (AA)
Ankylosing Spondylitis
Aqueductal Stenosis
Arachnoid Cysts
Arnold Chiari Malformation
Arteriovenous Malformation (AVM)
Astrocytoma
Ballism
Basilar Invagination
Brachial Plexus Injury
Brain Aneurysm
Brain Attack (Stroke)
Brain AVM
Brain Conditions
Brain Metastases
Brainstem Glioma
Carotid Dissection
Carotid Stenosis
Carpal Tunnel Syndrome
Causalgia
Cavernous Angioma
Cerebral Aneurysms
Cerebral Contusion and Intracerebral Hematoma
Chordomas
Chorea
Chronic Subdural Hematomas
Colloid Cyst
Coma
Concussion
Congenital Dermal Sinus
Cranial GunShot Wounds
Craniopharyngioma
Craniosynostosis
Cushing's Disease
Cyst Epidermoid Tumor
Dandy Walker Syndrome
Degenerative Disc Disease
Dermoid Tumor
Disc Herniation
Dural Arteriovenous Malformations
Dystonia
Ependymoma
Epidermoid Tumor (Cyst)
Epidural Hematomas
Epilepsy
Essential Tremor
Extratemporal Lobe Epilepsies
Facet Joint Syndrome
Fibromyalgia
Frontal Lobe Epilepsy
Ganglioglioma
Glioblastoma
Germinoma
Glioma
Glomus Jugulare Tumor
Glossopharyngeal Neuralgia
Hemangioblastomas
Hemi-Facial Spasm
Hydrocephalus
Hyperhidrosis
Intracerebral Hemorrhage
Intracranial Hypotension
JPA
Low-Grade Astrocytoma
Lymphocytic Hypophysitis
Lymphoma
Malignant Nerve Sheath Tumors
Medulloblastoma
Meningioma Brain Tumor
Meralgia Paresthetica
Metastatic Brain Tumors
Moyamoya Disease
Myelomeningocele
Myelopathy
Nelson's Syndrome
Neurocysticercosis
Neurofibromatosis Type 2 and Schwannomatosis
Normal Pressure Hydrocephalus
Oligodendroglioma
Optic Nerve Glioma
Osteoarthritis of the Peripheral Joint
Osteoarthritis of the Spine
Osteomyelitis
Osteoporotic Vertebral Fractures
Parkinsons Syndrome
Pediatric Conditions
Pediatric Hydrocephalus
Peripheral Nerve Injury
Phantom Limb Pain
Pineal Tumor
Pineoblastoma
Pineocytoma
Platybasia
Postherpetic Neuralgia
Post-Traumatic Seizures
Primary CNS Lymphoma
Pseudotumor Cerebri
Radiculopathy—Cervical & Lumbar (Pinched Nerve)
Recurrent Adenomas
Rheumatoid Arthritis
Schwannomas
Scoliosis
Seizure
Skull Fracture
Slit Ventricle Syndrome
Spasticity
Spinal Arteriovenous Malformation (AVM)
Spinal Compression Fractures
Spine Conditions
Spinal Cord Injury
Spinal Cord Lipomas & Lipomyelomeningoceles
Spinal Cord Tumors
Stenosis
Subarachnoid Hemorrhage
Syringomyelia
Tethered Cord Syndrome
Thoracic Outlet Syndrome
Thyrotroph (TSH) Secreting Adenomas
Torticollis
Traumatic Hematomas
Trigeminal Neuralgia
Trochanteric Bursitis
Ulnar Nerve Entrapment
Conditions Treated
Acoustic Neuroma
Adult Tethered Cord
Acromegaly
Acute Subdural Hematomas
Anaplastic Astrocytoma (AA)
Ankylosing Spondylitis
Aqueductal Stenosis
Arachnoid Cysts
Arnold Chiari Malformation
Arteriovenous Malformation (AVM)
Astrocytoma
Ballism
Basilar Invagination
Brachial Plexus Injury
Brain Aneurysm
Brain Attack (Stroke)
Brain AVM
Brain Conditions
Brain Metastases
Brainstem Glioma
Carotid Dissection
Carotid Stenosis
Carpal Tunnel Syndrome
Causalgia
Cavernous Angioma
Cerebral Aneurysms
Cerebral Contusion and Intracerebral Hematoma
Chordomas
Chorea
Chronic Subdural Hematomas
Colloid Cyst
Coma
Concussion
Congenital Dermal Sinus
Cranial GunShot Wounds
Craniopharyngioma
Craniosynostosis
Cushing's Disease
Cyst Epidermoid Tumor
Dandy Walker Syndrome
Degenerative Disc Disease
Dermoid Tumor
Disc Herniation
Dural Arteriovenous Malformations
Dystonia
Ependymoma
Epidermoid Tumor (Cyst)
Epidural Hematomas
Epilepsy
Essential Tremor
Extratemporal Lobe Epilepsies
Facet Joint Syndrome
Fibromyalgia
Frontal Lobe Epilepsy
Ganglioglioma
Glioblastoma
Germinoma
Glioma
Glomus Jugulare Tumor
Glossopharyngeal Neuralgia
Hemangioblastomas
Hemi-Facial Spasm
Hydrocephalus
Hyperhidrosis
Intracerebral Hemorrhage
Intracranial Hypotension
JPA
Low-Grade Astrocytoma
Lymphocytic Hypophysitis
Lymphoma
Malignant Nerve Sheath Tumors
Medulloblastoma
Meningioma Brain Tumor
Meralgia Paresthetica
Metastatic Brain Tumors
Moyamoya Disease
Myelomeningocele
Myelopathy
Nelson's Syndrome
Neurocysticercosis
Neurofibromatosis Type 2 and Schwannomatosis
Normal Pressure Hydrocephalus
Oligodendroglioma
Optic Nerve Glioma
Osteoarthritis of the Peripheral Joint
Osteoarthritis of the Spine
Osteomyelitis
Osteoporotic Vertebral Fractures
Parkinsons Syndrome
Pediatric Conditions
Pediatric Hydrocephalus
Peripheral Nerve Injury
Phantom Limb Pain
Pineal Tumor
Pineoblastoma
Pineocytoma
Platybasia
Postherpetic Neuralgia
Post-Traumatic Seizures
Primary CNS Lymphoma
Pseudotumor Cerebri
Radiculopathy—Cervical & Lumbar (Pinched Nerve)
Recurrent Adenomas
Rheumatoid Arthritis
Schwannomas
Scoliosis
Seizure
Skull Fracture
Slit Ventricle Syndrome
Spasticity
Spinal Arteriovenous Malformation (AVM)
Spinal Compression Fractures
Spine Conditions
Spinal Cord Injury
Spinal Cord Lipomas & Lipomyelomeningoceles
Spinal Cord Tumors
Stenosis
Subarachnoid Hemorrhage
Syringomyelia
Tethered Cord Syndrome
Thoracic Outlet Syndrome
Thyrotroph (TSH) Secreting Adenomas
Torticollis
Traumatic Hematomas
Trigeminal Neuralgia
Trochanteric Bursitis
Ulnar Nerve Entrapment
Home
Conditions Treated
Dystonia
Dystonia
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About Dystonia
Video: Dystonia Treated with Surgically Implanted Pacemaker
Functional and Movement Disorders Program »
Overview of Dystonia
Dystonia
is a disruption in the regulation of muscle tone, usually resulting in progressive rigidity throughout the body.
Increased tone in opposing muscles limits voluntary movement. When increased tone in opposing muscles is not symmetrical, the condition may distort posture.
The most common form of dystonia is dystonia musculorum deformans, an inherited disorder. The specific genetic abnormality is unknown.
Symptoms
The first symptoms of dystonia musculorum deformans typically begin just prior to puberty but may occur as early as age 3 or as late as young adulthood.
When the disease begins early it usually involves the legs. Disruption of gait, or walking, may be the only symptom for some time.
Symptom progression varies but occurs more rapidly in patients with symptoms at an early age.
When the disease begins later in life the trunk of the body and the neck are more likely to be involved.
Dystonia may progress to the point of almost complete immobility and death from secondary complications, usually pneumonia.
Treatment
Medical therapy is available but not uniformly effective. Nonetheless, it should be tried before considering surgical intervention.
Deep Brain Stimulation
is a surgical option.
DBS selection criteria
Primary dystonia > Secondary dystonia
Patients with primary dystonia due to a mutation of the DYT-1 gene respond best to DBS therapy. Those with secondary dystonias (usually due to an identifiable trauma) generally do not benefit as much but may still be considered (especially those with perinatal diffuse anoxic injury). Other brain-injury related symptoms, such as spasticity and seizures, are also relative contraindications, as these may be a greater source of disability than dystonia.
Generalized dystonia > Focal or Segmental Dystonia
Dystonia can be generalized (occurring throughout the body), focal (occurring in specific areas, such as cervical dystonia), or segmental (affecting two adjoining body parts). Patients with generalized dystonia respond best to DBS, although some focal dystonias (e.g., cervical dystonias) may also be considered.
Medication refractory dystonia
Although medical therapy is limited, to be considered for DBS therapy, patients should have failed medical therapy such as benzodiazepines, anticholinergics, Sinemet, and Botox (in the case of focal dystonia).
Intact cognitive function
In secondary dystonia, the greatest source of disability is often cognitive dysfunction. Because, DBS can exacerbate cognitive dysfunction, significant cognitive decline is therefore a contraindication.
Brain MRI that is amenable to DBS surgery
Because dystonia can be due to brain trauma, it is important to have a screening MRI to ensure that the brain is amenable to DBS surgery and that the proposed target for DBS can be identified.
Medically suitable for surgery
Patients should be medically suitable for surgery and have controlled blood pressure to be considered for surgery. It is preferable that patients be able to tolerate 3-6 hours of awake surgery, although the surgery can be performed asleep under certain circumstances.
Realistic expectations and interest
Patients must realize that DBS therapy is not a "cure" but symptomatic therapy. In most cases, patients achieve their best response between 3 and 12 months after implantation.
Outcome
Results of surgery can be dramatic in carefully selected patients. Some improvement occurs in 50 percent to 70 percent of patients. Improvements may not be apparent for several weeks or months.