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    • Cerebral Contusion and Intracerebral Hematoma
    • Chordomas
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    • Chronic Subdural Hematomas
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    • Oligodendroglioma
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    • Osteoarthritis of the Spine
    • Osteomyelitis
    • Osteoporotic Vertebral Fractures
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    • Pediatric Hydrocephalus
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    • 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
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    • Scoliosis
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UCLA Neurosurgery

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
  • 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
  1. Home
  2. Conditions Treated
  3. 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.
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