eMedicine Specialties > Neurology > Movement and Neurodegenerative Diseases

Movement Disorders in Individuals with Developmental Disabilities: Treatment & Medication

Author: Norberto Alvarez, MD, Assistant Professor, Department of Neurology, Harvard Medical School; Consulting Staff, Department of Neurology, Boston Children's Hospital
Contributor Information and Disclosures

Updated: May 6, 2009

Treatment

Medical Care

  • General considerations
    • In general, pediatricians and pediatric neurologists treat developmentally disabled people with and without mental retardation.
    • With the present trend of placing patients with developmental disability in community settings and with the expectation that community physicians will provide services, these patients are seen in adult clinical practices more frequently now than before.
    • In 1970, almost 190,000 persons with a handicap or mental retardation were living in institutions in the United States. By 1995, the number was reduced to approximately 63,000; the ultimate goal is to close these institutions. In many instances, caring for these patients requires a multidisciplinary approach.
    • In 1999, the American Board of Medical Specialties approved a conjoint certification by the American Board of Pediatrics and the American Board of Psychiatry and Neurology in the field of neurodevelopmental disabilities in child neurology. These new developmentally oriented neurologists, who will be trained in both adult and child neurology, are expected to improve the care of children and adults with developmental disabilities.
  • Cerebral palsy
    • Medications are indicated to diminish spasticity and treat movement disorders. However, no good medication is available, and the adverse effects might outweigh the benefits.
      • Diazepam and dantrolene sodium are used commonly to decrease muscle tone.
      • Botulinum toxin injected directly into spastic muscles, oral baclofen, and intrathecal baclofen might help to decrease muscle tone and severity of spasticity.
    • Physical therapy is indicated to decrease muscle tone, maintain function, and prevent deformities.
    • Several forms of rehabilitation therapies based on theories such as neurodevelopmental treatments, sensory integration, patterning, and conductive education are used, though no definitive evidence supports their use.
  • Delayed-onset movement disorder
    • Treatment options include botulinum toxin injections, trihexyphenidyl, tetrabenazine, benzodiazepines, intrathecal baclofen, and thalamotomy.
    • Results of these treatments are inconsistent and unproven.
  • Mild neuromotor disabilities
    • No specific medical treatment is known.
    • Patients benefit from training with occupational or physical therapy.
    • In many instances, the neuromotor disabilities are associated with hyperactivity or attention deficit disorders. In such cases, methylphenidate, amphetamines, or amoxetine might be useful for the treatment of these conditions.
  • Tardive dyskinesia
    • Several drugs have been tried, but none, including reserpine, tetrabenazine, bromocriptine, lecithin, choline, and benzodiazepines, have been proven effective.
    • Responses to these pharmacologic agents have been inconsistent.
    • The best strategy is to discontinue the neuroleptic medications, if possible.
    • In many patients, decreasing the neuroleptic is enough to decrease the symptoms.
    • Some studies suggest that the anticonvulsant levetiracetam may be useful in the treatment of tardive dyskinesia.66
  • Bruxism
    • No useful medical treatment is known.
    • A mouth guard or oral appliance might be indicated if the grinding noise is the main complaint.
    • No evidence supports or rejects the use of occlusants for the treatment of bruxism.67
    • Other options are behavior modification and biofeedback.
  • Stereotypies, SIB
    • No good medications are known.
    • Clinicians frequently use medications on the basis of unproven theories.
      • Neuroleptics, serotonin reuptake inhibitors, naltrexone, and naloxone have been used for SIB.
      • Results are variable.
      • Researchers quantitatively analyzed peer-reviewed publications from 1983-2003 that reported the use of naltrexone in people with developmental disabilities and other related condition (eg, autism) to treat SIB. About 80% of the subjects so treated had improvement, and in 47% SIB was reduced by at least 50%. Most subjects had severe-to-profound mental retardation, and around one third had autism. Doses of naltrexone were 12.5-200 mg/day; most were 50-100 mg/day. High doses seemed to be more effective than low doses, and male subjects had better responses than female subjects. Outcome was not related to age, presence or absence of autism, or topography.
      • Some reports described positive long-term effects lasting 1 year or more.
  • Drug-induced movement disorder
    • The best strategy is to stop the offending medication.
    • In the acute phase, sedatives (eg, diazepam) or anticholinergics (eg, diphenhydramine) might be effective symptomatic treatments.

Surgical Care

No specific surgical treatment exists for the movement disorders; however, some of the related conditions are indications for surgical procedures.

  • Cerebral palsy
    • Orthotic devices prevent deformities and improve function. An example is ankle-foot orthosis (AFO) to control motion of the foot and ankle.
    • Several surgical procedures might be indicated to relieve spasticity, maximize function, and prevent deformities.
      • These procedures are an adjunct to medical therapies and usually are indicated when fixed contractures interfere with function.
      • For example, lengthening the Achilles tendon is a common procedure for facilitating gait.
    • Ventral posterolateral thalamotomy may be indicated in the extrapyramidal movement disorders. However, this is not a common procedure in these cases.
    • Rhizotomy (cutting the dorsal roots of the spinal cord) permanently interrupts the spinal cord reflex that is responsible for spasticity. Ideal candidates for this surgery are children with spastic diplegia.
    • Cerebellar stimulation showed initial efficacy in the treatment of spasticity and athetosis, but this method is no longer used.
    • Electrical stimulation increases passive range of motion in children with hemiplegia, but this technique requires further study.

Consultations

Consultation with a neurologist or a pediatrist is recommended to manage the movement disorder and to evaluate the cause of the mental retardation.

  • Cerebral palsy
    • Orthopedic surgeons are usually involved from early stages. They should be consulted for the treatment of any condition that results in impairment of function.
    • Examples include scoliosis and spasticity leading to contractures.
  • Bruxism
    • Consultation with an oral surgeon may be indicated.
    • Sleep disorders might be associated with bruxism.

Diet

Special diet is indicated only in cases of specific metabolic disorders (eg, phenylketonuria).

Activity

No limitations on activity are necessary except those imposed by the motor or movement disorder.

Medication

The objective of pharmacotherapy is to reduce morbidity and prevent complications.

Skeletal muscle relaxants

These agents usually inhibit transmission of both monosynaptic and polysynaptic reflexes at the spinal cord level.


Baclofen (Lioresal)

Oral gamma-aminobutyric acid (GABA) agonist that causes presynaptic inhibition of small interneurons in spinal cord. Mean therapeutic half-life of 3.5 h (range, 2-6 h). Most clinical experience from patients with multiple sclerosis or spinal pathology. Few studies in spasticity of central origin.
For intrathecal (IT) administration, pump is implanted SC and catheter implanted in subarachnoid space of spinal canal (where medication is administered). Less medication needed and systemic effects decreased. Half-life approximately 5 h.

Adult

PO: 5 mg PO tid initially; may increase by 5 mg/dose qwk; maximal dose usually around 80 mg/d, though up to 150 mg has been used
IT: Approximately 1% of oral dose; initial dose 25 mcg/d; increase slowly; not to exceed 300-400 mcg/d

Pediatric

<2 years: Not established
> 2 years: 2.5-5 mg/d PO initially
2-7 years: Not to exceed 30 mg/d
> 8 years: Not to exceed 60 mg/d
IT (<18 y): Not established

Opiate analgesics, benzodiazepines, alcohol, tricyclic antidepressants, guanabenz, monoamine oxidase inhibitors (MAOIs), clindamycin, and hypertensive agents may increase effects

Documented hypersensitivity; nursing mothers

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution when spasticity needed to sustain upright posture and balance in locomotion; caution in patients with autonomic dysreflexia; teratogenic effects in animals reported; mothers taking baclofen should not nurse; excreted in urine; reduce dose if kidney function impaired; periodically monitor liver function; may interfere with seizure control; common adverse effects include sedation, drowsiness, and fatigue; may exacerbate psychiatric disorders; IT form not FDA approved for pediatric use; safety in children <18 y not established; pump dysfunction can lead to overdose with subsequent intoxication or failure then withdrawal symptoms


Dantrolene sodium (Dantrium)

Acts peripherally at muscle fiber rather than neural level; reduces muscle action potential–induced release of calcium and affects intrafusal and extrafusal fibers and spindle sensitivity. No action on smooth or cardiac muscle tissue. Benefits seem to be greater in children with spasticity than in adults.

Adult

25 mg/d PO; not to exceed 100 mg PO qid

Pediatric

<5 years: Not established
> 5 years: 0.5 mg/kg PO bid; titrate slowly prn; not to exceed 3-4 mg/kg qid or 100 mg/d

Hepatotoxicity occurs most often in women >35 y who are receiving estrogens; combination with verapamil not recommended; clofibrate and warfarin may reduce plasma protein binding of dantrolene

Documented hypersensitivity; active hepatic disease, such as hepatitis or cirrhosis; nursing mothers; concomitant verapamil

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in impaired pulmonary or cardiac function; hepatotoxicity is serious adverse effect (monitor liver function before and during therapy); may cause sedation and muscle weakness; use only for conditions as recommended; may cause photosensitivity with exposure to sunlight

Benzodiazepines

Agents in this category are effective as skeletal muscle relaxants.


Diazepam (Valium)

GABAergic effect increases presynaptic inhibition and reduces monosynaptic and polysynaptic reflexes. Peak absorption in 1 h; half-life 20-40 h. Effective in children and adults; in children, effective in spastic and choreic forms of CP. Suppresses spasms that interfere with sleep. Various dosing regimens may be used.

Adult

5 mg PO hs and smaller dose (eg, 2 mg) during day; titrate depending on tolerance (maximum depends on adverse effects); not to exceed 60 mg/d

Pediatric

0.1-0.8 mg/kg/d PO; titrate slowly

Toxicity in CNS increased by phenothiazines, barbiturates, alcohols, and MAOIs; cisapride significantly increases toxicity

Documented hypersensitivity, acute narrow-angle glaucoma

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Generally safe but might produce CNS depression in newborns; true physiologic addiction might occur, with withdrawal symptoms on discontinuation

Neuromuscular Blocking Agents, Toxin

These agents can be used for the treatment of muscle spasticity.


Botulinum toxin A (Botox®)

One of several toxins produced by Clostridium botulinum. Type A toxin approved for treatment of spasticity and other conditions (eg, blepharospasm and dystonia). Inhibits release of acetylcholine in neuromuscular junction; causes reversible denervation atrophy and reinnervation due to sprouting from unmyelinated terminal axon and nodes of Ranvier. Injected directly into spastic muscle.

Adult

Various regimens used depending on size of muscle and severity of symptoms; in some muscles, only 1 site injected; in others, >2 sites injected; not to exceed 50 U IM per injection site; not to exceed 400 U per visit; wait at least 3 mo before reinjection

Pediatric

1-4 U/kg or 80-400 U IM; not to exceed 12 U/kg or 400 U total per visit; not to exceed 400 U/kg in 3 mo; long-term safety not established; efficacy as in adults

Aminoglycoside antibiotics or any other drug that interferes with neuromuscular transmission may potentiate effect

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Generally safe; poisoning by accidental overdose unlikely; resistance associated with circulating antibodies may develop; may cause pain and irritation

More on Movement Disorders in Individuals with Developmental Disabilities

Overview: Movement Disorders in Individuals with Developmental Disabilities
Differential Diagnoses & Workup: Movement Disorders in Individuals with Developmental Disabilities
Treatment & Medication: Movement Disorders in Individuals with Developmental Disabilities
Follow-up: Movement Disorders in Individuals with Developmental Disabilities
References
Further Reading
[ CLOSE WINDOW ]

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Further Reading

Clinical guidelines

Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Ashwal S, Russman BS, Blasco PA, Miller G, Sandler A, Shevell M, Stevenson R. Practice parameter: diagnostic assessment of the child with cerebral palsy: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2004 Mar 23;62(6):851-63. [77 references] PubMed

Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and The Practice Committee of the Child Neurology Society. Shevell M, Ashwal S, Donley D, Flint J, Gingold M, Hirtz D, Majnemer A, Noetzel M, Sheth RD. Practice parameter: evaluation of the child with global developmental delay: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology 2003 Feb 11;60(3):367-80. [123 references] PubMed

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Keywords

mental retardation, MR, cerebral palsy, CP, Down syndrome, delayed-onset movement disorder, mild neuromotor disabilities, tardive dyskinesia, TD, bruxism, stereotypies, autism, drug-induced movement disorder, Smith-Magenis syndrome, Lesch-Nyhan syndrome, Prader-Willi syndrome, obsessive-compulsive disorder, Rett syndrome, pyramidal cerebral palsy, extrapyramidal cerebral palsy, developmental disability, movement disability, limited intellectual capacity, limited intellect, biomedical mental retardation, social mental retardation, behavioral mental retardation, educational mental retardation, self-injurious behavior, SIB

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Contributor Information and Disclosures

Author

Norberto Alvarez, MD, Assistant Professor, Department of Neurology, Harvard Medical School; Consulting Staff, Department of Neurology, Boston Children's Hospital
Norberto Alvarez, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

Rodrigo O Kuljis, MD, Esther Lichtenstein Professor of Psychiatry and Neurology, Director, Division of Cognitive and Behavioral Neurology, Department of Neurology, University of Miami School of Medicine
Rodrigo O Kuljis, MD is a member of the following medical societies: American Academy of Neurology and Society for Neuroscience
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Nestor Galvez-Jimenez, MD, MSc, MHA, Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida
Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

 
 
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