Cerebellar Ataxia Syndrome

Ataxia is a term that means incoordination, unsteadiness or wobbliness. Cerebellar ataxia is ataxia that is caused by problems with the cerebellum. The cerebellum is the area of your brain responsible for coordination and balance.

Common symptoms of cerebellar ataxia syndrome include:

• Clumsiness in daily activities

• Difficulty speaking or clumsy speech (dysarthria)

• Difficulty walking or unsteady gait

• Dizziness and headache

• Nausea and vomiting

• Uncoordinated body movements

• Uncoordinated eye movements (nystagmus)

The sudden appearance of the symptoms of cerebellar ataxia syndrome may be a sign of a serious and even life-threatening condition, including stroke, poisoning, encephalitis, or brain abscess. Seek immediate medical care (call 911) if you, or someone you are with, have a sudden onset of any of the above symptoms of cerebellar ataxia syndrome.

Cerebellar ataxia syndrome is caused by damage to or problems with the cerebellum. The cerebellum is the part of your brain that is responsible for coordination and balance. A wide variety of diseases, disorders and conditions can lead to problems with the cerebellum and cause ataxia.

Cerebellar ataxia syndrome can be categorized based on the cause of the problem.

Autoimmune and inflammatory causes may include:

• Behcet’s disease (disease that causes damage to blood vessels, especially the veins)

• Celiac disease (severe sensitivity to gluten from wheat and other grains that causes intestinal damage)

• Multiple sclerosis (disease that affects the brain and spinal cord causing weakness, coordination and balance difficulties, and other problems)

• Paraneoplastic degeneration (rare immune system disorder that attacks the cerebellum)

Congenital causes are disorders that are present at or before birth. They may include:

• Agenesis (failure of the cerebellum to fully develop)

• Chiari malformation (herniation of the cerebellum through the opening in the base of the skull)

• Dandy-Walker cyst (congenital malformation of the cerebellum)

• Joubert syndrome (absence or underdevelopment of the cerebellum)

There are many rare genetic disorders that can cause cerebellar ataxia syndrome including:

• Ataxia telangiectasia (rare disorder that causes degeneration of the cerebellum)

• Friedreich’s ataxia (rare disease that causes degeneration of the cerebellum, spinal cord, and peripheral nerves)

• Kearns-Sayre syndrome (rare neuromuscular disorder caused by DNA abnormalities)

• Mitochondrial encephalopathy with ragged red fibers (MERRF, a metabolic disorder)

• Mitochondrial encephalopathy, lactic acidosis, stroke-like episodes (MELAS syndrome, a metabolic disorder)

• Multiple systems atrophy (rare, progressive neurological disorder)

• Neuropathy, ataxia, retinitis pigmentosa (NARP, a metabolic disorder)

• Spastic ataxia of Charlevoix-Saguenay (gene mutation)

Infectious causes of cerebellar ataxia syndrome may include:

• Chickenpox

• Coxsackie disease (viral infection also called hand-foot-and-mouth disease)

• Creutzfeldt Jakob disease (rare disease believed to be an infection that causes mental deterioration)

• Encephalitis or brain abscess (brain inflammation or infection)

• Epstein-Barr virus

• HIV (human immunodeficiency virus)

• Lyme disease (inflammatory bacterial disease spread by ticks)

• Mycoplasma pneumonia (type of bacterial pneumonia)

Metabolic causes of cerebellar ataxia syndrome may include:

• Hypoparathyroidism (underactive parathyroid gland)

• Thyroid diseases

• Vitamin B₁₂ deficiency

• Vitamin E deficiency

Medications that may cause cerebellar ataxia syndrome include:

• Amiodarone (Cordarone)

• Carbamazepine (Tegretol)

• Certain cancer chemotherapies

• Cyclosporine (Gengraf, Neoral, Sandimmune)

• Lithium (Eskalith, Lithobid)

• Phenytoin (Dilantin)

Toxins and poisons that may cause cerebellar ataxia syndrome include:

• Alcohol

• Carbon monoxide

• Heavy metals, such as mercury, lead and thallium

• Heroin

• Organic solvents, such as toluene and benzene

• Phencyclidine (PCP)

Other causes of cerebellar ataxia syndrome may include:

• Brain tumors

• Stroke and other vascular conditions

• Trauma

A number of factors are thought to increase your chances of developing cerebellar ataxia syndrome. Common risk factors include:

• Exposure to certain drugs or toxins

• Having a family member with a genetic form of cerebellar ataxia

• Head trauma

• Recent vaccination

• Viral infections

Treatment of cerebellar ataxia syndrome varies depending on the cause, severity, and other factors. Cerebellar ataxia caused by viral infections will often go away on its own within a few months. Treating the underlying cause, if one is identified, can help improve problems with balance and coordination. Otherwise, treatment is supportive and is aimed at managing your symptoms and assisting with activities of daily life.

Treatment of cerebellar ataxia syndrome may include:

• Devices to assist with walking and other activities

• Medications to improve balance, tremor and nystagmus (rapid, uncontrolled eye movements)

• Nutritional counseling and therapy to correct vitamin deficiencies and improve your overall health and wellness

• Occupational therapy to improve your functionality

• Physical therapy to strengthen your muscles and reduce tension

• Speech therapy to improve speaking and communication

• Surgery to treat certain underlying causes of cerebellar ataxia such as a brain tumor

• Swallowing therapy to strengthen your swallowing muscles

Complications of the underlying causes of cerebellar ataxia syndrome and the prognosis vary based on the disease, disorder or condition. You can best treat cerebellar ataxia syndrome and its underlying cause, lower your risk of complications, or delay the development of complications by following the treatment plan you and your healthcare professional design specifically for you.

Complications of cerebellar ataxia syndrome include:

• Difficulties with work and school

• Difficulty performing everyday activities, such as walking, communicating and self-care

• Disability

• Falls and injuries