cerebellar-degenerative-ataxia

Cerebellar Degenerative Ataxia is a rare inherited condition that leads to progressive problems with voluntary movement. Much is yet to be understood about this rare condition, which is why patients diagnosed with it are often at a loss about where to seek help. Here, we offer a brief guide to what Cerebellar Degenerative Ataxia looks like and answer some commonly asked questions about it.

Understanding Cerebellar Degenerative Ataxia

Cerebellar Degenerative Ataxia is a type of Spinocerebellar Ataxia, which is a genetic disease caused by either a recessive or dominant gene. It refers to a group of ataxias that are known to be hereditary and cause progressive harm to the cerebellum. This part of the brain maintains balance and controls movements. Spinocerebellar Ataxia may result in non-coordinated gait, impaired hand-eye coordination, and abnormal speech. Since this condition affects the nervous system, it is known as a nervous disorder. Treatment in the form of neuro-rehabilitation, as well as Physical and Occupational Therapy, and Stem Cell Therapy can manage the symptoms.

Symptoms of Cerebellar Degenerative Ataxia

Cerebellar Degenerative Ataxia affects the neurons in the cerebellum, which controls muscle coordination and balance. It can also impact other parts of the central nervous system like the brain stem, cerebral cortex, medulla oblongata, and spinal cord. Cerebellar Degenerative Ataxia symptoms, therefore, mostly affect voluntary movements. They typically manifest when the patient is middle-aged, although this can vary. Classic cerebellar degeneration symptoms to watch out for include:

  • Wide-legged, unsteady gait with the trunk moving back and forth
  • Frequent falls
  • Uncoordinated movement in the limbs and/or fingers 
  • Tremors in the limbs
  • General clumsiness
  • Dizziness
  • Nystagmus (repetitive, involuntary eye movements)
  • Double vision
  • Slow, slurred speech
  • Difficulties with swallowing 
  • Difficulties with fine motor skills like writing
  • Psychiatric symptoms 
  • Memory and learning problems

As symptoms progress, the patient may lose the ability to walk altogether and require a wheelchair to move around.

Diagnosing Cerebellar Degenerative Ataxia

If the patient has been displaying the symptoms listed above, it is essential to get a check-up as soon as possible. The doctor will examine the patient’s movement, muscle tone, gait, and coordination and ask questions about when the symptoms first appeared, how frequently they appear, and what factors appear to improve or worsen the symptoms. They will then conduct some standard tests to eliminate other possible causes of the symptoms before recommending genetic testing. Cerebellar Degenerative Ataxia is a genetic condition and can be inherited in an autosomal recessive, autosomal dominant, or X-linked fashion. Particularly if there is a family history of Spinocerebellar Ataxia, therefore, this is a crucial step. The doctor may also conduct other tests like an MRI, lumbar puncture, or CT scan to assess the location and extent of the damage in the brain and central nervous system. This is also useful in case the exact gene responsible for the Cerebellar Degenerative Ataxia cannot be identified, which is what happens in a good number of cases.

Treatment for Cerebellar Degenerative Ataxia

In general, life expectancy is shorter than normal for those with Cerebellar Degenerative Ataxia, although many patients live well into their 50s or even their 60s. Doctors will typically prescribe a treatment plan based on the patient’s symptoms and needs with the goal of slowing down disease progression and enabling more functionality. The components of a Cerebellar Degenerative Ataxia treatment program will usually include:

  • Stem Cell Therapy: This revolutionary form of treatment involves using the patient’s own healthy cells to treat the ones damaged by cerebellar degeneration. The process is safe, easy, and allows patients to go home the very same day.
  • Physical Therapy: This is one of the key components of Cerebellar Degenerative Ataxia treatment. Physical therapists will teach a variety of exercises to enhance strength, improve flexibility and mobility, enhance coordination, and correct gait. They will also guide the patient through cardiovascular exercises for general fitness.
  • Occupational Therapy: This form of therapy focuses on teaching patients how to accomplish basic tasks like eating and getting dressed on their own, often with the aid of devices like specially made cutlery. The therapist will also provide suggestions on adjustments that can be made around the home for the patient’s safety, such as adding ramps for wheelchair usage and carpeting slippery floors to avoid injuries in the case of a fall. 
  • Assistive devices: As the patient’s motor abilities decline, they will need devices like canes, walkers, and wheelchairs to move around. Therapists can teach the patient how to operate and maintain each of these.
  • Medication: Certain medications can help with specific symptoms of Cerebellar Degenerative Ataxia, such as tremors, double vision, slurred speech, or impaired hearing.
  • Speech Therapy: Speech therapists work with patients whose mouth and jaw muscles have been affected by cerebellar degeneration. They teach patients how to chew their food properly, how to swallow, better articulation techniques, and how to control their breathing.
  • Psychiatric Therapy: Cerebellar degeneration frequently impacts the patient’s psychological health, and therapy to deal with depression, anxiety, anger issues, and other conditions may help.

It is also recommended that patients find a local support group that can connect them to important resources, such as information about new drug trials, and also offer emotional companionship with other patients. Caregivers and loved ones of the patient should also seek support and assistance as necessary. In this context, we also mention that parents worried about passing the Cerebellar Degenerative Ataxia gene to their children can get tested for it at genetic registries before planning a family.

FAQs

  • How long can you live with Cerebellar Ataxia?

In general, life expectancy is shorter than usual for Cerebellar Degenerative Ataxia patients. Many, however, may live into their 50s or even their 60s.

  • What is the best treatment for cerebellar degeneration?

Treatment for cerebellar degeneration focuses on managing symptoms and slowing the rate at which they progress. This includes Stem Cell Therapy, and certain medications that may be used to treat specific symptoms like muscle tremor or vision problems.

  • What vitamin is good for Ataxia?

Studies have shown that Vitamin E supplements can stabilize the neurological symptoms of Cerebellar Degenerative Ataxia, especially in the early stages.

  • How quickly does Ataxia progress?

The rate of progress for Ataxia can vary from patient to patient depending on the symptoms and the age of onset. It can take anywhere between several months to several years.

  • Is cerebellar degeneration fatal?

In general, Cerebellar Degenerative Ataxia is progressive and ultimately fatal, with most adults not making it past middle age.

  • Does Cerebellar Ataxia get worse?

Cerebellar ataxia is a progressive disease and the symptoms worsen as the neuron degeneration continues.

  • What is the most common cause of Cerebellar Ataxia?

Cerebellar Ataxia occurs due to damage to the cerebellum, which occurs due to inherited gene factors.

  • Is Ataxia considered a disability?

Yes, Ataxia can have a disabling effect on one’s life.

  • Is Ataxia an autoimmune disease?

Ataxia is generally classed as a neurological disease, as it occurs due to damage in the brain and central nervous system.

  • Is Ataxia a symptom of MS?

In serious cases of Multiple Sclerosis, cerebellar degeneration can lead to symptoms like ataxia or tremors.

  • What are the 3 types of Ataxia?

The three types of ataxia are proprioceptive, cerebellar, and vestibular.

While Cerebellar Degenerative Ataxia is a rare and serious condition to live with, the right support can go a long way in enhancing the patient’s quality of life. Patients and their families should read up as much as they can on the condition and ask their healthcare team as many questions as necessary. Above all, they should continue to do the things they love in conjunction with their cerebellar degeneration treatment so that they can enjoy a happy and fulfilling life.

Dr Na'eem Sadiq is a respected stem cell specialist at Plexus, and a prominent neurologist in Bangalore. He studied neurology and clinical neurophysiology in London, and worked with some of the most prestigious medical institutions in England, and the Middle East. He completed his MBBS at Bellary Government Medical College, and a postgraduate degree in psychiatry from NIMHANS in Bangalore.

Dr Na'eem has perfected his knowledge and expertise in Continuing medical education (CME), and training in tissue culture, Stem Cell Therapy, and neurology. Dr Na'eem Sadiq possesses an undying passion to improve people’s lives. This led to the creation of Plexus, a neuro and Stem Cell Research centre in Bangalore in neurosurgery, and neurorehabilitation.