CANVAS Syndrome: All you need to know!

Published Dec 11, 2022 • By Rahul Roy

CANVAS syndrome is a rare neurological disorder that is an amalgamation of 3 disorders. It is a slowly evolving disease, of unknown origin, affecting adults between 50 and 60 years old. 
What are the 3 different conditions that form this syndrome? What are its symptoms? How can it be diagnosed and treated? 
We explain it all in this article!

CANVAS Syndrome: All you need to know!

CANVAS Syndrome is an uncommon neurological condition that affects the brain, spinal cord and the nerves. CANVAS stands for Cerebellar Ataxia (CA), Neuropathy (N) and Vestibular Areflexia (VA) Syndrome. It is an amalgamation of problems that are generally found together and is normally seen in adults aged 50 and above. It is a progressive disease that gradually gets worse over time. It is so rare that it is estimated to affect one in 5 million individuals. 

To fully understand CANVAS Syndrome, it is important to first understand the 3 different components it affects as referenced by its full form- 

  • Cerebellar Ataxia is a disorder that affects coordination, balance and speech. It is caused due to a problem with the cerebellum, which is the part of the brain that is responsible for muscle control and coordination
  • Neuropathy relates to problems with the nerves. There are different types of neuropathies depending on problems to nerves with different functions. For example, motor neuropathy relates to damage to the nerves that influence muscles and movement in the body, sensory neuropathy for affected nerves that are responsible for pain, touch etc. and autonomic nerve neuropathy relating to autonomous functions that are unconsciously undertaken such as breathing and heart beating
  • Vestibular Areflexia is a problem associated with balance and reflexes where the vestibular system located in the inner ear doesn’t function as normal. For example, this is responsible for keeping the eyes steady even when the head is moving

Therefore, an individual who is diagnosed with all 3 aforementioned disorders is said to be suffering from CANVAS syndrome. 

Causes of CANVAS Syndrome 

A lot of research and studies have been undergone to understand the reasons for the cause of CANVAS Syndrome. It has been found that in some circumstances it was caused due to hereditary dispositions while in some cases it was caused due to genetic mutations, with unclear causes of mutation. Therefore, it is difficult to pinpoint the exact cause of CANVAS syndrome. 
However, what is certain is that all cases of the syndrome showed atrophy of the cerebellum, where it reduced in size as well as damage to some types of nerves. 

Symptoms of CANVAS Syndrome 

Since CANVAS Syndrome is a combination of 3 different components- Cerebellar Ataxia, Neuropathy and Vestibular Areflexia-, it follows the symptoms of these disorders. It is also entirely possible to have symptoms of one or two disorders before developing symptoms for all three. 

Symptoms of Cerebellar Ataxia 

  • Poor Coordination 
  • Poor balance or imbalance 
  • Difficulty swallowing 
  • Impaired speech 
  • Fast involuntary eye movements 
  • Difficulty doing motor activities such as writing, eating, buttoning a shirt etc 

Symptoms of Neuropathy 

This can vary depending on the type of neuropathy- 

  • Burning or sharp pain in affected body part 
  • Constipation or diarrhea 
  • Excessive or lack of sweating 
  • Double vision 
  • Balance issues 
  • Erectile dysfunction 
  • Muscle weakness 
  • Dry mouth/eyes 

Symptoms of Vestibular Areflexia 

  • Instability or dizziness 
  • Unusual muscle coordination 
  • Loss of balance 
  • Blurry vision 

Balance tends to get worse with all three parts of the CANVAS Syndrome. 

Diagnosis of CANVAS Syndrome 

CANVAS Syndrome is generally diagnosed by a qualified health practitioner but specifically specialists such as an otologist, a neurologist or an otolaryngologist
It is especially challenging as it requires the onset of symptoms from all 3 conditions, some which may take more than 10 years to fully show up. 

The practitioner will ask questions concerning present health as well as medical history to try to understand the nature of the disorder and may conduct some tests and exams to determine the extent of the disease. Some common diagnostic tests are – 

  • MRI Tests (Magnetic Resonance Imaging) 
  • Balance Tests 
  • Nerve Conduction Studies 
  • Genetic Testing 

In case of speech issues, the doctor might recommend a visit to a speech language pathologist to assess and treat these problems. 

Treatment of CANVAS Syndrome 

Unfortunately, at the moment, there are no treatments currently available to treat the disease neither to reverse nor to slow down its progress. The treatment is generally symptomatic and may require assistive devices to balance the 3 components . Research is being undertaken to find ways to treat this disorder but here are some measures that can help to alleviate some of the symptoms- 

Managing Dizziness, Blurry Vision and lightheadedness 

  • Staying hydrated 
  • Improving blood pressure by taking appropriate medication 
  • Avoiding medication that increases the symptoms 

Managing balance issues 

  • Practicing vestibular rehabilitation to help the brain compensate for missing signals 
  • Habituation exercises to get used to the symptoms that occur due to head movement 
  • Gaze stability exercises to help focus on objects 
  • Substitution exercises to rely more on the visual system 
  • Balance exercises 

Managing swallowing problems 

Practicing eating techniques and head movement positions to eat food properly through the aid of a speech language pathologist. 

Final Word

As can be seen above, there is still a lot to learn about this rare syndrome. It is always advisable to visit a doctor when these symptoms become visible so that he/she can recommend the best course of action. Hopefully with future advancements in technology, a cure will be seen sooner on the horizon. Don’t let this CANVAS spoil your canvas. 

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