CANVAS syndrome

Rare disorder of balance, nerves and the cerebellum

Rare condition

CANVAS syndrome is a rare, slowly progressive neurological disorder in which both balance organs, the sensory nerves and the cerebellum can be affected. It mainly causes problems with balance, walking and keeping the image stable during movement.

Key points

  • CANVAS stands for Cerebellar Ataxia, Neuropathy and Vestibular Areflexia Syndrome.
  • In CANVAS, both balance organs can have strongly reduced function.
  • The sensory nerves and the cerebellum can also be affected.
  • Balance problems are often most obvious in the dark and on uneven ground.
  • A chronic dry tickly cough can develop years before the balance symptoms.
  • A variation in the RFC1 gene is found in many people with CANVAS.

Symptoms

  • Unsteady or wide-based walking and reduced balance.
  • More balance problems in the dark or on uneven surfaces.
  • Blurred or jerky vision while walking or moving the head, also called oscillopsia.
  • Reduced feeling or tingling in hands and feet.
  • Problems with coordination of the arms and legs and sometimes changed speech.
  • A chronic, often unexplained dry irritating cough.

Causes

  • CANVAS is caused by a disorder of several parts of the nervous system that are important for balance and coordination.
  • In many people with CANVAS, biallelic repeat expansions in the RFC1 gene are found.
  • As a result, the balance organs, sensory nerves and cerebellum can gradually function less well.

Treatments

  • There is currently no treatment that can cure CANVAS.
  • Vestibular rehabilitation can help to improve balance, walking and coping with reduced function of both balance organs.
  • Exercises focus among other things on head and eye movements, balance, walking and making optimal use of other sensory information.
  • Depending on the symptoms, support can be provided by a neurologist, physiotherapist, occupational therapist and speech therapist.