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Consensus Statement: Management of Patients with Cerebellar Ataxia During the COVID-19 Pandemic

Key findings

  • A new COVID-19 Cerebellum Task Force has published a consensus statement that addresses the concerns of patients with cerebellar ataxia during the pandemic
  • Decisions about stopping intravenous immunoglobulin, corticosteroids, mycophenolate and other immunosuppressive therapies should be made with the patient's involvement and be based on age, comorbidities and severity of the immune ataxia
  • A patient who develops symptoms suggestive of COVID-19 should stop immunosuppressive drugs until they have fully recovered
  • If at all possible, initiation of immunotherapy should be delayed, but clinicians should weigh the risks of not starting, particularly in cases of rapidly progressive immune ataxias
  • Neurologists should maintain open dialogue with their patients and fellow practitioners about escalation to intensive care for patients with cerebellar ataxia who develop severe COVID-19

There are no data yet about the specific effects of COVID-19 on patients with cerebellar ataxia (CA), but the pandemic is likely to affect their well-being. CA patients are reliant on caregivers, which increases their risk of SARS-CoV-2 infection, and they are at high risk of complications if they do become infected.

Jeremy D. Schmahmann, MD, director of the Ataxia Unit at Massachusetts General Hospital, and colleagues have formed a COVID-19 Cerebellum Task Force to understand the concerns of patients with CA during the pandemic. They published their initial consensus statement in The Cerebellum.

Discontinuing Immunotherapy

No internationally accepted recommendation addresses whether therapies should be stopped for patients with immune-mediated ataxias, including intravenous immunoglobulin (IVIg), corticosteroids, plasma exchange, mycophenolate and rituximab.

There is no evidence that patients receiving IVIg, corticosteroids or plasma exchange have a higher risk of contracting COVID-19. Decisions should be made with the patient's involvement and be based on age, comorbidities and severity of the immune ataxia.

The task force endorses the U.K. recommendation to continue immunosuppressive treatments—but with patients considered high risk, necessitating shielding, a step beyond physical isolation. As a general rule, a patient who develops symptoms suggestive of COVID-19 should stop immunosuppressive drugs until they have fully recovered.

Starting Immunotherapy

If at all possible, the initiation of immunotherapy should be delayed. However, clinicians should weigh the risks of not starting immunotherapies, particularly in cases of rapidly progressive immune ataxias.

Intensive Care

For patients with CA who become severely ill with COVID-19, decisions about escalation to intensive care are likely to be made by intensivists and/or internists without input from the patient's neurologist.

Because of limited numbers of ICU beds, some institutions are using algorithms for decision-making. Teams may lump all ataxia patients together, despite a large variation in severity and prognosis.

Neurologists should stay alert to this possibility and maintain an open dialogue with their patients and fellow practitioners about the optimal approach to decision-making in these difficult situations.

Telemedicine

The consensus statement endorses telemedicine as efficient, practical and appreciated by CA patients for providing continued access to care providers, including behavioral therapists, psychologists and psychiatrists. Telemedicine can't fully replace in-person encounters because it isn't optimal for assessing tremor, the oscillating nature of movements or rigidity.

Delayed timing of speech during virtual visits and visual limitations of telemedicine platforms might be a disproportionate burden to CA patients because their automatized social models of timing and sequencing may be weaker and/or less flexible. Now that telemedicine is being adopted so broadly, research is needed into how to make it more accessible to CA patients.

Rehabilitation

Expert panels on cerebellar disorders are working to develop online rehabilitation programs. For now, planning is necessary at the institutional level to adapt existing materials. For example, the American Parkinson's Disease Association webpage offers tutorials for home use, and balance can be practiced remotely by following online Tai-Chi or yoga exercises.

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Learn more about the Ataxia Unit in the Department of Neurology

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Jeremy D. Schmahmann, MD, director of the Ataxia Unit at Massachusetts General Hospital, discusses the advances in the rapidly growing field of the clinical cognitive neuroscience of the cerebellum, including the syndrome that bears his name.