National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Subacute cerebellar degeneration



I need SCD answers, research trials, a support group and help finding a doctor who is familiar with SCD. I recently learned I have SCD. After an MRI confirmation, I was told it was incurable, progressive, not treatable, and eventually I would need to use a walker before eventually winding up in a wheelchair - and be unable to speak. What else will happen to me? What other coordinated voluntary functions might be affected? Chewing? Bowel/bladder control? Writing?


What is subacute cerebellar degeneration?

Subacute cerebellar degeneration is the breakdown of the area of the brain that controls muscle coordination and balance (the cerebellum).[1] Less commonly, the area connecting the spinal cord to the brain is involved. Subacute cerebellar degeneration may occur in association with a cancer (paraneoplastic cerebellar degeneration) or lack of thiamine (alcoholic or nutritional cerebellar degeneration).[2] Signs and symptoms may include ataxia, speech and swallowing problems, dementia, vision problems, and vertigo.[2]
Last updated: 10/14/2014

What are the signs and symptoms of subacute cerebellar degeneration?

Signs and symptoms of subacute cerebellar degeneration, include ataxia, speech and swallowing problems, dementia (in about half of people with this condition), and difficulty walking. People with subacute cerebellar degeneration due to thiamine deficiency may also experience quick involuntary movements of the eyeball (nystagmus), double-vision, dizziness, and paralysis of the eye muscles.[2]

In paraneoplastic cerebellar degeneration, dizziness, nausea, and vomiting may precede the neurological symptoms.[3] Paraneoplastic cerebellar degeneration may occur in association with Lambert Eaton myasthenic syndrome or encephalomyelitis.[3]
Last updated: 10/14/2014

What causes subacute cerebellar degeneration?

Subacute cerebellar degeneration may occur when the body's immune system attacks healthy tissue, either for unknown reasons or as an abnormal reaction to an underlying cancer. These cases are referred to as paraneoplastic cerebellar degeneration.[2]

Subacute cerebellar degeneration may also occur due to thiamine deficiency. Causes of thiamin deficiency include alcoholism, recurrent vomiting, gastric surgery, and diets poor in this B vitamin.[4] These cases are referred to as alcoholic/nutritional cerebellar degeneration. For further information pertaining to the neurological effects of severe thiamine deficiency, see the following link to the Wernicke-Korsakoff syndrome resource page.
http://rarediseases.info.nih.gov/gard/6843/wernicke-korsakoff-syndrome/Resources/1
Last updated: 10/14/2014

How might subacute cerebellar degeneration be treated?

The aim of treatment of the paraneoplastic type of subacute cerebellar degeneration is to address the underlying cancer if possible. Paraneoplastic cerebellar degeneration may occur in association with any cancer, but the most commonly associated cancers are of the lung, breast, gynecologic cancer, and lymphoma (e.g., Hodgkin disease). Early treatment with immunotherapy (e.g., intravenous immune globulin) may improve symptoms in some patients.[3] 

The alcoholic/nutritional cerebellar degeneration is treated with thiamine and other B vitamin supplementation, normal diet, and avoidance of alcohol.[2]
Last updated: 10/14/2014

What is the long term prognosis for people with subacute cerebellar degeneration?

The long term outlook for people with the paraneoplastic type of subacute cerebellar degeneration is variable. Treatment of the underlying cancer can help stop the progression of the disease, however improvement of existing neurological symptoms is less likely, but can occur. Early treatment (within first three months of symptoms) with immunotherapy may improve long term outlook. There are rare reports of people who have improved without treatment. Advanced age, long history of symptoms, and having anti-Hu or anti-Yo antibodies appear to negatively affect long-term outlook.[3]

The long term outlook for people with the alcoholic/nutritional type of subacute cerebellar degeneration is also variable. Most symptoms (such as ataxia and vision problems) can be reversed if detected and treated promptly. However, improvement in memory function and cognitive skills may be slow and, usually, incomplete. Without treatment, this condition can be disabling and life-threatening.[5][6] 
Last updated: 10/14/2014

How can I find a doctor experienced in treating people with subacute cerebellar degeneration?

We have developed a guide on finding knowledgeable doctors, which we hope you will find helpful. Please visit the following link to view the guide.
http://rarediseases.info.nih.gov/resources/pages/25/how-to-find-a-disease-specialist
Last updated: 10/15/2014

Are there support groups for people with subacute cerebellar degeneration?

You may be interested in learning more about the following online rare disease communities: 

RareConnect has an online community for people with paraneoplastic disorders and their families. The project is a joint collaboration between EURORDIS (European Rare Disease Organization) and NORD (National Organization for Rare Disorders).

RareShare is an online social hub dedicated to patients, families and healthcare professionals affected by rare medical disorders.

Fight Paraneoplastic Syndrome Facebook group is an online group for people affected by paraneoplastic disorders.

You can view a list of organizations that provide general support on our Web site at the following link:
http://rarediseases.info.nih.gov/gard/1193/cerebellar-degeneration-subacute/resources/5 
Last updated: 10/15/2014

How can I learn about research opportunities for people with subacute cerebellar degeneration ?

The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies. Clinical trials in the US are enrolling people with cerebellar degeneration, paraneoplastic syndrome, as well as people with alcoholism. To find these trials, click on "cerebellar degeneration" "paraneoplastic syndrome" and "alcoholism." After you click on a study, review its "eligibility" criteria to determine its appropriateness. Use the study’s contact information to learn more. Check this site often for regular updates.

 

You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling 1-800-411-1222 to speak with a specialist, who can help you determine if you are eligible for any clinical trials.

 

Patient Recruitment and Public Liaison Office
NIH Clinical Center
Bethesda, Maryland 20892-2655
Toll-free: 1-800-411-1222
Fax: 301-480-9793
E-mail: prpl@mail.cc.nih.gov
Web site:  http://clinicalcenter.nih.gov/

 

ClinicalTrials.gov provides a helpful guide for information about participating in a clinical trial. Resources for travel and lodging assistance are listed on the National Center for Advancing Translational Sciences Office of Rare Diseases Research (NCATS-ORDR) website, part of the National Institutes of Health (NIH).

Last updated: 10/15/2014

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. NINDS Cerebellar Degeneration Information Page. National Institute of Neurological Disorders and Stroke (NINDS). June 27, 2008; http://www.ninds.nih.gov/disorders/cerebellar_degeneration/cerebellar_degeneration.htm. Accessed 10/2/2008.
  2. Cerebellar Degeneration, Subacute. NORD. 2007; https://rarediseases.org/rare-diseases/cerebellar-degeneration-subacute/.
  3. Dalmau J, Rosenfeld MR. Paraneoplastic cerebellar degeneration. In: Basow M. UpToDate. Waltham, MA: UpToDate; 2014; Accessed 10/14/2014.
  4. Kumar N. Neurologic presentations of nutritional deficiencies. Neurol Clin. February 2010; 28(1):107-170. https://www.ncbi.nlm.nih.gov/pubmed/19932379.
  5. NINDS Wernicke-Korsakoff Syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). 2016; https://www.ninds.nih.gov/Disorders/All-Disorders/Wernicke-Korsakoff-Syndrome-Information-Page.
  6. Dugdale DC. Wernicke-Korsakoff syndrome. MedlinePlus. February, 2014; http://www.nlm.nih.gov/medlineplus/ency/article/000771.htm.