Neurological Complications of Celiac Disease

Source: Tietge, et al, American Journal of Gastroenterology, 92:40, 1997.
Lifeline, Winter 1998, Vol XVI, No 1, pp 1-2

 

Cooke and Smith first described the association of neurological symptoms and celiac sprue in 1966. Neurological complications can exhibit features including peripheral neuropathy, myelopathy, optic myopathy, epilepsy and dementia. Malabsorption of vitamins has been implicated and reaction to toxic or antigenic material because of increased mucosal permeability has been speculated in celiac disease. Onset of neurological complications can occur even when vitamin levels are normal and without coexisting villous atrophy. Thus, supplementation is contraindicated.

Because celiac disease is an autoimmune disorder, neurological symptoms were thought to be of autoimmune origin. However, immunosuppressive therapy did not show benefit. It appears that the onset of neurological complications may follow or precede symptoms and diagnosis of celiac disease. A recently reported study showed antigliadin antibodies in 30 of 53 patients with neurological disease of an unknown cause.

Our conclusions are that severe neurological complications of celiac disease may arise, even when the jejunal biopsy reveals normal results and the patient is asymptomatic. Therefore, we feel that celiac patients should be evaluated for neurological symptoms even in the clinically stable long-term course. In addition, celiac disease needs to be considered when making a differential diagnosis for patients presenting with neurological symptoms of unknown primary cause even in the absence of gastrointestinal symptoms.

Hadjivassiliou, et al proposed [Lancet, 347:369-71, 1996] that antigliadin antibodies may be directly or indirectly neurotoxic or a marker of neurotoxic autoimmune process. Neurological complications of celiac disease presenting to gastroenterologists have been considered rare, but the findings of high incidence of celiac disease in patients with neurological disease of unknown cause, emphasizes that clinicians need to be vigilant for the atypical presentation and complications of celiac disease.

Frisoni, et al conclude that the immune changes in celiac disease are unlikely to play a role in Alzheimer's disease. In control groups none was positive for anti-endomysium antibodies.

Patients with a family history of neurological problems or who are presenting questions related to neurological complications should be proactive in discussing concerns with their physicians. Patients with interests in genetic counselors, physicians and geneticists with expertise in counseling about familial risk may be interested in the directory of genetic counselors available through the cancer society in most states.

On of the concerns arising from the increasing availability of genetic tests is whether people who contemplate such tests understand their appropriate appropriateness and their implications. That base knowledge, the particular need, what information would be obtained and the probable use or value to the self-managed celiac patient becomes primary and important before taking on both the time and financial obligations involved.

REFERENCES:

Cooke, WT and Smith, WT,"Neurological disorders associated with adult coeliac disease." [Brain, December 1966]

Hadjivassiliou, M et al, "Neuromuscular disorder as a presenting feature of coeliac disease." [J Neurol Neurosurg Psychiatry. December 1997];

Hadjivassiliou, M et al, "Does cryptic gluten sensitivity play a part in neurological illness?." [Lancet, 347:369-71, February 1996]

Frisoni, GV et al, "Is celiac disease associated with Alzheimer's disease?" [Acta Neurol Scand., 95(3):147-51,March 1997]

 

CSA Library Series

CSA Library Series is a collection of articles that pertain to celiac disease and dermatitis herpetiformis. Most of these articles have appeared in CSA’s quarterly newsletter, Lifeline, which all CSA members receive. Historic articles included in these resources may or may not include updated notes. Updated information indicated in red type. Articles represent the work of the author.