Coeliac disease and neurological complications
Last reviewed 01/2018
Coeliac disease is associated with various neurological complications - the two most common being cerebellar ataxia and peripheral neuropathy. However the evidence suggests that these neurological complications are not the result of vitamin or trace element deficiencies - but instead seem to be the result of neurotoxic effects of gluten sensitivity and particularly the pathophysiologic role of antigliadin antibodies.
- Hyperexcitable brain and refractory coeliac disease is the proposed term
to encompass a spectrum of immune mediated diseases triggered by gluten ingestion
(1)
- a syndrome associated with Gluten related disorders (GRD)
- whilst coeliac disease (gluten sensitive enteropathy) remains one
of the best characterised GRD, neurological dysfunction is one of
the commonest extraintestinal manifestations with a range of presentations
such as:
- cerebellar ataxia,
- peripheral neuropathy,
- sensory ganglionopathy
- and encephalopathy (headaches and white matter abnormalities)
- neurological manifestations can occur with or without enteropathy
- neurological manifestations occur with normal vitamin levels
e.g. B12 levels
- whilst coeliac disease (gluten sensitive enteropathy) remains one
of the best characterised GRD, neurological dysfunction is one of
the commonest extraintestinal manifestations with a range of presentations
such as:
- a syndrome associated with Gluten related disorders (GRD)
- several neurological disorders have been described in association with coeliac
disease, including epilepsy, myoclonus, ataxia, myelopathy and peripheral
neuropathy (2)
- gluten neuropathy is defined as apparently sporadic idiopathic neuropathy
in the absence of an alternative aetiology and in the presence of serological
evidence of gluten sensitivity
- most common type is symmetrical sensorimotor axonal peripheral neuropathy, but other types of neuropathies have also been reported (4)
- gluten neuropathy is defined as apparently sporadic idiopathic neuropathy
in the absence of an alternative aetiology and in the presence of serological
evidence of gluten sensitivity
- the pathogenesis of neurological complications in coeliac disease remains
unknown. Dietary and immune-mediated mechanisms have been suggested, but conclusive
evidence is lacking
- has been suggested that there is a potential pathophysiologic role of antigliadin antibodies in a neurotoxic autoimmune process (3)
- current evidence suggests that neurological manifestations are immune
mediated. Vitamin and trace element deficiencies rarely play a part,
particularly as most patients with neurological manifestations have no
enteropathy and are thus not prone to malabsorption and vitamin deficiencies
(4)
- most patients who present with neurological manifestations of gluten sensitivity
have no gastrointestinal symptoms. Patients with coeliac disease might not
have gastrointestinal symptoms either. Therefore, gluten sensitivity cannot
be diagnosed on a clinical basis alone
- untreated patients typically have circulating antibodies to gliadin
and to one or more type of transglutaminase
- transglutaminase 6 in patients with neurological manifestations (4)
- untreated patients typically have circulating antibodies to gliadin
and to one or more type of transglutaminase
Reference:
- Hadjivassiliou M et al. Hyperexcitable brain and refractory coeliac disease: a new syndrome.J Neurol Neurosurg Psychiatry. 2013 Nov;84(11):e2.
- Ryan AM et al.Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease. BMJ Case Rep. 2009;2009.
- Hadjivassiliou M, Gibson A, Davies-Jones CA, et al . Does cryptic gluten sensitivity play a part in neurological illness? Lancet 1996;347:369-71
- Hadjivassiliou M et al. Gluten sensitivity: from gut to brain. Lancet Neurol. 2010 Mar;9(3):318-30.