gluten

Question 1

define gluten sensitivity

Answer 1

a state of heightened immunological responsiveness to ingested gluten in genetically susceptible individuals (marsh 1995)

Question 2

what are some common neurological presentations of gluten sensitivity?

Answer 2

ataxia
peripheral neuropathy
(sensorimotor axonal neuropathy, sensory gangliopathy)
encephalopathy
myoclonic ataxia (hyperexcitable brain)

Question 3

less common neurological manifestations of gluten include:

Answer 3

myopathy,
myelopathy,

epilepsy with or without occipital calcifications

chorea
migratory neuritis
stiff person syndrome

Question 4

what is gluten ataxia

Answer 4

sporadic idiopathic ataxia ( some familial cases described)

where serological markers of sensitivity to gluten are present

Question 5

which antibodies are associated with gluten ataxia?

Answer 5

antigliadin antibodies

Question 6

what is the outcome of treatment with gluten free diet for gluten ataxia

Answer 6

improvement of the ataxia within a year of strict gluten-free diet even in those without enteropathy (hadjivassiliou et al 2003)

Question 7

what is gluten neuropathy

Answer 7

symmetrical sensorimotor length dependant neuropathy

caused by gluten insensitivty

26% of all neuropathies
34% of idiopathic neuropathies

Question 8

what is the effect of treatment with a gluten free diet for gluten neuropathy?

Answer 8

Neurophysiological evidence of improvement of
the neuropathy within a year of adherence to a
strict gluten-free diet

Hadjivassiliou et al 2006

Question 9

what is gluten encephalopathy?

Answer 9

encephalopaphy ( meaning disease, damage or malfunction of the brain) caused by gluten insensitivity

causes episodic often severe and intractable headaches, rarely with focal deficits

headache improves on gluten free diet

white matter changes do not progress but do not resolve either

Question 10

what are the differences between patients with CD presenting with ataxia vs GI symptoms?

Answer 10

patients presenting with neurological dysfunction are diagnosed with CD much later

neurologically more severely affected at time or presentation

diet has stabilising effect but they remain disabled

Question 11

what is non-coeliac gluten sensitivity

Answer 11

patients who benefit (GI or neurological symptoms) from a gluten free diet in the absense of enteropathy

most positive for AGA and may have a low titre for TG2 antibodies

Question 12

what percent of non neurologically gluten sensitive patients have enteropathy on biopsy?

Answer 12

41%

37% no enteropathy but have HLA DQ2

Question 13

what is TG2

Answer 13

transglutimase 2 is a protein that was found to be the autoantigen in CD

Question 14

what percent of those with CD were found to have TG6

Answer 14

40%

73% with positive antigliadan antibodies and ataxia

Question 15

what is TGM6

Answer 15

the gene identified as a novel causative gene of spinocerebellar ataxia

related to TG6 autoantibody