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Cerebellar Ataxia, An Unusual Neurological Manifestation of Coeliac Disease- A Case Study

: Coeliac disease was considered as a gluten sensitive enteropathy but now due to its wide clinical presentation is considered as multisystem autoimmune disorder. Ataxia with peripheral neuropathy is a rare manifestation of gluten sensitivity. The presence of gluten-related immune markers in normal population however complicates the reliable diagnosis of gluten related neurological disorders and clinical improvement on gluten free diet can serve as a diagnostic tool for this disease. We report a case of sporadic progressive cerebellar ataxia with peripheral neuropathy with positive anti tissue transglutaminase (anti-tTG) antibodies and subtotal villous atrophy in duodenal biopsy. This case highlights an important diagnostic and therapeutic principle in management of late onset idiopathic ataxia.

On examination, patient was fully conscious and alert. She was moderately anaemic, nonicteric with vitiligo over extremities and around lips. There was diffuse non tender thyromegaly. Her vitals and other systemic examination were normal. Higher mental functions were essentially normal (MMSE-27/30). All the cranial nerves were intact with normal fundus examination and full ocular movements without nystagmus. There was no motor weakness but all the deep tendon reflexes were diminished and planters were bilaterally flexor. Pain & Temperature is lost in gloves and stocking pattern. Vibration is lost distally in both upper and lower limbs with positive Rhomberg's sign. She had bilateral cerebellar signs with in-coordination of finger nose test, impairment of heel shin test, and gait was broad based ataxic type.
In view of a history of progressive cerebellar ataxia and peripheral neuropathy being accompanied by chronic diarrhea and a positive blood test for anti TTG (tissue trans-glutaminase) antibody and positive histopathology report of partial villous atrophy, a diagnosis of gluten sensitive disorder was confirmed. The patient was advised to follow a strict gluten free diet, following which her loose motions abated within a few days and was given some supportive management like iron and vitamin supplementation including vitamin E and she was discharged home with the advice to remain compliant with her diet plan and regular follow up.

Discussion:
Coeliac disease (CD) is a life-long gluten-sensitive autoimmune disease of the small intestine affecting genetically susceptible individuals worldwide and is seen more commonly in association with HLA DQ2 and DQ8 positivity 1 . CD individuals may present gastrointestinal symptoms, extraintestinal symptoms or no signs or symptoms. The classical symptoms include gastrointestinal-related symptoms such as diarrhea, steatorrhea and weight loss due to malabsorption. About 50% of CD patients present with extraintestinal or atypical symptoms, such as anemia, osteoporosis, dermatitis herpetiformis, neurological problems and dental enamel hypoplasia 2, 3 . The prevalence of CD has been estimated to approximate 0.5%-1% in different parts of the world 4 . A study from north India reported a prevalence of 1 in 310 individuals 5 . Overall prevalence of gluten sensitivity is probably much higher, since Coeliac disease is frequently referred to as 'iceberg disease.
Coeliac disease occurs in approximately 9% of patients with idiopathic cerebellar ataxia. Patients can present with ataxia of limb and/or dysarthria, oculomotor, sensory, or bladder dysfunction 16 .The ataxia occasionally improves after a prolonged gluten-free diet 17 . Ataxic patients also have a higher incidence of gluten sensitivity, defined as the presence of antibodies to gliadin or transglutaminase in the absence of histological evidence of coeliac disease 16 .
In patients with neurologic manifestations, gastrointestinal symptoms are detectable in 10% of the cases only, but biopsy evidence of coeliac disease can be found in upto one-third of the patients. In our case, the patient had a history of chronic diarrhea but the intestinal biopsy also shows partial villous atrophy.
Serological tests used to confirm the diagnosis of gluten sensitivity include: IgA anti gliadin antibody, IgA anti tissue trans-glutaminase (Anti-TTG) and anti endomysial antibodies. Anti TTG and endomysial antibodies are specific for enteropathy but these are often undetectable in patients with neurological manifestations but in our case this test also reveals positive at low titre. MR imaging shows cerebellar atrophy in around 60% of the cases, as similar lesion was found in the present case also (fig-2).
IVIg has had a beneficial effect in patients with sporadic cerebellar ataxia, in the context of GAD antibodies 18 and gluten sensitivity 19 but has not yet been shown to work for the combination of ataxia and coeliac disease. It would be interesting to test plasmapheresis or corticosteroids, as an alternative to costly IVIg, and to ascertain whether all ataxic patients with coeliac disease eventually respond to therapeutic long-term immunosuppression.
The mechanisms for neurological manifestations of coeliac disease remain uncertain. The first proposed mechanism is malabsorption of nutrients exerting neurotrophic and neuroprotective effects 20 but dietary gluten restriction and vitamin supplementation rarely improve the neurological deficits 8,12 . Now an immunological mechanism is given priority in the literature: Anti gliadin Abs have been suggested to be directly or indirectly neurotoxic 15 .

Conclusions:
Neurologic manifestations can also be encountered in cases with gluten sensitivity and which may occur with or without gastrointestinal symptoms and can often be missed if it occurs in isolation. As gluten ataxia is a potentially treatable and reversible disorder, all patients presenting with sporadic, unexplained chronic cerebellar ataxia should be tested for serological evidence of gluten sensitivity. Patients testing positive for antigliadin or anti TG antibodies, should be put on a strict gluten free diet to reduce disability and arrest further disease progression. To the best of our knowledge, we report the first case of elderly lady with cerebellar atxia due to coeliac disease.