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Jefferson Adams posted an article in Ataxia, Nerve Disease, Neuropathy, Brain Damage and Celiac DiseaseCeliac.com 01/15/2018 - Cerebellar ataxia with sensory ganglionopathy is a disabling combination of neurological dysfunction that usually occurs as part of certain hereditary ataxias. However, some patients present this combination with no apparent genetic cause. A team of researchers recently set out to if autoimmunity might have a role to play in SG. The research team included Panagiotis Zis, Ptolemaios Georgios Sarrigiannis, Dasappaiah Ganesh Rao, Nigel Hoggard, David Surendran Sanders, and Marios Hadjivassiliou. They are variously affiliated with the Academic Department of Neurosciences, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK; the University of Sheffield, Sheffield, UK; the Department of Neuroradiology, Sheffield Teaching Hospitals NHS Foundaiton Trust, Sheffield, UK; the University of Sheffield, Sheffield, UK; and the Academic Unit of Gastroenterology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK. The team reviewed records of all patients that have been referred to the Sheffield Ataxia Centre who had neurophysiological and imaging data suggestive of SG and cerebellar ataxia respectively. We excluded patients with Friedreich's ataxia, a common cause of this combination. All patients were screened for genetic causes and underwent extensive investigations. They found 40 patients with combined cerebellar ataxia and sensory ganglionopathy. The majority of patients were initially diagnosed with cerebellar dysfunction, and about one-third were initially diagnosed with sensory ganglionopathy. For that one-third, the two diagnoses were made together. The average time between the two diagnoses was 6.5 ± 8.9 years, ranging from 0 up to 44 years. The most common initial symptom was unsteadiness, in 77.5% of patients, followed by patchy sensory loss in 17.5%, and peripheral neuropathic pain in 5%. Nineteen patients had gluten sensitivity, of whom 3 patients had biopsy proven celiac disease. Other abnormal immunological tests were present in another 15 patients. Six patients had malignancy, which was diagnosed within 5 years of the neurological symptoms. Only 3 patients were classified as having a truly idiopathic combination of cerebellar ataxia with sensory ganglionopathy. This study shows that immune pathogenesis plays a significant role in patients with the unusual combination of cerebellar ataxia and sensory ganglionopathy. Source: Cerebellum & Ataxias 20174:20
Jefferson Adams posted an article in Ataxia, Nerve Disease, Neuropathy, Brain Damage and Celiac DiseaseCeliac.com 10/28/2010 - A team of researchers recently found that gluten sensitivity can play a role in triggering a certain type of neurologic dysfunction, called sensory ganglionopathy, and that the condition may respond to a strict gluten-free diet. The team conducted a retrospective observational case study on 409 patients with different types of peripheral neuropathies, including seventeen patients with sensory ganglionopathy and gluten sensitivity. The research team was made up of M. Hadjivassiliou, MD, D.G. Rao, MD, S.B. Wharton, PhD, D.S. Sanders, MD, R.A. Grünewald, DPhil, and A.G.B. Davies-Jones, MD. They are affiliated variously with the Departments of Neurology, Neurophysiology, Neuropathology, and Gastroenterology at Royal Hallamshire Hospital in Sheffield, UK. Neurological issues are common in people with celiac disease and gluten-sensitivity. On eof the most common neurological issues in these people is called peripheral neuropathy. The most common type of neuropathy seen in people with gluten sensitivity is sensorimotor axonal. The team reviewed data on 409 patients with different types of peripheral neuropathies. All of these patients had been followed for a number of years in dedicated gluten sensitivity/neurology and neuropathy clinics. Fifty-three of these patients (13%) showed clinical and neurophysiologic evidence of sensory ganglionopathy. Seventeen of these fifty-three patients (32%) showed positive blood screens for gluten sensitivity. The median age of those with gluten sensitivity was 67 years, with symptom onset starting at 58 years on average. Seven of those with positive blood screen evidence gluten sensitivity showed enteropathy upon biopsy. Fifteen patients went on a gluten-free diet, resulting in stabilization of the neuropathy in eleven of the fifteen. The remaining four patients did not follow the gluten-free diet and their conditions worsened, as did the two patients who declined dietary treatment. Autopsy tissue from three patients showed inflammation in the dorsal root ganglia with degeneration of the posterior columns of the spinal cord. These results led the team to conclude that sensory ganglionopathy can result from gluten sensitivity and may respond positively to a strict gluten-free diet. Source: Neurology 2010;75:1003–1008