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References

McFarland R, Taylor R, Turnbull D. The neurology of mitochondrial DNA disease. Lancet (Neurology). 2002; 1:343-351
Morin C, Mitchell G. Clinical, metabolic and genetic aspects of Cytochrome C Oxidase Deficiency in Saguenay-Lac-Saint-Jean. Am J Hum Genet. 1993; 53:488-496
Ellis Z, Bloomer C. Outpatient anaesthesia for oral surgery in a juvenile with Leigh disease. Anaesth Prog. 2005; 52::70-73
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Management of patients with dental disease and mitochondrial disorders

From Volume 39, Issue 9, November 2012 | Pages 654-655

Authors

Adam A Shakir

Senior House Officer, Department of Oral and Maxillofacial Surgery, Newcastle General Hospital

Articles by Adam A Shakir

Doug M Turnbull

Professor of Neurology, Mitochondrial Research Group, Institute for Ageing and Health, University of Newcastle

Articles by Doug M Turnbull

James R Adams

Consultant Oral and Maxillofacial Surgeon, Department of Oral and Maxillofacial Surgery, Newcastle General Hospital, Newcastle, UK

Articles by James R Adams

Abstract

This case report describes dental disease associated with mitochondrial disease (Leigh's disease) in a young adult. Normal preventive dentistry is difficult in these groups of patients and often leads to management required in secondary care. An awareness of the background pathology is needed when managing these groups of patients. Management of dental pathology in this particular patient often required input from other specialties to ensure a successful outcome.

Clinical Relevance: To raise awareness of the dental pathologies patients with mitochondrial disease may experience as they present to the general dental practitioner, and what treatments may be appropriate.

Article

Leigh's disease is a subacute, necrotizing, encephalomyopathy of the nervous system. Presentation is usually before the age of two, although can occur in juveniles and early adulthood.1

Usual presenting features of the condition are of motor impairment, ie dysphagia, dysphonia, regurgitation, motor retardation and hypotonia.

Leigh's disease is progressive and is often associated with acute relapses associated with infections. The progression involves progressive neurological disability associated with spasticity and movement disorders. Respiratory failure is common and metabolic acidosis is also a feature in some patients.

The underlying defect in many patients with Leigh's syndrome is a defect in mitochondrial function. These defects often involve the mitochondrial respiratory chain and can be due to mutations in either the mitochondrial or nuclear genome. The defect in our patient was a mitochondrial DNA mutation.1

Nutrition is important in patients with Leigh's disease in view of the primary defect of mitochondrial function. Periods of impaired nutrition can lead to episodes of encephalopathy but maintaining nutrition can be problematic owing to increasing difficulty with dysphagia and other motor abnormalities. Many patients have their nutrition maintained by PEG feeding, but this was not possible in our patient because of concerns about safety in maintaining the tube.

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