Leigh syndrome, a mitochondrial encephalo(myo)pathy

Outpatient Anesthesia for Oral Surgery in a Juvenile With Leigh Disease

“…2 The disease is transmitted by an autosomal recessive mode of inheritance, possibly maternal, and no genetic basis supports a distinction among groups for differing ages of onset. 2,5 Histopathologic findings substantiate diagnosis of Leigh disease. Magnetic resonance imaging or computed tomography often reveals symmetrical necrotic lesions along the brainstem, diencephalon, and basal ganglion.…”

“…8 The necrosis shows no partiality between gray and white matter, particularly on the brainstem, and the brainstem tegmentum is the structure most frequently damaged. 5,8 Although certain areas are inclined to suffer lesions, the specific topography of the lesions may vary from case to case. 8 These incidents may be influenced by the age of the patient at the onset of the disease.…”

“…5 Other symptoms include motor retardation, hypotonia, mental retardation, seizures, tachycardia, and bradycardia. 5 Respiratory abnormalities are prevalent with Leigh disease.…”

“…5 Other symptoms include motor retardation, hypotonia, mental retardation, seizures, tachycardia, and bradycardia. 5 Respiratory abnormalities are prevalent with Leigh disease. Irregular respiration, apnea, sighing, and stridor are symptoms and often warning signs of impending respiratory failure, the most common cause of death.…”

Anaesthetic management of a patient with Leigh’s syndrome

“…These findings are dynamic and may vary with tilde. 12 Anaesthesia has rarely been reported in patients with Leigh's syndrome, s0L14 In addition, to the best of our knowledge, urinary calculi have never been associated with this syndrome. In previously reported anaesthetics, thiopentone S,n04 and volatile agents s were used, although the potential adverse effects of these in Leigh's syndrome have been described.…”