ABSTRACT
A 2-month-old girl developed motor neuron disease (MND) with autonomic disturbances and died at the age of 5 months. Neuropathological examination revealed Bunina bodies (BBs) in the lower motor neurons of the lumbar spinal cord. The significance of the presence of BBs and the classification of the MND in this child are discussed.
Subject(s)
Autonomic Nervous System Diseases/pathology , Inclusion Bodies/pathology , Motor Neuron Disease/pathology , Central Nervous System/pathology , Female , Humans , Infant , Muscle, Skeletal/innervation , Muscle, Skeletal/pathologyABSTRACT
Gastrointestinal symptoms in myotonic dystrophy are increasingly observed, but major intestinal movement disorders such as intestinal pseudo-obstruction appear to be an infrequent complication. We describe a 13-year-old boy who, after appendectomy, developed intestinal pseudo-obstruction syndrome as the first clinical manifestation of myotonic dystrophy. He developed several similar episodes thereafter, which responded to conservative measures. When a child with myotonic dystrophy presents with an ileus, the diagnosis of intestinal pseudo-obstruction should be considered and therapy should then be conservative.
