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deviation of both eyes, absent oculocephalic responses, marked bifacial weakness, absent corneal reflexes, and bilateral tongue atrophy, as well as persistence of the previously noted hypertonia. Laboratory evaluation included normal computed tomography and magnetic resonance imaging of the brain. Sequential electroencephalogram examinations were appropriate for age, except for 1 study that showed a less mature pattern than expected. Electromyography and nerve conduction studies were normal. Ophthalmologic examination showed immature retina in zone 2 and exotropia. Brainstemevoked potentials performed between 11 and 12 weeks showed no transmission through the brainstem. Karyotype was normal female (46,XX). Because of the striking impairment in brainstem function, the diagnosis of Mobius syndrome was considered. The infant’s condition did not improve during a 3-month period. The neurologic condition was considered fatal regardless of underlying cause, and, after consultation with the infant’s parents, life support was withdrawn. The infant died at approximately 3 months of age. An unrestricted postmortem examination was performed.

We report the case of a severely hypotonic and weak term newborn who required ventilatory support from the time of birth. Serial neurophysiologic studies were consistent with severe demyelinating polyneuropathy. The infant's condition deteriorated over several weeks despite treatment with corticosteroids and intravenous immunoglobulin (IVIG) for presumed inflammatory demyelinating polyneuropathy. Histopathologic findings in a sural nerve biopsy, however, were similar to those previously reported in congenital hypomyelinating neuropathies. After 12 weeks of hospitalization and after discontinuation of corticosteroids, the patient began to recover and required no further ventilatory support. Remarkable improvement has continued for 18 months. This patient raises questions about the underlying mechanisms of hypomyelinating neuropathies in early infancy.

This chapter covers a varied group of disorders of inherited, postinfectious, ischemic, or undetermined etiology All lead to abnormal development of the nervous system. Some disorders have been selected because of the challenge they pose in diagnosis and management, and others because they illustrate important concepts in neuroembryology or pathophysiology.

Neuromuscular blocking agents are routinely used as an adjunct therapy for critically ill patients. Unlike many neuromuscular blocking agents, vecuronium does not cause significant histamine release, which may lead to bronchoconstriction. Due to a short duration of action and limited accumulation, vecuronium has been widely used. Prolonged ventilatory dependence due to persistent neuromuscular blockade has been reported in patients treated with vecuronium. We report a case of an 8-year-old girl who had a primarily motor axonopathy presenting with weakness after extended vecuronium administration with prolonged course of recovery. This primarily motor neuropathy with axonal features may be a variant of critical illness polyneuropathy, a rarely reported condition in pediatric patients.