ABSTRACT
During a 15-year period, 29 children, under the age of 6 years, with acute Guillain-Barré syndrome were seen at our institution. A review of their charts revealed that pain was a symptom in all patients and was present on admission in 79% of cases. Pain was often the most important symptom and led to misdiagnosis in 20 patients (69%). In 11 of these children, symptoms were present for more than a week before the correct diagnosis was made. The most common pain syndrome was back and lower limb pain, present in 83% of patients. Pediatricians should consider Guillain-Barré syndrome in their differential diagnosis when faced with a child who has lower limb pain and areflexia.
Subject(s)
Pain/etiology , Polyradiculoneuropathy/physiopathology , Child, Preschool , Diagnosis, Differential , Electrophysiology , Female , Humans , Infant , Leg , Male , Polyradiculoneuropathy/cerebrospinal fluid , Reflex, Abnormal , Retrospective StudiesABSTRACT
We report the clinical, electrophysiologic, ophthalmologic, and neuropsychologic features of six patients with hyperammonemia-hyperornithinemia-homocitrullinuria syndrome, an inborn error of ornithine metabolism. Pyramidal signs, decreased vibration sense, bucco-facio-lingual dyspraxia, and learning difficulties or subnormal intelligence were found in the majority. Anomalies of peripheral nerve conduction velocity and of evoked potentials were common, and one patient had markedly abnormal white matter images on cranial magnetic resonance imaging. One patient had retinal depigmentation and chorioretinal thinning. The clinical severity varied greatly among patients; in general, the three younger patients had less neurologic and intellectual impairment than did the three older patients. Only two of our patients have had episodes of symptomatic hyperammonemia. We conclude that hyperammonemia-hyperornithinemia-homocitrullinuria syndrome can be associated with widespread manifestations in the central and peripheral nervous systems. Although the control of hyperammonemia is an essential element in the treatment of these patients, the relationship of hyperammonemia to the chronic neuropsychologic problems of these patients is unclear.