Padraic J. Grattan-Smith

Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome

We reviewed the clinical history, electrophysiologic and pathologic findings, and response to therapy of 16 children with chronic inflammatory demyelinating polyneuropathy. The majority presented with lower limb weakness. Sensory loss was uncommon. The illness was monophasic in seven children, relapsing in six, and three had a slowly progressive course. All patients were treated with immunosuppressive agents. In 11, the initial treatment was prednisolone. All had at least a short-term response but five went on to develop a relapsing course. Intravenous immunoglobulin was the initial treatment in four patients. Three responded rapidly, with treatment being stopped after a maximum of 5 months. In resistant chronic inflammatory demyelinating neuropathy, in addition to prednisolone and immunoglobulin, plasma exchange, azathioprine, cyclosporine, methotrexate, cyclophosphamide and pulse methylprednisolone were tried at different times in different patients. On serial neurophysiologic testing slowing of nerve conduction persisted for long periods after clinical recovery. Follow-up was for an average of 10 years. When last seen 14 patients were asymptomatic, two having mild residual deficits. Childhood chronic inflammatory demyelinating neuropathy responds to conventional treatment and generally has a favourable long-term outcome.

The Value of Partial Sleep Deprivation as a Routine Measure in Pediatric Electroencephalography

For more than 50 years it has been known that in patients with epilepsy, sleep markedly increases the diagnostic yield of the electroencephalogram (EEG). Sleep deprivation could have an additional activating role. Many laboratories do not use these methods routinely but reserve them for a second EEG if equivocal or negative findings are present in the initial EEG. We studied a regime of routine partial sleep deprivation without the use of hypnotic agents in 396 children younger than age 17 years who were referred for EEG with a diagnosis of epilepsy or suspected epilepsy. Sleep was achieved for the EEG in 77% (96% in the 1 month to 2 year age group, 78% in the 2 to 8 year age group, and in 64% of those more than 8 years old). In a comparison group of 72 children who had not been sleep-deprived, sleep was achieved in 44% (69% of those less than 2 years old, 27% of those between 2 and 8 years of age, and 33% of those older than 8 years). The differences were highly significant. The regime was well tolerated. Routine partial sleep deprivation is a practical and effective method of obtaining sleep and thus maximizing the information obtained from a single EEG. (J Child Neurol 2000;15:26-29).

Neurofibromatosis involving the lower urinary tract in children. A report of three cases and a review of the literature.

Three children with neurofibromatosis involving the lower urinary tract are reported and their clinical, radiological and pathological findings are described. Lower urinary tract involvement in neurofibromatosis has previously been reported in 17 children, 12 of whom had other stigmata of von Recklinghausens disease. Lower urinary tract involvement may be asymptomatic and can be found incidentally. Every case with neurofibromatosis presenting with what may appear to be even only insignificant urinary symptoms should be thoroughly investigated urologically. Symptoms are usually related to urinary tract obstruction or neurogenic bladder dysfunction due to the involvement of the nerves supplying the bladder. Urinary diversion is the treatment of choice as surgical removal of the extensive tumour is seldom feasible. Following treatment the prognosis for survival appears to be good.

Early indomethacin in patent ductus of the very small premature

ABSTRACT. Thirty‐six babies below 1500g birth weight with patent ductus arteriosus were treated orally with indomethacin, for symptomatic reasons on 30 occasions and prophylactically on 44. Closure was significantly more frequent before 12.5 days of age and when treatment was given with 48 hours of development of clinical signs. Independently of age at treatment it was five times more successful when given within 48 hours of signs developing than when given later. Babies below 1000g birth weight responded as well as those above. Responsiveness did not appear to depend on individual idiosyncrasy. The approach described was aimed to minimise the contribution of a patent ductus to the dangerous recurrent apnoea of the very low birth weight infant.

PULMONARY VALVE ATRESIA WITH INTACT VENTRICULAR SEPTUM A SERIES OF TEN CASES EXAMINED BY SELECTIVE ANGIOCARDIOGRAPHY

Ten cases of pulmonary valve atresia with intact ventricular septum have been studied by selective angiocardiography during the last 3 years. An awareness of the clinical features and chest radiograph appearances of this condition may lead to prompt angiocardiography and perhaps to a happier prognosis than is now the case.

Chapter 20 – Acute Polyneuropathies

The acute polyneuropathies are a group of acquired, generally immune-mediated or infectious disorders causing dysfunction and sometimes degeneration of peripheral nerves, spinal sensory and motor nerve roots, and occasionally the cranial nerves. The most common of these conditions is the Guillain-Barre syndrome (GBS). GBS is the most common cause of acute flaccid paralysis in children. Recent reports have highlighted the variable clinical and neurophysiologic subtypes of pediatric GBS, and emphasized the value of imaging in diagnosis of this disorder. This chapter discusses the heterogeneous presentations of this disorder, the frequency of disease-related complications, and the importance of assiduous clinical care in pediatric GBS. The chapter also covers the natural history, presentation, and treatment of tick paralysis, a rare condition that mainly affects children and that is potentially life-threatening if not appropriately diagnosed and managed.