Pediatric acute flaccid myelitis: Evaluation of diagnostic criteria and differentiation from other causes of acute flaccid paralysis.

BACKGROUND: Acute flaccid paralysis (AFP) is characterized by rapidly progressive limb weakness with low muscle tone. It has a broad differential diagnosis, which includes acute flaccid myelitis (AFM), a rare polio-like condition that mainly affects young children. Differentiation between AFM and other causes of AFP may be difficult, particularly at onset of disease. Here, we evaluate the diagnostic criteria for AFM and compare AFM to other causes of acute weakness in children, aiming to identify differentiating clinical and diagnostic features. METHODS: The diagnostic criteria for AFM were applied to a cohort of children with acute onset of limb weakness. An initial classification based on positive diagnostic criteria was compared to the final classification, based on application of features suggestive for an alternative diagnosis and discussion with expert neurologists. Cases classified as definite, probable, or possible AFM or uncertain, were compared to cases with an alternative diagnosis. RESULTS: Of 141 patients, seven out of nine patients initially classified as definite AFM, retained this label after further classification. For probable AFM, this was 3/11, for possible AFM 3/14 and for uncertain 11/43. Patients initially classified as probable or possible AFM were most commonly diagnosed with transverse myelitis (16/25). If the initial classification was uncertain, Guillain-Barré syndrome was the most common diagnosis (31/43). Clinical and diagnostic features not included in the diagnostic criteria, were often used for the final classification. CONCLUSION: The current diagnostic criteria for AFM usually perform well, but additional features are sometimes required to distinguish AFM from other conditions.

Walking and periventricular leukomalacia: locomotor characteristics and brain imaging (MRI).

The aim of the present study was to compare the walking abilities in infants with and without periventricular leukomalacia and to see whether the severity of the brain damage was related to locomotor outcome of the infants at 12 and 18 months. 47 newborns were included in the study based on white matter abnormalities on ultrasound. Magnetic resonance imaging (MRI) recordings during the neonatal period were used to identify and quantify the location and severity of the brain lesions. Locomotor outcome was assessed in terms of disability at 12 and 18 months. The quality of walking, including global and segmental gait parameters, was measured for the infants who could walk independently at 18 months and compared to a group of healthy control infants. The number of children who could walk was related to the extent of white matter abnormalities seen on the neonatal MRI, but the quality of walking was not.

Pediatric median neuropathy due to pruritus in Alagille syndrome.

Median entrapment neuropathy or carpal tunnel syndrome is uncommon in children. The majority of cases are related to genetic conditions which result in skeletal dysplasia or altered connective tissue characteristics, direct injury to the median nerve caused by intensive sports or trauma, or hereditary neuropathy with liability to pressure palsies. This report describes a 10-year-old patient with Alagille syndrome who presented with poor fine motor skills because of an entrapment neuropathy of the median nerve at the wrist. This condition was probably caused by intermittent external compression at the wrists due to years of rubbing both wrists and hands to relieve pruritus. To our knowledge, median neuropathy has never been associated with Alagille syndrome, although severe pruritus is considered a major symptom and many patients exhibit widespread scratching and rubbing.

Secondary parkinsonism in childhood: A rare complication after radiotherapy.

Secondary parkinsonism is uncommon in children and exceedingly rare after cranial radiotherapy. This report describes a 14-year-old female who presented with growth retardation as a result of a craniopharyngioma, which was partially resected. A secondary hydrocephalus responded well to shunting. She gradually developed a severe hypokinetic-rigid syndrome 6 months after radiotherapy (54 Gray in 30 daily fractions of 1.8 Gray). In addition, her vigilance decreased. Magnetic resonance imaging revealed increased signal intensity on T2-weighted images in the globus pallidus bilaterally. Nuclear scans indicated only a marginal striatal dopaminergic deficit and revealed decreased metabolism in the thalamus bilaterally. Treatment with dopamine agonists resulted in minor improvement in motor function. Magnetic resonance imaging investigations 3 months later disclosed a decrease of signal intensity changes of the globus pallidus. Gradually, bradykinesia diminished slightly and vigilance increased little. In conclusion, secondary and partially reversible parkinsonism can occur in children after radiotherapy. We suggest that focal encephalopathy resulting from postradiation edema secondary to microangiopathy led to dysfunction of the globus pallidus and thalamus.

Muscle power development in preterm infants with periventricular flaring or leukomalacia in relation to outcome at 18 months.

Periventricular flaring (PVF) or periventricular leukomalacia (PVL) was diagnosed by brain ultrasound during the neonatal period in 44 infants (34 males, 10 females; mean gestational age 31 weeks 2 days, SD 2 weeks 1 day) admitted between 1995 and 1997. The infants were divided into three groups according to the severity of their condition. At 0, 3, and 6 months' corrected age an age-adequate neurological examination with special emphasis on the relation between active and passive muscle power was performed and symmetry between right and left sides was assessed. Results for the whole body, as well as for the shoulders, trunk, and legs were classified as optimal, suspect, or abnormal. Motor outcome at 18 months' corrected age was graded in the same way. An overall optimal muscle power regulation was found in one infant at 0, two at 3, and one at 6 months. Suspect outcome was found at all ages in the three groups. At 0 months muscle power regulation did not differ between the three groups. At 3 and 6 months overall poor muscle power, primarily caused by poor muscle power regulation in the shoulders and trunk, was found in infants with PVL grades III or IV. At 18 months' corrected age 24 infants showed no neurological impairment, eight infants had minor impairment, and 12 infants had severe impairment, including all 10 infants categorized as having PVL grades II or IV. The best predictors of impairment at 18 months were the combined results of muscle power in the shoulders and trunk at 3 months with those of the shoulders at 6 months.

Spontaneous leg movements in infants with and without periventricular leukomalacia: effects of unilateral weighting.

The present study was designed to investigate the contribution of the corticospinal tracts in the regulation and coordination of interlimb couplings and the spatio-temporal organization of kicking movements in young infants. Both healthy infants and those with differing degrees of periventricular leukomalacia (PVL) were subjected to a unilateral weight manipulation at the (corrected) age of 26 weeks. Infants with PVL were grouped according to the amount of damage in the area in which the corticospinal tracts are located as shown by neonatal MRI and confirmed with MRI recordings at 18 months. The main question asked was whether unilateral weighting would reveal different adjustment in infants with and without PVL and whether these differences were related to the severity of the lesions, if present. The major finding was that no differences were evident between groups in adjusting to the weight manipulation with regard to the tightness of interlimb couplings. This finding corroborates the suggestion that corticospinal influences are not directly involved in the regulation of these parameters. Although the same conclusion could be drawn concerning the kinematic details of kicks on the basis of group data, individual analyses revealed that kinematics in a few infants with PVL were markedly affected by the weighting. Thus, combining group with individual analyses may have additional value in the clinical interpretation of the effects of PVL on the neural functions of young infants.