Acute transverse myelitis in childhood: nine cases and review of the literature

Acute transverse myelitis (ATM) is a rare disease in childhood and adolescence. It is characterized by paraplegia with or without sensory symptoms and bladder dysfunction, and typically manifests itself over a period of hours to 1 week. This is a report of nine patients who were treated between 1993 and 1996. To exclude treatable conditions, spinal and cranial MRI with and without contrast medium, electrophysiologic tests, and CSF examinations are performed as soon as possible after onset. At present post- or parainfectious inflammation is thought to be the most frequent cause of ATM. Some causes of ATM can be proved only by follow-up examination. The most important differential diagnoses are multiple sclerosis and Guillain-Barré syndrome with its variants. After exclusion of spinal cord compression, and if specific antibiotic treatment is not possible, a 3-day high-dose IV steroid pulse therapy is the most promising treatment. Prognosis is variable and residual symptoms are common. A controlled multicenter study is suggested to assess epidemiology, etiology, and prognosis of ATM.     

Clinical and electrophysiological features of acute flaccid myelitis: A national cohort study

ObjectiveTo summarize the neurophysiological properties of acute flaccid myelitis (AFM) and evaluate limb-based motor outcomes.MethodsNerve conduction studies (NCS) in 49 patients (21 females, 28 males; median age = 52 m) with AFM (median = 7 d after onset; range 1–122 d) were reviewed. Neurophysiological findings, together with treatment and prognosis, and neurophysiology–neuroimaging correlations were analyzed.ResultsThe findings indicated that 64% of paralytic limbs during the acute stage (≤14 d after onset) showed diminished or absent compound muscle action potentials (CMAPs), 79% showed normal motor nerve conduction velocities, 55% showed decreased persistence or absent F-waves, and 95% showed normal sensory nerve conduction velocities. The rate of CMAP abnormalities increased from 41% on days 1–2 to 83% on days 13–14. The reduction in CMAP amplitude was correlated with weaker muscle strength at both the peak neurological deficit and the last follow-up. The baseline limb-based muscle strength at nadir and anterior horn-localized magnetic resonance imaging lesions at recovery stage (>14 d) were strong predictors of outcome at the last follow-up.ConclusionsAFM typically shows neurophysiological features of neuronopathy.SignificanceNCS is probably useful in the diagnosis and evaluation of AFM.     

Acute transverse myelitis in children: clinical course and prognostic factors

The objective of this study was to describe the clinical course of acute transverse myelitis in children, to identify prognostic factors, and to compare our findings with published data Twenty-four children, aged 2 to 14 years and admitted with a diagnosis of acute transverse myelitis, were studied. Clinical features and results of investigations were collected at admission and during the course of the disease. Motor, sphincter, and global outcomes were compared with those in the main adult and pediatric series. During the initial phase, the most common presenting symptoms were pain (88%) and fever (58%). Motor loss preceded sphincter dysfunction in two thirds of patients and became bilateral in half of the patients. When maximal deficit was achieved (plateau), the patients presented a combination of sensory, motor, and sphincter dysfunctions without radicular involvement The motor loss consistently involved the lower limbs but was inconsistent and moderate in the upper limbs. The mean duration of the plateau was 1 week. The recovery phase was characterized by a progressive improvement of all deficits. Sphincter dysfunction improved more slowly than did the other deficits. A full recovery was achieved by 31% of the patients; minimal sequelae were present in 25% and mild to severe sequelae in 44%. An unfavorable outcome was associated with complete paraplegia (P = .03) and/or a time to maximal deficit shorter than 24 hours (P = .005). A favorable outcome was associated with a plateau shorter than 8 days (P = .03), the presence of supraspinal symptoms (P = .01), and a time to independent walking shorter than 1 month (P = .01). The course of acute transverse myelitis in children proceeds through three stages, an initial phase, a plateau, and a recovery phase, each characterized by specific clinical features. The global outcome was favorable in 56% of patients. Several prognostic factors were identified.     

Limbic encephalitis – a review

The clinical features of limbic encephalitis are diverse and early diagnosis of the disorder is frequently difficult. Four patients with limbic encephalitis are described. An antineuronal antibody was identified in three of these patients. Antibodies directed against voltage-gated potassium channels, the N-methyl-D-aspartate receptor and an unidentified neuropil antigen were each found in one patient. The fourth patient had multifocal paraneoplastic encephalitis associated with small cell lung cancer. The clinical and imaging findings associated with these antibodies and the other antineuronal antibodies described in patients with limbic encephalitis are reviewed. An approach to the diagnosis and management of limbic encephalitis is presented.     

Multiple sclerosis – a review

Multiple sclerosis (MS) is the commonest non‐traumatic disabling disease to affect young adults. The incidence of MS is increasing worldwide, together with the socioeconomic impact of the disease. The underlying cause of MS and mechanisms behind this increase remain opaque, although complex gene–environment interactions almost certainly play a significant role. The epidemiology of MS indicates that low serum levels of vitamin D, smoking, childhood obesity and infection with the Epstein–Barr virus are likely to play a role in disease development. Changes in diagnostic methods and criteria mean that people with MS can be diagnosed increasingly early in their disease trajectory. Alongside this, treatments for MS have increased exponentially in number, efficacy and risk. There is now the possibility of a diagnosis of ‘pre‐symptomatic MS’ being made; as a result potentially preventive strategies could be studied. In this comprehensive review, MS epidemiology, potential aetiological factors and pathology are discussed, before moving on to clinical aspects of MS diagnosis and management.