Multiple sclerosis in children under 10 years of age

Neurological Sciences - Despite the consistent amount of information accumulated in recent years on multiple sclerosis (MS) in childhood, many clinicians still view this condition as an exclusively...     

Multiple sclerosis in black children.

Six cases of childhood multiple sclerosis (MS) are presented. All patients (three boys and three girls) were black and came from the Washington, DC, metropolitan area. Age at onset of the disease ranged from 8 to 17 years. Although motor deficits, optic neuritis, and ataxia were similar to those previously reported in pediatric patients, mental symptoms were more prominent and the course more intractable. The pattern of disease was characterized by a high frequency of relapses, with short remission and rapid progression. Within 1 to 7 years, one patient had died, one became severely handicapped, and three were steroid dependent with recurrent relapses. Our findings suggest that MS, though uncommon, exists also in black children. In the cases presented, the clinical course seems to be more malignant than previously reported.     

Multiple sclerosis in children

Multiple sclerosis can develop during childhood, even among children under 10 years of age, and the initial diagnosis can be difficult. A first demyelinating event in children may be an episode of monophasic acute disseminated encephalomyelitis or a first episode of a macrophage activation syndrome, angiitis affecting the central nervous system or MS. The risk of developing MS is lower if: the child is younger than 10 years old; onset is associated with severely altered consciousness; presentation is polysymptomatic; or there are large and poorly limited lesions of the white matter. MS in children probably has a slightly better outcome than MS in adults. Initial treatment mainly relies on methylprednisolone, and there is little information on the results of beta interferon treatment in children with MS.     

Multiple sclerosis in children

Multiple sclerosis (MS) is being increasingly diagnosed in children and adolescents. Clinical and magnetic resonance imaging (MRI) features of MS in the pediatric population are similar to adult-onset disease, with some important distinctions. Case vignettes, recently published clinical definitions, and an approach to disorders considered in the differential diagnoses are provided in this article. Immunomodulatory therapies approved for use in adults with MS are safe and well-tolerated in children, although monitoring of liver function is of particular importance. Finally, this article presents recent research studies performed in an MS population for whom disease onset occurs in unique temporal proximity to the events involved in MS pathogenesis.     

Multiple sclerosis after age 50

When multiple sclerosis (MS) presents late in life (after the age of 50), it frequently gives diagnostic difficulty. In a large MS clinic population (N = 838), 9.4% of the patients had late onset of MS. Slow deterioration of motor function characterized the initial symptoms and subsequent course of this group of older patients. Progression of disability was more rapid than in younger patients. Evoked response studies and CSF electrophoresis were of high diagnostic yield in the older patient group. Reasons for the differing clinical features in the late-onset patients are discussed. We suggest that the age-of-onset criterion for MS be raised to 60 in adequately investigated cases.     

Stroke in children under 16 years of age. Clinical and etiological difference with adults

Objectives - The aim of this study was to re-evaluate the clinical features of stroke in children, their outcome and the place of the different mechanisms, in the light of CT-scan and magnetic resonance imaging. Methods - A 10-year review of the Dijon Childhood Neurology Clinic experience (1985–1995) identified 54 patients with arterial stroke. Diagnosis was established by CT-scan and angiography and by magnetic resonance imaging from 1987. When an hemorrhagic stroke was identified, a cerebral arteriogram and an investigation of the coagulation factors were performed. When an ischemic stroke was identified, the following tests were performed: an ultrasound examination of the cervical arteries, a cerebral arteriogram, a lumbar puncture, an investigation of the coagulation factors and lipid status, a measurement of homocystine in the plasma and the urine, an electrocardiogram, a Holter procedure and a cardiac echography. Results - During the 11 full calendar years of this study, we observed 54 stroke patients. There were 31 cases of ischemic stroke, representing some 57% of the total, as well as 23 cases of hemorrhagic stroke, representing 43% of the total. Of the 31 cases with ischemic stroke, 4 had no known predisposing condition, 6 occurred in children with pre-existing heart disease, 2 had moya-moya disease, 2 had leukemia, 2 had a carotid dissection, 1 had an hemolytic-uremic syndrome, and 14 were observed in patients with other associated conditions, such as infections (7 cases) or slight cranial trauma (7 cases). On CT-scan, a basal ganglia infarction was identified in 14 cases. Among the 23 hemorrhagic strokes, 9 were due to arteriovenous malformations, 2 to arterial aneurysm and 5 to cavernomas. Two cases occurred in hemophilia, 2 in idiopathic thrombocytopenic purpura, and 2 after throat infections. One case had no etiologic factor. Clinical course was marked by a low mortality rate even in hemorrhagic stroke, long-lasting seizures, and hemidystonia. Conclusion - This neuro-imaging survey focused on the real place of hemorrhage and basal ganglia infarctions in children under 16 years of age, compared to adulthood. Follow-up demonstrated good or complete resolution of neurological deficits despite the frequent late hemidystonia and late epilepsy.     

Long-term survival in children under 3 years of age with low-grade astrocytoma

Objective The purpose of this study is to analyze clinical aspects and disease-free survival (DFS) in children less than 3 years of age diagnosed with low-grade astrocytoma. Methods In a period of 24 years (1980–2004), a total of 43 (5.4%) children were registered with these characteristics. Twenty-three patients had pilocytic astrocytoma, 18 diffused, and 2 mixed. Thirty-one (72.1%) children had incomplete surgical tumor resection and 12 (27.9%) had a complete tumor resection. Twelve (27.9%) patients had cranial radiotherapy and 17 (39.5%) received chemotherapy. Overall survival was recorded in 23 (53%). DFS was 50% at 250 months of follow-up for the whole group. DFS for the supratentorial group was 60% at 250 months, whereas, for the infratentorial, it was 22% at 120 months (p = 0.008). Conclusion The only favorable prognostic pattern was the supratentorial presentation. Radiotherapy and chemotherapy did not alter the outcome.     

Neurodevelopmental outcomes in children with HIV infection under 3 years of age

Following the introduction of combination antiretroviral therapy, children vertically infected with the human immunodeficiency virus (HIV-1) living in the developed world are surviving into adult life. This paper reviews the neurodevelopmental outcomes of 62 consecutively-presenting children with HIV-1 infection diagnosed before 3 years of age (32 males, 30 females; median age at presentation 6 mo). Neurological and developmental data are presented with immunological and virological responses to antiretroviral therapy. Fourteen children (22%) had abnormal neurological signs and 25 (40%) demonstrated significant developmental delay on standardized developmental assessments. Children presenting with more severe HIV-1 disease and immune compromise had significantly more abnormal neurological signs and developmental delays than children presenting with milder HIV-1 symptomatology. Immune function, control of HIV-1 viral replication, and growth parameters improved with antiretroviral therapy (median age at last follow-up 7 y 3 mo); however, abnormal neurological signs and significant gross motor difficulties persisted.     

Headaches in children younger than 7 years of age.

Headache in young children is frequently a cause of concern to parents and physicians. We have reviewed our experience with 104 children with onset of headaches prior to 7 years of age seen by age 9 years. Headaches could be classified in more than 90% of cases. The most common headache type in this population referred to a child neurologist was migraine that constituted 75% of the cases. Seventy-two of 78 cases were common migraine. Posttraumatic headaches accounted for an additional 12%. Associated symptoms such as autonomic signs, nausea, and vomiting were common, particularly in the migraine group. Neuroimaging studies when performed did not reveal any significant abnormalities. Other laboratory tests were also generally unhelpful. No child has gone on to develop new neurologic abnormalities or evidence of an intracranial tumor. We conclude that even in young children headaches are generally benign. Even in this population, neuroimaging studies have a very low yield in the absence of other symptoms and findings and are not always indicated.     

Multiple sclerosis diagnostic criteria: three years later

New diagnostic criteria for multiple sclerosis (MS) were developed by an International Panel in 2001 and have had wide distribution and discussion since publication. These provided the first formal incorporation of magnetic resonance imaging (MRI) in a diagnosis work-up for patients suspected of having MS. The so-called McDonald criteria have been studied in retrospective and prospective analyses for sensitivity, specificity and utility, and have been proven to compare favourably or to be an improvement upon prior MS diagnostic criteria. The purpose of the current review is to present and evaluate the key studies that have been performed using the McDonald criteria since 2001 and to set the stage for an upcoming re-evaluation of the new criteria based on data-driven information gathered since their development.     

Amaurosis fugax under the age of 40 years.

Sixteen patients who presented under the age of 40 years with amaurosis fugax have been studied. Follow up from the time of presentation was one to 13 years with a median of 3 years. One patient whose attacks of uniocular visual loss were associated with headache developed a permanent uniocular field defect. None of the other patients has suffered permanent visual loss, or had symptoms of cerebral or myocardial ischaemia. All angiograms were normal and it is suggested that carotid angiography is unnecessary in this age group. Four out of ten patients studied demonstrated evidence of platelet hyperaggregability to low concentrations of arachidonic acid and adenosine diphosphate with spontaneous aggregation. However, in six patients treated with aspirin, including three with previous platelet hyperaggregability, there was no change in the frequency of their attacks implying that the observed platelet abnormalities were not the cause of the amaurosis fugax.     

Impaired motor performance and movement quality in very-low-birthweight children at 6 years of age

Motor performance and movement quality were quantitatively examined (Zurich Neuromotor Assessment: timed motor performances and associated movements) in 87 prospectively enrolled very-low-birthweight (VLBW; <1250g) children (38 males, 49 females; mean birthweight 1016.2g [SD 141.5]:, range 720-1240g; mean gestational age 28.7wks [SD 2], range 25.7-33.4wks) at 6 years of age. All motor tasks were below the reference population: pure motor (median z-score) -0.46; adaptive fine motor (pegboard) -0.99; adaptive gross motor -0.88; static balance -0.48; and associated movements -1.90. All tasks correlated with the degree of neurological abnormalities (p相似文献   

Multiple sclerosis: Indian experience in the last thirty years

A total of 354 cases of multiple sclerosis (MS) were collected from various published reports from India over the last three decades. MS is definitely an uncommon disease in India. Certain clinical characteristics, previously noted individually, have been confirmed to exist in India. When compared to MS in Western countries, the Indian cases show a relatively high incidence of optic nerve involvement (both at the onset and during the course of the disease). Neuromyelitis optical is also seen more frequently. MS may be more common in northern as compared to southern India. In isolated studies HLA-B12 has been reported to be associated with MS in India and positive CSF oligoclonal bands have been seen in only 30% of the cases.