Cerebellar‐ataxic Syndrome in Children and Adolescents With Hypothyroidism Under Treatment

Wiebel, J

doi:10.1111/j.1651-2227.1976.tb16537.x

Cited by 20 publications

(10 citation statements)

References 14 publications

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“…7 11 15 16 The progression of cerebellar dysfunction despite normalised thyroid function suggests that in these patients the ataxia may have been due to a mechanism other than the hypothyroid state, as in our six patients.…”

Section: Discussionmentioning

confidence: 59%

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Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis

Selim

Drachman²

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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Acquired cerebellar ataxia has been described with hypothyroidism, and is typically reversible by thyroid hormone replacement therapy. The cerebellar dysfunction has been attributed to metabolic and physiological eVects of the endocrine disorder. In a few patients, however, ataxia has persisted despite thyroid replacement therapy. Other mechanisms may be involved in ataxia associated with thyroid disorders. Objective-To document progressive nonfamilial adult onset cerebellar degeneration (PNACD) occurring in six patients with raised antithyroid antibodies (Hashimoto's/autoimmune thyroiditis), and other autoimmune manifestations, in the absence of hypothyroidism; and to document the independence of the cerebellar disorder from the endocrine dysfunction. Methods-A case study of six patients with PNACD reviewing the clinical course and relation to endocrine and autoimmune status. Results-All six patients were euthyroid when they developed their symptoms; had raised antithyroid antibodies consistent with Hashimoto's autoimmune thyroiditis; and had strong personal or family histories of organ specific autoimmune diatheses. Brain MRI disclosed atrophy of the cerebellar vermis in four patients and olivopontocerebellar atrophy in two. Other possible causes of cerebellar degeneration were excluded. De novo treatment (two patients) or continued treatment (three patients) with L-thyroxine did not modify the progression of the ataxia. Conclusions-Cerebellar degeneration in these patients with raised antithyroid antibodies may be immune mediated. The presence of antithyroid antibodies may signal or cause the autoimmune process producing cerebellar degeneration. "Hashimoto's associated ataxia" seems to represent a recognisable and not uncommon condition; a trial of immunomodulating therapy should be considered in these patients. (J Neurol Neurosurg Psychiatry 2001;71:81-87)

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Section: Discussionmentioning

confidence: 59%

“…11 12 14 In some patients, however, despite thyroid replacement therapy the cerebellar syndrome has persisted and progressed 5-711 15 16 …”

mentioning

confidence: 99%

Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis

Selim

Drachman²

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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“…This aspect has been well described in recent publications (1 1, 21, 27, 34, 37). The clinical picture is characterized by various degrees of cerebellar ataxia and motor coordination disabilities (14,21,33,36), strabismus (17), nystagmus (31), enuresis (3,37), behaviour disorders with problems of restlessness, poor attention, neurotic symptoms, impaired spatial orientation and mathematics learning disability (3,21,24).…”

mentioning

confidence: 99%

Neuropsychological study in treated thyroid dysgenesis

Wolter¹,

Verstraeten²,

Vanderschueren-Lodeweyckx³

et al. 1978

Pediatric Research

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Wolter, R., Noel, P., De Cock, P., Craen, M., Ernould, Ch., Malvaux, P., Verstraeten, F., Simons, J., Mertens, S., Van Broeck, N. and Vanderschueren-Lodeweyckx, M. (Departments of Paediatrics, Universities of Brussels, Leuven, Ghent, Liege and Louvain, Belgium). Neuropsychological study in treated thyroid dysgenesis. Acta Paediatr Scand, Suppl. 277: 41, 1979.-Neuropsychological assessment was carried out in 57 patients aged 3.0 to 17.5 years (mean 8.5) with thyroid dysgenesis under adequate long-term therapy. Starting age of hypothyroidism as estimated by bone age at diagnosis was prenatal in 32 cases, close to birth in 13 cases and postnatal of 1-12 months in 12 cases. Hypothyroidism ofprenatal onset results in severe neuropsychological disorders and mental retardation if not treated early. Only some signs of "minimal brain dysfunction" which compensate with advancing age and normal IQ are found in these children if therapy is started before one month of age. Hypothyroidism starting at birth does not result in mental retardation but neuropsychological disorders are found. They are more frequent if treatment is started after 6 months of age. Children who become hypothyroid between I and 12 months of age are usually not mentally retarded and show minor neuropsychological disorders.

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“…The adult group had more fine motor problems than the children, possibly as a result of different degrees of thyroid deficiency, inadequate initial treatment, test differences or poor test motivation. Cerebellar problems were present in both children and adults similar to the findings of Wiebel [25] in a group of children but unlike those of Hulse [9] who found no evidence of classical cerebellar signs. The adults had fewer gross motor abnormalities and appeared to have compensated for these.…”

Section: Discussionmentioning

confidence: 55%

A comparison between the neurological and intellectual abnormalities in children and adults with congenital hypothyroidism

Frost

Parkin

1986

Eur J Pediatr

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Standardised intellectual, motor and behavioural tests have been undertaken in a group of 73 children and 43 adults with congenital hypothyroidism in a regional study in the north of England. These provide comprehensive data with which to compare the results of studies of children diagnosed by screening and indicate the degree of abnormalities persisting into adult life in those patients diagnosed before screening was introduced. The mean IQ scores of the hypothyroid children and adults were 1-2 standard deviations (SD) below the population means. Longitudinal IQ data indicate persistence of intellectual abnormalities, though there was an increase in IQ score in several patients. The mean motor scores were reduced by 1-2 SD in the children and 1-3 SD in the adults. Behavioural problems and personality difficulties mainly of a neurotic nature also persisted into adult life.

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Cerebellar‐ataxic Syndrome in Children and Adolescents With Hypothyroidism Under Treatment

Cited by 20 publications

References 14 publications

Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis

Ataxia associated with Hashimoto's disease: progressive non-familial adult onset cerebellar degeneration with autoimmune thyroiditis

Neuropsychological study in treated thyroid dysgenesis

A comparison between the neurological and intellectual abnormalities in children and adults with congenital hypothyroidism

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