Myxedema neuropathy and myopathy

Nickel, Stewart N.; Frame, Boy; Bebin, José; Tourtellotte, W. W.; Parker, J. A.; Hughes, B.R.

doi:10.1212/wnl.11.2.125

Cited by 83 publications

(36 citation statements)

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“…Under the electron microscope, aggregates of glycogen granules, mitochondria, lipid droplets, and lamellar bodies were found in Schwann cell cytoplasm. In contrast with Nickel et al (1961), however, they saw only a slight increase in mucoid substances in nerves from their patients. They assumed from their observation that the Schwann cell changes appeared to predominate despite evidence of axis cylinder disease as well.…”

Section: Discussionmentioning

confidence: 64%

Myxoedematous polyneuropathy: a light and electron microscopic study of the peripheral nerve and muscle.

Shirabe¹,

S²,

Terao³

et al. 1975

Journal of Neurology, Neurosurgery & Psychiatry

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SYNOPSIS Histopathological findings of biopsied peripheral nerve and muscle were studied in a case with myxoedematous polyneuropathy. The most striking findings in the sural nerve were segmental demyelination and onion bulb formation with scanty mucinous deposits in addition to marked loss of large myelinated nerve fibres. The peroneus brevis muscle revealed the association of neuropathic and myopathic changes. It is suggested that myxoedematous polyneuropathy might be intrinsic neuropathy due to metabolic disorder of Schwann cells related to hypothyroidism, resulting in segmental demyelination, not merely compressive neuropathy due to mucinous deposits in the peripheral nerves.

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Section: Discussionmentioning

confidence: 64%

Myxoedematous polyneuropathy: a light and electron microscopic study of the peripheral nerve and muscle.

Shirabe¹,

S²,

Terao³

et al. 1975

Journal of Neurology, Neurosurgery & Psychiatry

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“…Perkins and Morgenlander [29] and Bracker and Ralph [30] recorded a high incidence of carpal tunnel syndrome among patients with hypothyroidism and concluded that thyroid function tests were needed in all patients with carpal tunnel syndrome. In 1961, Nickel et al [31] suggested that the mucinous infiltrates found in the peripheral nerves could interfere mechanically with the metabolic exchange of nutrients and catabolic products to and from the neuron resulting in entrapment neuropathy. Some investigators [25] found morphological evidence of primary axonal degeneration.…”

Section: Discussionmentioning

confidence: 99%

Peripheral and Central Nervous System Alterations in Hypothyroidism: Electrophysiological Findings

et al. 2000

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The purpose of this study is to evaluate objectively the functional changes in the nervous system in hypothyroidism by different electrophysiological parameters and to determine the frequencies of these changes in patients with hypothyroidism. We enrolled 23 patients (17–64 years old, mean 38.2) with biochemical evidence of hypothyroidism, with thyroxine less than 4 μg/dl and thyrotropin above 4.5 mU/ml, and 200 age- and sex-matched normal subjects. Detailed clinical examination and electrophysiological measurements included electromyography, motor conduction velocity, visual-evoked potentials (VEPs), brainstem auditory-evoked potentials (BAEPs) and event-related potentials. Determinations of P300, Wechsler Adult Intelligence Scale (IQ) and electroencephalography (EEG) were performed. Of the hypothyroid patients 52% had peripheral nervous system (PNS) involvement. Entrapment neuropathy was the commonest (35%). Axonal neuropathy was recorded in 9% and myopathy was recorded in another 9%. The central nervous system (CNS) was affected in 78% of the cases. Significant prolongations of P100 latency of VEP, latency and interpeak latency of BAEPs of different waves of hypothyroid patients were compared to the control group. 52% had abnormal VEPs and BAEPs above the mean ± 2 SD of the normal control group. Six patients (26%) had prolonged P300 latency while 16 patients had an IQ below 90. Eight patients (35%) had EEG changes. Diffuse slowing of background activity was the commonest. No significant correlation was observed between hormonal levels and the different electrophysiological parameters. Thus, the CNS is more vulnerable to the effect of hypothyroidism than the PNS. Therefore, we suggest performing electrophysiological studies in hypothyroid patients, even in the asymptomatic ones, early in the course of disease in order to detect the nervous system involvement.

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“…Myopathy is a widely recognized complication of hypothyroidism, with an estimated incidence of 30-80% in patients with myxedema [2,6,7]. Generally the severity of the myopathy parallels the duration and degree of the hypothyroidism.…”

mentioning

confidence: 99%

“…Histological changes include type 1 fiber predominance, type 2 fiber atrophy, and increased numbers of central nuclei. Ultrastructural changes include excessive amounts of glycogen, lipid accumulations, abnormal mitochondria, dilated sarcoplasmic reticulum, and focal myofibrillar degeneration [2,6,7]. Magnetic resonance spectroscopy of hypothyroid muscle shows low intracellular pH in resting muscle and delayed glycogen breakdown in exercising muscle [8].…”

mentioning

confidence: 99%