Muscle hypertrophy in multifocal motor neuropathy is associated with continuous motor unit activity

O’Leary, Colin; Mann, A C; Lough, Jane; Willison, Hugh J.

doi:10.1002/(sici)1097-4598(199704)20:4<479::aid-mus11>3.3.co;2-w

Cited by 12 publications

(18 citation statements)

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“…Two retrospective studies on the natural course of MMN have been published; both reported a slowly progressive course. 22,32 Step-wise 31,55 and spontaneously remitting 2,19 disease courses have also been described but are less common than the slow progressive course. In a study of 38 patients with MMN, we 32 showed that disease duration and disease severity are related.…”

Section: Natural Coursementioning

confidence: 99%

Multifocal motor neuropathy

Franssen

et al. 1997

Journal of Neurology

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Section: Natural Coursementioning

confidence: 99%

Multifocal motor neuropathy

Franssen

et al. 1997

Journal of Neurology

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“…Localized muscle hypertrophy can be because of increased mechanical stimulus [6,10] but occurs also in nerve, muscle, or skeletal disorders with nerve compression and denervation, e.g. axonal neuropathy, discopathy [19], cervical spondylosis, radiculopathies [11], and in various myopathies [3,20] and neuropathies [8,12,21]. Further underlying causes can be adolescence or medication with testosterone, anabolic steroids, or growth hormones.…”

Section: Discussionmentioning

confidence: 99%

“…A number of neuromuscular disorders can cause muscle hypertrophy [17] or hyperplasia [15]. Laboratory tests accompanied by ultrasound [17], magnetic resonance imaging (MRI) [1,14] and neurophysiologic analyses [12], supplemented by histopathology [20], are used in concert to find the correct diagnosis. Idiopathic hypertrophy of hand muscles is rare.…”

Section: Introductionmentioning

confidence: 99%

Myopathic changes in bilateral hypertrophy of the first dorsal interosseus muscle of the hand

et al. 2009

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A 43-year-old healthy man presented with bilateral hypertrophy of the first dorsal interosseus muscles of both hands. Neurophysiologic and laboratory examinations revealed no pathologic findings. Doubled muscle width was found by ultrasound. Myopathic changes were present in muscle biopsy with varying fiber sizes but without inflammatory features. The clinical impression of massive bilateral symmetric enlargement of the first dorsal interosseus muscles was characterized by subtle, non-specific muscular pathology.

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“…Neuralgic muscle hypertrophy of the limbs has been reported in several types of lower motor neuron disease, such as multifocal motor neuropathy [1], Guillain-Barré syndrome [1], and radiculopathy [3,4], but such hypertrophy involving cranial nerves has not been reported previously to our knowledge. Prior studies suggest that continuous, spontaneous electrical activity of a muscle associated with disease conditions can cause neuralgic hypertrophy [1][2][3][4][5].…”

Section: Dear Sirsmentioning

confidence: 99%

Focal pseudohypertrophy of the left hemi-tongue

Kataoka

Ueno

2011

J Neurol

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A 72 year-old man with a history of diabetes mellitus noticed that his tongue deviated to the left when it was stuck out. He consulted our hospital since this feature persisted for 9 months. The diabetes mellitus was well controlled by voglibose (0.6 mg/day) and gliclazide (80 mg/day), and serum levels of glucose and glycated hemoglobin were 104 mg/dl and 6.1%, respectively. The patient had no signs of neurologic involvement attributable to diabetes mellitus. The left side of the tongue was hypertrophic and the tongue deviated to left (Fig. 1). Left hemi-tongue weakness was evident. There was no fasciculation, and deep tendon reflexes were normal, with no pathological reflexes. The results of other neurological examinations including swallowing, speaking and tasting were negative. Electromyography of the left side of the tongue showed neuropathic motor unit action potentials, polyphasic motor unit potentials, positive sharp wave and fibrillation potentials. The number of motor units per second was decreased. The right side of the tongue showed none of these findings. All other muscles examined (deltoid, biceps, triceps, adductor of thumb, quadriceps, gastrocnemius, and tibialis anterior) showed no evidence of neuropathic or myopathic motor unit action potentials. Magnetic resonance imaging (MRI) of the tongue revealed markedly increased signal intensity in the left side of the tongue on T1-weighted images (Fig. 1a, c) and T2-weighted images (Fig. 1b). The left side of the tongue was enlarged. Contrast-enhanced, T1-weighted MRI images obtained with the use of a bolus of gadoliniumdiethylenetriamine pentaacetic acid showed a circular lesion with ringed enhancement in the root of the left side of the tongue (Fig. 1d, white arrow). Intracranial abnormal signals including the brainstem were not evident on MRI. Anti-IL2 receptor antibody (303 U/ml), antinuclear antibody, and M-protein on immunoelectrophoresis were negative. Hypertrophy of the root of the left side of the tongue was evident on laryngoscopy. A biopsy of the root of the tongue showed lymphocyte infiltration with no malignant cells. Monoclonality was absent on immunohistochemical studies. One year later, short inversion time inversion recovery (STIR) images on repeated MRI showed the decreased signal intensity in the left hemitongue with increased signal intensity on T1-weighted images and T2-weighted images. The size of the circular lesion with ringed enhancement was unchanged. On the basis of the ringed enhancement on MRI and the presence of lymphocyte infiltration with no malignancy on histopathological examination, the lesion was diagnosed to be an inflammatory cystic lesion ''abscess''. On 20 months after consultation to our hospital, the left hemi-tongue weakness was static.These findings could be explained by denervation and reinnervation or by increasing frequencies of motor unit firing from the left hypoglossal nerve and adjacent motor fibers, which were impaired by the cystic lesion in the left side of the tongue. Neuralgic muscle hypertrophy of ...

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Muscle hypertrophy in multifocal motor neuropathy is associated with continuous motor unit activity

Cited by 12 publications

References 0 publications

Multifocal motor neuropathy

Multifocal motor neuropathy

Myopathic changes in bilateral hypertrophy of the first dorsal interosseus muscle of the hand

Focal pseudohypertrophy of the left hemi-tongue

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