Myoedema with pseudohypertrophic hypothyroid myopathy (Hoffman’s syndrome)

Takkar, Aastha; Sachdeva, Julie; Vojjala, Nikhil; Lal, Vivek

doi:10.1136/practneurol-2020-002898

Cited by 3 publications

(5 citation statements)

References 4 publications

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“…Myoedema is localised contraction of striated muscle produced by direct percussion; and it does not induce electrical activity on electromyogram 7. This phenomena has been reported extensively in cases of hypothyroidism and severe malnutrition 10.…”

Section: Discussionmentioning

confidence: 98%

“…Elevated creatine kinase (CK) and classic myotonic discharges on needle electromyogram help confirm the diagnosis. We describe a bedside neurological sign, myoedema, a mounding phenomenon of the muscle elicited by percussion, that can be helpful in diagnosing acute colchicine myopathy and distinguishing it from GBS and other causes of acute flaccid paralysis 7. This sign is particularly useful in under-resourced regions where blood tests and electrodiagnosis may not be readily accessible.…”

Section: Introductionmentioning

confidence: 99%

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Myoedema: a forgotten sign in acute colchicine myopathy

Le Tri,

Nguyen Vinh,

Dang

et al. 2023

BMJ Case Rep

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Colchicine myopathy typically presents acutely to subacutely with progressive limb weakness. The patients may not be on high doses of colchicine but almost always have acute kidney injury. Dehydration from colchicine-induced diarrhoea is often a precipitating factor. The concomitant neurotoxicity may produce mild sensory complaints. This combination of acute neurological symptoms preceded by diarrhoea prompts the diagnosis of Guillain-Barre syndrome (GBS). The absence of cranial nerve deficits, raised creatine kinase and myotonic discharges on electromyogram may help in differentiating this condition from GBS. We describe a clinical sign, myoedema — a mounding phenomenon of muscle that is elicited by percussion and resolves when the patient recovers. It aids in the bedside diagnosis of acute colchicine myopathy as well as distinguish it from other more common causes of acute flaccid paralysis. We also discuss the possible mechanism of colchicine toxicity and the mounding phenomenon.

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

Myoedema: a forgotten sign in acute colchicine myopathy

Le Tri,

Nguyen Vinh,

Dang

et al. 2023

BMJ Case Rep

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“…These changes lead to the muscles’ pseudohypertrophy and the slowness of movement initiation. Myoedema occurs because tapping the muscle belly leads to calcium ion release, but the reuptake is slow or impaired, leading to persistent muscle contraction that manifests as myoedema 9…”

Section: Discussionmentioning

confidence: 99%

Hoffman syndrome with the classic sign of myoedema and an unusual finding of systolic heart failure

Paul,

Nair,

Krupa

et al. 2023

BMJ Case Rep

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A man in his 20s presented with a 6-year history of calf muscle hypertrophy, proximal muscle weakness and muscle cramps. Along with this, he also had patchy hair loss, facial puffiness and slurring of speech. On examination, he had mild symmetrical proximal muscle weakness, a delayed relaxation phase of his deep tendon reflexes and a rare neurological sign of myoedema. His laboratory investigations revealed elevated thyroid-stimulating hormone levels with high creatine kinase levels, and an echocardiogram showed left ventricular systolic dysfunction. He was diagnosed with Hoffman syndrome, a rare pseudohypertrophic myopathy associated with severe and prolonged hypothyroidism. He had an excellent response to thyroid hormone replacement.

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“…Up to 80% of patients with untreated hypothyroidism develop muscle-related symptoms ranging from myalgia, cramps, muscle stiffness, and fatigue to more severe limb-girdle weakness. 45,46 A minority of adult patients may exhibit muscle pseudohypertrophy. Myopathic pathology includes selective type II fiber atrophy, type I fiber hypertrophy, increased central nuclei, and "corelike" inclusions that may disappear after thyroid replacement therapy.…”

Section: Commentmentioning

confidence: 99%

Section: Thyroid Disordersmentioning

confidence: 99%

Neurologic Complications of Endocrine Disorders

Reda¹

2023

CONTINUUM: Lifelong Learning in Neurology

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OBJECTIVE This article provides an overview of the neurologic complications of the most prevalent endocrine disorders in adults with an emphasis on relevant neurologic symptoms, signs, and laboratory and neuroimaging findings. LATEST DEVELOPMENTS Although the mechanisms of many of the neurologic complications discussed here remain unclear, our understanding of the impacts of diabetes and hypothyroidism on the nervous system and muscle, including complications of rapid correction of chronic hyperglycemia, has advanced in recent years. Recent large studies have not demonstrated a convincing association between subclinical or overt hypothyroidism and cognitive decline. ESSENTIAL POINTS Neurologists must become familiar with the neurologic complications of endocrine disorders not only because they are common and treatable (and often reversible) but also because they may be iatrogenic, as is the case with adrenal insufficiency in the setting of long-term corticosteroid therapy.

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Myoedema with pseudohypertrophic hypothyroid myopathy (Hoffman’s syndrome)

Abstract: Myoedema is an under-recognised neurological sign that can help the bedside diagnosis of metabolic or endocrine myopathies. Myoedema together with pseudo-hypertrophy make a likely diagnosis of hypothyroid myopathy, and its identification may avoid unnecessary investigations.

Cited by 3 publications

References 4 publications

Myoedema: a forgotten sign in acute colchicine myopathy

Myoedema: a forgotten sign in acute colchicine myopathy

Hoffman syndrome with the classic sign of myoedema and an unusual finding of systolic heart failure

Neurologic Complications of Endocrine Disorders

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