Syndrome de Hoffmann aux urgences

Kaux, Jean-François; Castermans, C.; Delmotte, P.; Bex, Michèle

doi:10.1016/j.annrmp.2007.02.006

Cited by 3 publications

(1 citation statement)

References 7 publications

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“…If Hoffmann's syndrome presents without other manifestations of hypothyroidism, then a high degree of caution is necessary. Kaux et al [ 15 ] reported a patient with Hoffmann's syndrome who presented emergently with severe asthenia and arthralgia without other prominent characteristics of hypothyroidism; in that case, early diagnosis and hormone replacement brought rapid stabilization.…”

Section: Discussionmentioning

confidence: 99%

A Rare Manifestation of Hypothyroid Myopathy: Hoffmann's Syndrome

Lee

Kim

et al. 2015

Endocrinol Metab

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Hypothyroid myopathy is observed frequently and the resolution of the clinical manifestations of myopathy following thyroid hormone replacement is well known. However, a specific subtype of hypothyroid myopathy, Hoffmann's syndrome, characterized by increased muscular mass (pseudohypertrophy), proximal muscle weakness, muscle stiffness and cramps, is rarely reported. Herein, we describe a 34-year-old male who presented with proximal muscle weakness and non-pitting edema of the lower extremities. He initially visited the neurology department where he was suspected of having polymyositis. Additional laboratory evaluation revealed profound autoimmune hypothyroidism and elevated muscle enzymes including creatine kinase. The patient was started on levothyroxine treatment and, subsequently, clinical symptoms and biochemical parameters resolved with the treatment. The present case highlights that hypothyroidism should be considered in the differential diagnosis of musculoskeletal symptoms even in the absence of overt manifestations of hypothyroidism. To our knowledge, this is the first case reported in Korea.

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Section: Discussionmentioning

confidence: 99%

A Rare Manifestation of Hypothyroid Myopathy: Hoffmann's Syndrome

Lee

Kim

et al. 2015

Endocrinol Metab

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Hypothyroid myopathy: A peculiar clinical presentation of thyroid failure. Review of the literature

Sindoni

Rodolico

Pappalardo

et al. 2016

Rev Endocr Metab Disord

103

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Abnormalities in thyroid function are common endocrine disorders that affect 5-10 % of the general population, with hypothyroidism occurring more frequently than hyperthyroidism. Clinical symptoms and signs are often nonspecific, particularly in hypothyroidism. Muscular symptoms (stiffness, myalgias, cramps, easy fatigability) are mentioned by the majority of patients with frank hypothyroidism. Often underestimated is the fact that muscle symptoms may represent the predominant or the only clinical manifestation of hypothyroidism, raising the issue of a differential diagnosis with other causes of myopathy, which sometimes can be difficult. Elevated serum creatine kinase, which not necessarily correlates with the severity of the myopathic symptoms, is certainly suggestive of muscle impairment, though it does not explain the cause. Rare muscular manifestations, associated with hypothyroidism, are rhabdomyolysis, acute compartment syndrome, Hoffman's syndrome and Kocher-Debré-Sémélaigne syndrome. Though the pathogenesis of hypothyroid myopathy is not entirely known, proposed mechanisms include altered glycogenolytic and oxidative metabolism, altered expression of contractile proteins, and neuro-mediated damage. Correlation studies of haplotype, muscle gene expression and protein characterization, could help understanding the pathophysiological mechanisms of this myopathic presentation of hypothyroidism.

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Hoffmann’s syndrome in the differential work-up of myopathic complaints: a case report

Winter,

Heiling,

Eckardt

et al. 2023

J Med Case Reports

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Background Hoffmann’s syndrome is a rare form of hypothyroid myopathy in adults, which is mainly characterized by muscular weakness and muscular pseudohypertrophy. Case presentation We report about a 61-year-old Western European man with myalgia, myxedema and pseudohypertrophy of the calf muscles. Laboratory tests revealed significantly elevated thyroid stimulating hormone (TSH) and creatine kinase (CK). Muscle MRI showed muscular hypertrophy of the lower limbs, but no signs of myositis or myopathy (no gadolinium enhancement, no edema, no fatty degeneration). In addition, electromyography (EMG) detected spontaneous activity. After the beginning of thyroxin-therapy it took six months until the muscle weakness improved and the myalgia regressed. Conclusions Here, we focus on diagnostic routines and typical findings to differentiate Hoffmann’s syndrome from other myopathies. Clinical hallmarks of Hoffmann’s syndrome are pseudohypertrophy and weakness of the calf muscles in combination with elevated CK and elevated TSH. EMG is well suited to detect the involvement of the muscles and muscle MRI helps to differentiate it from other myopathies. Hoffmann’s syndrome is a rare myopathy due to hypothyroidism and plays a role in the differential diagnosis of myopathic complaints even if hypothyroidism has not been detected before.

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Syndrome de Hoffmann aux urgences

Cited by 3 publications

References 7 publications

A Rare Manifestation of Hypothyroid Myopathy: Hoffmann's Syndrome

A Rare Manifestation of Hypothyroid Myopathy: Hoffmann's Syndrome

Hypothyroid myopathy: A peculiar clinical presentation of thyroid failure. Review of the literature

Hoffmann’s syndrome in the differential work-up of myopathic complaints: a case report

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