Signo de Beevor «extendido». Un nuevo signo clínico en miositis con cuerpos de inclusión

Milisenda, José C.; Caballero, Verónica Rico; García, Ana Isabel Bonachera; Tomás, Xavier; Grau, Josep M.

doi:10.1016/j.medcli.2016.06.024

Cited by 3 publications

(1 citation statement)

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“…It is diagnostic for facioscapulohumeral muscular dystrophy, especially in typical cases, with a specificity of 92.9% to 100% and a sensitivity of 53.6% to 95.0% [ 14 – 16 ]. Beevor's sign can also be present in the following conditions: GNE myopathy (observed in nearly 90% of patients) [ 17 ], late-onset Pompe disease [ 16 , 18 ], tubular aggregate myopathy [ 15 ], myotonic dystrophy type 1 [ 16 ], myopathy with TCAP mutations [ 19 ], sporadic inclusion body myositis [ 20 , 21 ], amyotrophic lateral sclerosis [ 22 ], and acute disseminated encephalomyelitis associated with demyelinating polyneuropathy [ 23 ]. Less frequently, Beevor's sign can also be present in an “inverted” form, namely as the inverted Beevor's sign, where the umbilicus moves downward because of upper rectus abdominis weakness.…”

Section: Discussionmentioning

confidence: 99%

Chronic inflammatory demyelinating polyneuropathy with hypoglossal nerve involvement and inverted Beevor’s sign: case report

et al. 2021

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Background Cranial nerve involvement is not commonly encountered in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); this is especially true for involvement of the hypoglossal nerve. Neither Beevor's sign nor its inverted form has previously been described in CIDP. Case presentation A 28-year-old man presented with distal-predominant limb weakness and numbness at the age of 18. A diagnosis of CIDP was made, which was confirmed by electrodiagnostic evidence of demyelination. He responded well to intravenous immunoglobulin and glucocorticoid treatment and achieved remission for 5 years. However, the same symptoms relapsed at the age of 28 and lasted for 10 months. On examination, in addition to limb sensory impairment and muscle weakness, mild bilateral facial paresis, tongue atrophy and fasciculations, and inverted Beevor's sign were also observed. A brief literature review of cranial nerve involvements in CIDP and Beevor's sign or its inverted form were also performed. Conclusions Cranial nerves may be affected in patients with CIDP. Facial palsy is most frequently present, while hypoglossal nerve involvement is rare. Inverted Beevor's sign can appear in CIDP patients.

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