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Beevor Sign
Definition/Introduction
The Beevor sign is characterized by abnormal cephalad movement of the umbilicus during truncal flexion while a patient is supine.[1] In individuals with normal muscle function, the rectus abdominis contracts as a single unit without a predominance of the upper over the lower portion or the left over the right side.[2] Consequently, the navel remains stationary during trunk flexion.
Traditionally, the Beevor sign has been associated with weakness or paralysis of the rectus abdominis due to spinal cord lesions between T10 and T12. The sign is named after Dr. Charles Edward Beevor (1854–1908), a neurologist and clinician-scientist who worked at the National Hospital for the Paralysed and Epileptic (Queen Square Hospital) in London from 1883 to 1908. The Beevor sign was first described in his 1898 textbook, Diseases of the Nervous System: A Handbook for Students and Practitioners. Dr. Beevor initially identified the sign in a patient with a spinal cord tumor affecting the T11 and T12 segments and later reported its presence in a patient with myopathy.[3][4]
Less frequently used terms include the "inverted Beevor sign" and the "extended Beevor sign. The "inverted Beevor sign" refers to a downward movement of the umbilicus due to weakness in the upper rectus abdominis. Dr. Beevor also observed this downward displacement in his myopathic patient. The "extended Beevor sign" describes a lateral deviation of the umbilicus in the standing position, resulting from paraspinal muscle asymmetry caused by edema, atrophy, or mild fatty infiltration on one side. This finding reflects asymmetry in the abdominal muscles, with preservation of the transversus abdominis and asymmetric atrophy of the rectus abdominis. The umbilicus shifts toward the stronger rectus muscle.[5]
Performing the Beevor Test
The patient should be in a supine position and accompanied by a chaperone. To elicit the sign, the patient is instructed to either flex the neck or sit up from a recumbent position without using the arms, keeping them crossed over the chest.[6] The examiner observes the movement of the umbilicus. The Beevor sign is considered positive if the umbilicus moves upward by more than 1 cm and negative if it remains in place. A downward movement of more than 1 centimeter indicates an inverted Beevor sign (see Video. Beevor Sign).
Differential Diagnosis
Several publications after Dr. Beevor's time have reported this sign in an array of neurological and neuromuscular disorders. While not pathognomonic, the Beevor sign is considered diagnostic in certain conditions, such as facioscapulohumeral muscular dystrophy (FSHD), particularly when accompanied by other muscular features.
The Beevor sign has been observed in spinal cord lesions and tumors affecting the T10 to T12 segments. Notably, cases presenting acutely with this manifestation have been reported in spinal cord infarction due to vascular lesions below T10.[7]
FSHD is an autosomal dominant muscular dystrophy in which the Beevor sign is regarded as a sine qua non clinical feature.[8] While some studies suggest that the sign has 90% sensitivity and specificity for FSHD, others argue that it is highly specific (over 90%) but less sensitive (54%) and may aid in diagnosis.[9] Additionally, the Beevor sign is more commonly found in typical than atypical cases of FSHD.
Less frequent reports of this sign have been documented in the following diseases:
- Pompe disease or type 2 glycogen storage disease
- Glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase (GNE) myopathy, an autosomal recessive disorder. One study reported this sign in 90% of patients with GNE.[10]
- Tubular aggregate myopathy
- Myotonic dystrophy
- Sporadic inclusion body myositis
- Amyotrophic lateral sclerosis [11]
- Acid maltase deficiency in an adult patient [12]
- Autosomal recessive limb-girdle muscular dystrophy type 2G, a telethoninopathy [13]
- FLAD1-related lipid storage myopathy [14]
- Limb-girdle dysferlinopathy with a heterozygous missense variation in exon 10 of the DYSF gene [15]
- COL6A3 c.6817-2(IVS27)A>G variant associated with Bethlem myopathy [16]
Radiological Findings
In a GNE myopathy study, abdominal magnetic resonance imaging using T2 half-Fourier acquisition single-shot turbo spin-echo (HASTE) sequences revealed sparing of the supraumbilical portion of the rectus abdominis, while the infraumbilical portion exhibited significant atrophy with fatty infiltration. Similarly, an abdominal computed tomography scan showed comparable findings in a patient with sporadic inclusion body myositis.[17]
Issues of Concern
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Issues of Concern
This sign is sensitive to subtle lower rectus abdominis weakness but cannot be elicited in individuals with severe obesity. Additionally, the Beevor sign is difficult to observe in FSHD cases presenting with profound, generalized weakness, impairing a patient's ability to flex the neck or sit upright without assistance.
Clinical Significance
The Beevor sign is a motor sign that allows clinicians to determine the level of a spinal cord lesion in the absence of sensory findings. Additionally, this manifestation can help differentiate organic causes of paraplegia from hysterical paralysis.[18]. The Beevor sign is diagnostic for FSHD.
This feature reflects weakness in the rectus abdominis muscle, typically at or below the level of the umbilicus, whether caused by neurological or muscular disorders. Eliciting the Beevor sign requires a simple technique that may be used by medical students, nurses, and physiotherapists. Furthermore, this finding is crucial during the examination of patients with neurological or neurosurgical conditions. Some authors have used the term "extended Beevor sign" to broaden the range of disorders associated with this manifestation. Additionally, some medical websites describe an oblique umbilical movement as a positive Beevor sign, which occurs in cases of asymmetric weakness of the rectus muscles on either side.
Nursing, Allied Health, and Interprofessional Team Interventions
Measures that should be taken when a patient demonstrates the Beevor sign include the following:
- Assessing for extremity weakness
- Checking for emptying of the bladder. Some patients may require a Foley catheter.
- Providing deep vein thrombosis prophylaxis
- Ensuring that the patient has a pressure sore prevention program in place
- Ensuring that physical therapy provides exercise to strengthen muscles
Taking these precautions ensures comprehensive care for the neurological conditions that may be associated with this clinical feature.
Nursing, Allied Health, and Interprofessional Team Monitoring
Ins and outs must be checked, as urinary retention may occur. Fecal incontinence can be an outcome and must be evaluated periodically. The patient may require skincare to address pressure sores and maintain skin integrity.
Media
(Click Video to Play)
Beevor Sign. This video shows the abnormal upward movement of the umbilicus during truncal flexion in a supine patient.
Contributed by S Munakomi, MD
References
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Eger K, Jordan B, Habermann S, Zierz S. Beevor's sign in facioscapulohumeral muscular dystrophy: an old sign with new implications. Journal of neurology. 2010 Mar:257(3):436-8. doi: 10.1007/s00415-009-5342-9. Epub 2009 Oct 17 [PubMed PMID: 19838767]
Desai JD. Beevor's sign. Annals of Indian Academy of Neurology. 2012 Apr:15(2):94-5. doi: 10.4103/0972-2327.94990. Epub [PubMed PMID: 22566720]
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Milisenda JC, Rico Caballero V, García AI, Tomás X, Grau JM. «Extended» Beevor's sing as a new clinical sign in sporadic inclusion body myositis. Medicina clinica. 2017 Apr 21:148(8):e43. doi: 10.1016/j.medcli.2016.06.024. Epub 2016 Aug 25 [PubMed PMID: 27567333]
Awerbuch GI, Nigro MA, Wishnow R. Beevor's sign and facioscapulohumeral dystrophy. Archives of neurology. 1990 Nov:47(11):1208-9 [PubMed PMID: 2146943]
Leon-Sarmiento FE, Bayona EA, Bayona-Prieto J. A sudden Beevor's sign. Clinical medicine & research. 2007 Jun:5(2):121-2 [PubMed PMID: 17607047]
Level 3 (low-level) evidenceShahrizaila N, Wills AJ. Significance of Beevor's sign in facioscapulohumeral dystrophy and other neuromuscular diseases. Journal of neurology, neurosurgery, and psychiatry. 2005 Jun:76(6):869-70 [PubMed PMID: 15897515]
Level 3 (low-level) evidenceSharma C, Acharya M, Kumawat BL, Nath K. Beevor's sign in facioscapulohumeral muscular dystrophy. BMJ case reports. 2014 May 14:2014():. doi: 10.1136/bcr-2013-203411. Epub 2014 May 14 [PubMed PMID: 24827829]
Level 3 (low-level) evidencePreethish-Kumar V, Pogoryelova O, Polavarapu K, Gayathri N, Seena V, Hudson J, Nishino I, Prasad C, Lochmüller H, Nalini A. Beevor's sign: a potential clinical marker for GNE myopathy. European journal of neurology. 2016 Aug:23(8):e46-8. doi: 10.1111/ene.13041. Epub [PubMed PMID: 27431025]
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Usman S, Khan FS, Subir AH, A Ghafoor FP. Beevor's Sign in Limb Girdle Dysferlinopathy Due to a Novel Mutation. Neurology India. 2023 Sep-Oct:71(5):1061-1062. doi: 10.4103/0028-3886.388111. Epub [PubMed PMID: 37929471]
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