Beevor Sign


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Alwalaa Althagafi


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Mustafa Nadi


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Updated:
7/26/2019 7:33:22 PM

Definition/Introduction

Beevor sign is an abnormal upward (cephalad) umbilicus movement upon truncal flexion while the patient is in a supine position.[1] In normal people, the rectus abdominis muscle contracts as one unit with no predominance of upper half over the lower part or left over the right side.[2] Therefore, normally on trunk flexion, the navel does not move. Traditionally, this sign was an indication for rectus abdominis weakness or paralysis due to spinal cord lesions between T10-12. This sign derives its name from a neurologist and a clinician-scientist Dr. Charles Beevor who served in Queen Square Hospital, London, the UK between 1883-1908. It first appeared in Dr. Beevor’s textbook “Diseases of the nervous system: A Handbook for Students and Practitioners” in 1898.[1] He first described it in a patient with a spinal cord tumor that involved T11 and T12 segments.[3][4] Dr. Beevor also reported his sign in a myopathic patient.[1]

How to perform the test: the patient should be in a supine position. To elicit the sign, the patient is asked either to flex his neck or to sit up from the recumbent position without using the arms (the patients can keep their arms across their chest).[5][6] Once the umbilicus moves upward, it is a positive Beevor sign. It is negative if the umbilicus remains in its position. 

Differential diagnosis: several publications after Dr. Beevor era reported this sign in an array of neurological and neuromuscular disorders. It becomes diagnostic to certain diseases such as facioscapulohumeral muscular dystrophy (FSHD) particularly when accompanied by other muscular features, yet not pathognomonic. Beevor sign can be present in the following conditions:

  1. Spinal cord lesion between T10 and T12 segment, tumors for instance.[1] Noteworthy, reports exist of acute Beevor’s sign with spinal cord infarction due to vascular lesion below T10.[7]
  2. FSHD is autosomal dominant muscle dystrophy. Beevor sign is considered as a "sine qua non" clinical sign of this disease.[6][8] Although some authors reported that this sign is 90% sensitive and specific for FSHD,[9] other researchers believe it is specific (over 90%) but less sensitive (54%)  and can help in diagnosis.[2] Furthermore, it is found more frequently in typical than atypical FSHD.
  3. There are less frequent reports fo this sign in the following diseases:
  1. Pompe disease: type 2 glycogen storage disease.[2][5]
  2. GNE myopathy (autosomal recessive myopathy): this sign was observed in 90% of the patients in one study.[10]
  3. Tubular aggregate myopathy.[3]
  4. Myotonic dystrophy.[10] 
  5. Sporadic inclusion body myositis (IBM).[5]
  6. Amyotrophic lateral sclerosis.[11] 
  7. Acid maltase deficiency in an adult patient.[12]

Radiological findings: in one GNE myopathy study,[10] the author reported the results of abdominal MRI -T2 HASTE sequences. MRI showed sparing of the supra-umbilical portion of rectus abdominis muscle, while the infra-umbilical part demonstrated significant atrophy with fatty infiltration. Moreover, abdominal CT scan showed similar findings in a patient diagnosed with IBM.[3][13] 

Less frequently used terms: inverted Beevor sign where the umbilicus moves downward due to upper rectus abdominis weakness.[2] Beevor also reported downward movement of navel in his myopathic patient.[1]

Issues of Concern

Although this sign is sensitive to covert lower rectus abdominis weakness, it is not possible to elicit it with considerably obese patients.[6] Additionally, it is difficult to observe in cases with severe generalized weakness of FSHD, where the patient is unable to flex the neck or to sit in an upright position without assistance.

Clinical Significance

Beevor sign is a motor sign. Thus, the clinician may determine the spinal cord lesion level without sensory findings.[1] Furthermore, this sign can be used to differentiate between the organic causes of paraplegia from the hysterical paralysis.[14] It is a diagnostic sign for FSHD.

This sign reflects rectus abdominis muscle weakness usually at the level of or below the umbilicus whether due to neurological or muscular disorders.[5][6] [Level III] It is easy to perform by medical students, nurses, physiotherapist as well. Moreover, it is an important sign to examine neurological and neurosurgical patients. Some authors used the term extended Beevor sign to widen the disorders that express Beevor sign.[5] [Level V] Additionally, some medical websites referred to the oblique umbilical movement as a positive Beevor sign. [Level V - expert opinion] In this situation, there is an asymmetrical weakness of recti abdominis muscles on either side.

Nursing Actions and Interventions

  • Assess for weakness in the extremities
  • Check for emptying of the bladder; some patients may require a Foley catheter
  • Provide DVT prophylaxis
  • Ensure that the patient has a pressure sore prevention program in place
  • Ensure physical therapy provides exercise to strengthen muscles

Nursing Monitoring

Check-ins and out: urinary retention may occur

Fecal incontinence can be an outcome and patient may require good skincare


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Beevor Sign - Questions

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A 28-year-old male was involved in a motor vehicle accident. He is stable. When he is asked to sit up, the umbilicus moves up toward the neck area. What type of injury can this indicate?



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A 32-year-old male was involved in a motor vehicle accident where he was the restrained passenger. He immediately felt pain in his midback with an inability to move both of his legs. He remained aware of all the events of the accident and has no associated pain in his neck and upper limb. Spinal precautions were applied at the time of rescue and in the emergency unit, he is hemodynamically stable, GCS 15/15 and has no signs of a head injury. An ECG is normal. A neurological examination of the upper limb shows normal reflexes, power, and tone, and the trunk examination is normal for pinprick and light touch. Abdominal reflexes are difficult to elicit. Lower limb strength is 0/5 bilaterally in all muscle groups. The position sensation is intact, yet he has hypoesthesia from L1 downward bilaterally. He has a weak anal tone with preservation of anal sensation. Once Foley catheter is inserted, it drains 800 milliliters of urine. Analgesia is given to control the pain, and his neck is cleared clinically after performing a portable c-spine x-ray. The provider wants to identify the level of the injury before a CT scan of the spine to direct the imaging at the affected area. When the patient is asked to flex his neck, the umbilicus moves in cephalad direction. What type of injury does the patient most likely have?



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A 13-year-old white female patient presents with a sudden onset of severe lower extremities weakness and urine incontinence. At the onset of weakness, the patient also reports pain that is stabbing in nature, focused around the lower part of the thoracic spine. Past medical history is significant for nocturnal episodes of spontaneous epistaxis happened on several occasions for the last 3 years. No further investigation was carried on in this respect. The patient’s mother reports that the patient’s father who is 45 years old was diagnosed with a disease with multiple lesions in the lungs, gastrointestinal tract (GIT) and liver after investigation for GI bleeding. She added that the patient’s father had a positive genetic test. General physical examination of the patient showed that she is conscious and cooperative. There are dilated vessels under her tongue and some cutaneous dilated blood vessels in different areas of her body. The neurological exam is normal in upper limbs, upper abdominal cutaneous reflexes elicited on both upper quadrants, but they are absent on both lower quadrants of the abdomen, muscle strength is 0/5 in all muscle groups of both lower limbs. Knee and ankle jerks are +3, and Babinski is detected bilaterally, weak anal tone with preservation of some perianal sensation for pinprick. She also has hypoesthesia starting from T11 level downward. What is the other clinical sign that best supports the sensory level finding to confirm the lesion level?



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A 58-year-old male presents with lower limbs muscle cramps for the last 3 years. He has a history of ischemic heart disease, hypertension, myocardial infarction, hypercholesterolemia, type 2 diabetes (well-controlled), and a stroke involving left facio-brachial distribution. The patient is on diet and regular exercise program. He has been on oral diabetic medication with insulin support. He also receives statin drug for hypercholesterolemia along with other medications for his vascular disease. His BMI is 21. He has facial weakness, scapular winging, symmetrical weakness of shoulder abduction, elbow flexion, hip and knee flexion (4 to 4+/5 MRC). His trunk exam showed that he has a protruded lower abdomen, which is disproportionate to his general body built and a positive Beevor sign. Blood work reveals that his CK is 10 folds more than the normal level. His primary care provider stopped his lipid-lowering drug and ordered abdomen MRI for his protruded abdomen. Although the CK came down to the half, it was still abnormal. The MRI findings directed his clinicians for a muscle biopsy to confirm the diagnosis of facioscapulohumeral muscular dystrophy (FSHD). What are the most likely radiological findings that best explain the histological basis of Beevor sign in this patient?



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Beevor Sign - References

References

McCarter SJ,Burkholder DB,Klaas JP,Boes CJ, Charles E. Beevor's lasting contributions to neurology: More than just a sign. Neurology. 2018 Mar 13;     [PubMed]
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;     [PubMed]
Desai JD, Beevor's sign. Annals of Indian Academy of Neurology. 2012 Apr;     [PubMed]
Pearce JM, Beevor's sign. European neurology. 2005;     [PubMed]
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;     [PubMed]
Awerbuch GI,Nigro MA,Wishnow R, Beevor's sign and facioscapulohumeral dystrophy. Archives of neurology. 1990 Nov;     [PubMed]
Leon-Sarmiento FE,Bayona EA,Bayona-Prieto J, A sudden Beevor's sign. Clinical medicine     [PubMed]
Shahrizaila N,Wills AJ, Significance of Beevor's sign in facioscapulohumeral dystrophy and other neuromuscular diseases. Journal of neurology, neurosurgery, and psychiatry. 2005 Jun;     [PubMed]
Sharma C,Acharya M,Kumawat BL,Nath K, Beevor's sign in facioscapulohumeral muscular dystrophy. BMJ case reports. 2014 May 14;     [PubMed]
Preethish-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;     [PubMed]
Pandian JD,Mathuranath PS, Beevor's sign in amyotrophic lateral sclerosis. Neurology India. 1997 Oct-Dec;     [PubMed]
Oya Y,Morita H,Ogawa M,Nonaka I,Tsujino S,Kawai M, [Adult form of acid maltase deficiency presenting with pattern of muscle weakness resembling facioscapulohumeral dystrophy]. Rinsho shinkeigaku = Clinical neurology. 2001 Jul;     [PubMed]
Sugie K,Kumazawa A,Ueno S, Sporadic Inclusion Body Myositis Presenting with Beevor's Sign. Internal medicine (Tokyo, Japan). 2015;     [PubMed]
Tashiro K, Charles Edward Beevor (1854-1908). Journal of neurology. 2001 Jul;     [PubMed]

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