Lhermitte Sign


Article Author:
Dac Teoli
Franklyn Rocha Cabrero


Article Editor:
Sassan Ghassemzadeh


Editors In Chief:
Rodrigo Kuljis
Oleg Chernyshev
Aninda Acharya


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
7/13/2019 3:26:29 PM

Introduction

Demyelinating lesions often lead to paroxysmal attacks of motor and/or sensory phenomena. Frequently occurring and triggered by movement or sensory stimuli, short, stereotypic, events characterize these unique symptoms. Although sometimes bothersome or even frightening, to patients, these events do not prove to be a true advancement of disease conditions (such as in multiple sclerosis) or lead to further injury to the central nervous system (CNS).

When a patient has multiple sclerosis, common disease symptoms include sensory symptoms in the upper and lower limbs. These are sometimes on one side of the face and include visual loss or diplopia, acute or subacute motor weakness, limb ataxia and gait disturbances, vertigo, bladder incontinence or retention, acute transverse myelitis, pain, and also a phenomenon known as Lhermitte's sign.

Lhermitte's sign (sometimes also referred to as Lhermitte's phenomenon or barber chair phenomenon) is a transient neurologic symptom which arises in patients in whom develop it as sequelae of their neurologic condition. Specifically, it is described as an unpleasant electrical shock sensation that travels down the back and into the limbs when a patient flexes their neck forward toward their chest. As previously stated, Lhermitte's sign is categorized as one of the pain syndromes of multiple sclerosis. Of note, it is not to be confused with Uhthoff phenomenon, another often-noted condition of multiple sclerosis, which is characterized by heat sensitivity.[1][2][3]

Etiology

The condition is named after a French neurologist and neuropsychiatrist, Jean Lhermitte. Pierre Marie and Chatelin first described the sign in 1917. In patients that do not experience the phenomenon by flexing their neck alone, it can be further elicited by having the healthcare provider tap on the cervical spine while in the flexed position. The sensation of electricity traveling down the spinal column is believed to originate in the posterior columns.[4][5]

Epidemiology

While the condition is undoubtedly most known for its association with patients who have multiple sclerosis, there is a broad range of medical ailments and expected causes which also lead to its occurrence. Some of these include tumors, trauma, cervical disc herniation, spondylosis, myelopathy, vitamin B12, transverse myelitis, Behcet's disease and Arnold-Chiari malformation (or any other condition leading to spinal cord compression in the cervical region). The phenomenon may also appear in cancer patients, during or after high-dose chemotherapy or irradiation of the cervical spine. It is also occasionally reported as a facet of a discontinuation syndrome related to certain medications. Psychotropic medications such as SSRIs and SNRIs, specifically paroxetine and venlafaxine have been shown to have an association. When being on these medications for some length of time, and then suddenly halting to drastically reducing dosages, some patients enforce experiencing symptoms similar to Lhermitte's sign. In dentistry, there have been studies which found Lhermitte's sign associated with nitrous oxide abusers (believed to be tied back to depleting vitamin B12).

There are no available statistics regarding the incidence or prevalence of Lhermitte's sign in today's global population. However, there have been studies which found that, at least regarding the disease entity of multiple sclerosis, 16% of patients reported experiencing the symptom.[6][7]

Pathophysiology

Lhermitte's sign is described as a transient sensory symptom. To best understand its underlying pathophysiology (which is theoretical in nature) the sensation that is experienced by patients should be first reviewed. Lhermitte's sign is often explained as an almost painful electric shock radiating down the spinal column, and sometimes into the limbs, upon an individual flexing their neck.  While it is most frequently encountered in multiple sclerosis, as previously noted, the symptom can arise in many other conditions of the cervical cord. These conditions including various tumors, vitamin deficiencies, cervical disc herniation, Behçet's disease, postradiation myelopathy, and following trauma. One thing that all of these conditions have in common when associated with the presence of Lhermitte's sign is that the patient is believed to have a lesion of the spinal cord in the cervical region or lower brainstem. These lesions are often compressive in nature, typically of the dorsal columns or the caudal medulla. Of course, demyelination can also play a part, in likely causing an ephaptic transmission of nerve impulses at sites of disease activity.[8]

History and Physical

Neck movements, tiredness, stress, and heat can trigger Lhermitte’s sign. Patients often describe Lhermitte's like an electric shock of pain that runs from the head down to the back, and through the arms and legs. It often happens when they bend their head down and touch their chin to their chest.[4]

Evaluation

There are no laboratory, radiological, or other tests to assess or manage Lhermitte's sign. It is a physical exam finding.[4]

Treatment / Management

If the discomfort is severe, carbamazepine or gabapentin may be beneficial for some patients.

Regarding SSRI withdrawal symptoms, fluoxetine, given the extended length of its half-life, can be given at a single small dosage, and as a result, avoid Lhermitte's sign and other similar symptoms.[9]

Differential Diagnosis

The differential diagnosis of Lhermitte symptom in cancer patients includes tumor progression causing spinal cord compression, as well as other treatment complications. Other causes of spinal cord disease that can result in Lhermitte symptom include demyelinating diseases, vitamin B12 deficiency, and structural abnormalities of the spinal canal.[9][10]

Radiation Oncology

Lhermitte's sign has been noted as a potential side effect of radiation oncology therapies, specifically as an early delayed radiation injury. These often occur within 4 months of radiation therapy.[11][10]

Medical Oncology

Cisplatin or docetaxel neurotoxicity has been tied to Lhermitte's sign.[10]

Deterrence and Patient Education

No active intervention is required by healthcare providers beyond an explanation and reassurance; the syndrome usually resolves spontaneously over a period of months to a year.[12]

Pearls and Other Issues

  • Lhermitte's sign generally occurs with pathologies involving the cervical spinal cord but is not specific to etiology.
  • It often occurs in patients with multiple sclerosis, cervical spondylotic myelopathy, chemotherapy, radiation myelopathy, and B12 deficiency, among others.
  • Increased spinal cord metabolic activity and positive positron emission tomography (PET) imaging have been described in association with Lhermitte's sign.
  • The incidence and prevalence of Lhermitte symptom are difficult to estimate. 
  • A patient can experience Lhermitte's sign intermittently. It does not have to always happen with the same degree of neck flexion. It may be infrequent or occur with slight to dramatic movement of the head or neck.
  • Delayed onset Lhermitte’s sign has been reported following head and/or neck trauma. This occurs several months following injury, without associated neurological symptoms or pain, and typically resolving within 12 months.
  • As a misnomer, Lhermitte's "sign" is not truly a sign at all, at least in the traditional sense of medical terminology. Rather, it is a symptom.[13][14][12][14]

Enhancing Healthcare Team Outcomes

Multiple sclerosis is a chronic disease which can sometimes follow an unpredictable trajectory. It is best to enhance patient outcomes by integrating a team-based approach, including nurses, physician assistants, and physicians should be used in managing patients who live with multiple sclerosis. Consider speaking to the patient about implementing new physicians and services to their overall care, such a palliative medicine, as such resources can offer a new range of support to the family and the patient. [Level V]


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Lhermitte Sign - Questions

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A 35-year-old female with multiple sclerosis has clonus of her lower extremities and bladder spasticity. What symptom is she likely to report if her neck is flexed briskly?



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During the physical exam of a patient, you perform the maneuver shown in the image and the patient complains of an electric shock like sensation in her back. Discontinuation of which class of drugs is associated with this finding?

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    Image courtesy S Bhimji MD
Attributed To: Image courtesy S Bhimji MD



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What medication is required to treat Lhermitte sign?



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Given the most likely etiology, which study is most appropriate to assess a patient who presents with new-onset Lhermitte sign?



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Lhermitte sign is described as which of the following types of sensations?



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A 36-year-old female presents to the clinic for a scheduled appointment. She has a history of depression exacerbated six months ago with the death of her father, as well as a recent severe purulent cellulitis which required a brief hospitalization for intravenous antibiotics. The patient takes venlafaxine on an outpatient-basis but did not mention it to the clinicians treating her cellulitis. Her recovery was uneventful. Today, in the clinic, the patient requests a trial of stopping venlafaxine. The patient mentions that when her neighbor stopped taking this medication, she had terrible sensations of electricity coursing down her spine. In order to avoid this phenomenon, the physician wishes to prescribe a single dose of the appropriate medication as a bridge. Which of the following is the strongest contraindication to prescribing the appropriate medication to avoid Lhermitte sign in this patient?



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Lhermitte Sign - References

References

The Relationship Between Preoperative Clinical Presentation and Quantitative Magnetic Resonance Imaging Features in Patients With Degenerative Cervical Myelopathy., Nouri A,Tetreault L,Dalzell K,Zamorano JJ,Fehlings MG,, Neurosurgery, 2017 Jan 1     [PubMed]
Lhermitte Sign as a Presenting Symptom of Thoracic Spinal Pathology: A Case Study., Hills A,Al-Hakim M,, Case reports in neurological medicine, 2015     [PubMed]
Lhermitte's Sign: The Current Status., Khare S,Seth D,, Annals of Indian Academy of Neurology, 2015 Apr-Jun     [PubMed]
Multiple sclerosis: a primary care perspective., Saguil A,Kane S,Farnell E,, American family physician, 2014 Nov 1     [PubMed]
Ependymal cyst presenting with lhermitte sign., Franceschini PR,Worm PV,, Global spine journal, 2014 Jun     [PubMed]
Multifocal central nervous system demyelination and Lhermitte's phenomenon secondary to combination chemotherapy for chronic lymphocytic leukaemia., Hafner J,Kumar K,Mulligan S,Ng K,, Journal of the neurological sciences, 2014 Mar 15     [PubMed]
Unusual symptoms and syndromes in multiple sclerosis., Rae-Grant AD,, Continuum (Minneapolis, Minn.), 2013 Aug     [PubMed]
Physiopathology of symptoms and signs in multiple sclerosis., Sá MJ,, Arquivos de neuro-psiquiatria, 2012 Sep     [PubMed]
Utilization behavior: clinical manifestations and neurological mechanisms., Archibald SJ,Mateer CA,Kerns KA,, Neuropsychology review, 2001 Sep     [PubMed]
[Lhermitte's sign in three oncological patients]., Porta-Etessam J,Martínez-Salio A,Berbel A,Balsalobre-Aznar J,Esteban J,Benito-León J,Ruiz J,, Revista de neurologia, 2000 Apr 1-15     [PubMed]
Lhermitte's sign., Pearce JM,, Journal of neurology, neurosurgery, and psychiatry, 1994 Jul     [PubMed]
Lhermitte's sign. From observation to eponym., Gutrecht JA,, Archives of neurology, 1989 May     [PubMed]
Neuropathy following abuse of nitrous oxide., Layzer RB,Fishman RA,Schafer JA,, Neurology, 1978 May     [PubMed]
Lhermitte sign following head injury., Anderson FH,Lehrich JR,, Archives of neurology, 1973 Dec     [PubMed]
Ekbom K, Carbamazepine: A Symptomatic Treatment of the Paresthesiae Associated with Lhermitte's Sign. Annals of Indian Academy of Neurology. 2017 Apr-Jun;     [PubMed]
Sandyk R,Dann LC, Resolution of Lhermitte's sign in multiple sclerosis by treatment with weak electromagnetic fields. The International journal of neuroscience. 1995 Apr;     [PubMed]
Reeves RR,Pinkofsky HB, Lhermitte's sign in paroxetine withdrawal. Journal of clinical psychopharmacology. 1996 Oct;     [PubMed]
Yakovlev AE,Parmentier AM, Resolution of Lhermitte's sign with spinal cord stimulation. Pain medicine (Malden, Mass.). 2014 Jan;     [PubMed]

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