Lhermitte's sign: Review with special emphasis in oncology practice

https://doi.org/10.1016/j.critrevonc.2009.04.009Get rights and content

Abstract

Lhermitte's sign (LS) is characterized by electric shock like sensation, spreading along the spine in a cervico-caudal direction and also into both arms and legs, which is felt upon forward flexion of the neck. It is a myelopathy resulting from damage to sensory axons at the dorsal columns of the cervical or thoracic spinal cord and a well-known clinical sign in neurology practice. Patients with cancer may present with LS due to various causes either related to the tumor itself or to its treatment. Spinal cord tumors, radiotherapy and chemotherapy are possible causes of LS observed in oncology practice. While LS is observed with a frequency of 3.6–13% in large patient groups receiving radiotherapy for head and neck and thoracic malignancies, the true incidence of chemotherapy and spinal cord tumor induced LS is unknown with only few reported cases in the literature. In the present article, various pathologies causing Lhermitte's sign are reviewed with special emphasis on the implications of this sign in oncology practice.

Introduction

Lhermitte's sign (LS) is a rare nonspecific neurologic complication arising from involvement of spinal cord and can be associated with various conditions. It is characterized by an unpleasant sensation or pain in form of electric discharge spreading down the spine and limbs with neck flexion. In fact it is rather a symptom than a sign and it can be stimulated by certain maneuvers like Valsalva, bending of the head, coughing. This sign was first reported by Marie and Chatelin in 1917, during the First World War [1]. They described ‘pins and needles’ type sensations traveling the spine which were induced by forward flexion of the head in soldiers who had suffered cranial trauma [1]. Although first observed and reported by Marie and Chatelin, LS was defined in detail and introduced to the neurology literature by Jean Lhermitte, as a clinical entity like the Babinski's sign [2], [3]. Beside traumatic lesions of the head and spinal cord which were initially identified as the cause of this sign, Lhermitte was the first to report it in a patient with multiple sclerosis (MS) [2], [3]. He emphasized the importance of this sign in the early diagnosis of multiple sclerosis. He contributed to the pathogenesis of this sign and published several papers. He suggested that this symptom was due to the irritation of the spinal cord resulting in axonal demyelination with preservation of axonal continuity at the cervical level of the spinal cord. He also showed the existence of this sign in other diseases that could affect the spinal cord, like pernicious anemia [3].

Besides being a well-known clinical sign in neurology practice, LS is also encountered in oncology practice in association with various oncologic pathologies involving the spinal cord.

Section snippets

Search strategy and selection criteria

Data for this review were identified by searches of PubMed using the search terms “Lhermitte's sign”, “Lhermitte's”, “Lhermitte” “myelopathy” and “neurotoxicity”, and using references from relevant articles. One abstract from Proceedings of American Society of Clinical Oncology published in 1995 was included as it was directly related to the subject. Papers published until the end of 2008 were included.

The study was not funded by any source.

Pathophysiology

The exact pathophysiology of LS is not known. Any event causing damage, irritation or compression of myelinated sensory axons in dorsal columns of the spinal cord at cervical or rarely thoracic level can give rise to this sign. The characteristic electric-like sensation of the sign, which spreads down the spine to the arms and legs, is triggered by the flexion of the neck. How neck flexion causes generation and transmission of electric-like sensory discharges is unclear; but it is presumed that

LS in oncology practice

Although first described as a neurological sign occurring with head and neck traumas [1], [17], [18], [26], [27], [28], [29], multiple sclerosis [12], [23], [24], [25], and thus mainly encountered in neurology and neurosurgery practice, LS later became a well-known neurological sign in oncology practice (Table 1).

Treatment

There is no specific treatment. Corticosteroids may be beneficial [40]. LS appearing in the context of transient radiation myelopathy is not associated with chronic progressive myelitis, but delayed radiation myelopathy which is irreversible and results in paralysis, may be preceded by LS [6]. LS that predates delayed radiation myelopathy is observed later in onset than the usual latency period of the LS observed in transient radiation myelopathy [6], [42].

Patients experiencing LS related with

Discussion

The sensation of a sudden electrical impulse that shoots down the back and into the limbs when the neck is flexed has been known as Lhermitte's sign or Barber's Chair sign. This sign is not specific to any disease and indicates a lesion in the cervical spinal cord. Many different conditions, pathologies and treatments can give rise to this sign, and thus, it may be encountered in different medical disciplines and may be perceived differently in diverse clinical settings. For example, presence

Conflict of interest

None.

Contribution of authors: The study was conducted by a single author, so all the work needed to be done was performed by Cengiz Gemici MD.

Reviewers

Prof. Esat Mahmut Ozsahin, Senior Consultant, Centre Hospitalier Universitaire Vaudois CHUV, Department of Radiation Oncology, Bugnon 46, CH-1011 Lausanne, Switzerland.

Prof. Jean-Charles Soria, Institut Gustave Roussy, Department of Medicine, 39, rue Camille, Desmoulins Villejuif, Cedex 94805, France.

Dr Cengiz Gemici is a radiation oncologist at the Department of Radiation Oncology, Dr Lutfi Kirdar Kartal Education and Research Hospital, Istanbul Turkey. He received his M.D. in 1990 at the University of Marmara, Istanbul and completed his residency at S.S.K Okmeydani Hospital, Department of Radiation Oncology, between 1990 and 1994. He later worked as a resident first at the Department of Radiation Oncology and then at the Department of Medical Oncology, Tenon Hospital, Paris, France,

References (71)

  • J.K. Sul et al.

    Neurologic complications of cancer chemotherapy

    Semin Oncol

    (2006)
  • J.M. Jurado et al.

    ‘Out of blue’ Lhermitte's sign: three cases due to low cumulative doses of oxaliplatin

    Ann Oncol

    (2008)
  • F. Denis et al.

    Late toxicity results of the GORTEC 94-01 randomized trial comparing radiotherapy with concomitant radiochemotherapy for advanced-stage oropharynx carcinoma: comparison of LENT/SOMA, RTOG/EORTC, and NCI-CTC scoring systems

    Int J Radiat Oncol Biol Phys

    (2003)
  • M.R. Rosenfeld et al.

    Neurologic complications of bone marrow, stem cell, and organ transplantation in patients with cancer

    Semin Oncol

    (2006)
  • C.R. Lewanski et al.

    Lhermitte's sign following head and neck radiotherapy

    Clin Oncol (R Coll Radiol)

    (2000)
  • P. Marie et al.

    Note sur certains symptomes vrais amblement d’origine radiculaire chez les blessées du crane

    Rev Neurol

    (1917)
  • J. Lhermitte et al.

    Les douleurs a type de décharge électrique consécutives a la flexion céphalique dans la sclérose en plaques. Un cas de forme sensitive de la sclerose multiple

    Rev Neurol

    (1924)
  • J.A. Gutrecht

    Lhermitte's sign. From observation to eponym

    Arch Neurol

    (1989)
  • O. Esik et al.

    A review on radiogenic Lhermitte's sign

    Pathol Oncol Res

    (2003)
  • W.M. Butler et al.

    Lhermitte's sign in cobalamin (vitamin B12) deficiency

    JAMA

    (1981)
  • D. Martin

    Lhermitte's sign and cobalamin deficiency

    JAMA

    (1982)
  • R. Sandyk et al.

    “Lhermitte's sign” as a presenting symptom of subacute combined degeneration of the cord

    Ann Neurol

    (1983)
  • P.C. Gautier-Smith

    Lhermitte's sign in subacute combined degeneration of the cord

    J Neurol Neurosurg Psychiatry

    (1973)
  • J.A. Gutrecht et al.

    Anatomic-radiologic basis of Lhermitte's sign in multiple sclerosis

    Arch Neurol

    (1993)
  • J. Fritschi et al.

    Spinal MRI supporting myelopathic origin of early symptoms in unsuspected cobalamin deficiency

    Eur Neurol

    (2003)
  • D.H. Miller et al.

    Magnetic resonance imaging in isolated noncompressive spinal cord syndromes

    Ann Neurol

    (1987)
  • T. Imai et al.

    Lhermitte's sign in alcoholic myelopathy without portosytemic shunting: MRI evaluation

    Intern Med

    (2005)
  • S.S. Bassi et al.

    MRI of the spinal cord in myelopathy complicating vitamin B12 deficiency: two additional cases and a review of the literature

    Neuroradiology

    (1999)
  • S.D. Hodges et al.

    Cervical epidural steroid injection with intrinsic spinal cord damage. Two case reports

    Spine

    (1998)
  • V.C. Traynelis et al.

    Magnetic resonance imaging and posttraumatic Lhermitte's sign

    J Spinal Disord

    (1990)
  • H.B. Newton et al.

    Lhermitte's sign as a presenting symptom of primary spinal cord tumor

    J Neurooncol

    (1996)
  • P. Klimo et al.

    Congenital partial aplasia of the posterior arch of the atlas causing myelopathy: case report and review of the literature

    Spine

    (2003)
  • V. Ventafridda et al.

    On the significance of Lhermitte's sign in oncology

    J Neurooncol

    (1991)
  • O. Esik et al.

    Increased metabolic activity in the spinal cord of patients with long-standing Lhermitte's sign

    Strahlenther Onkol

    (2003)
  • R. Kanchandani et al.

    Lhermitte's sign in multiple sclerosis: a clinical survey and review of the literature

    J Neurol Neurosurg Psy

    (1982)
  • Cited by (19)

    • Late toxicities management

      2023, Palliative Radiation Oncology
    • Three-dimensional kinematic stress magnetic resonance image analysis shows promise for detecting altered anatomical relationships of tissues in the cervical spine associated with painful radiculopathy

      2013, Medical Hypotheses
      Citation Excerpt :

      For example, many patients exhibit cervical radiculopathy only as a result of specific neck positioning. The clinician can reproduce this position and the associated symptoms through physical examination including performance of Spurling’s maneuver or production of Lhermitte’s phenomenon [40,41]. Coupling such physical examination maneuvers with an adequate imaging method could help to identify if, and how, the architectural relationship between the hard and soft tissues of the spine can elicit pain.

    • Lhermitte sign after chemo-IMRT of head-and-neck cancer: Incidence, doses, and potential mechanisms

      2012, International Journal of Radiation Oncology Biology Physics
      Citation Excerpt :

      Although LS is not usually associated with a progression to chronic progressive irreversible myelitis, delayed radiation myelopathy causing paralysis may be preceded by LS (4). The incidence of LS in series of patients receiving RT for head-and-neck (HN) or thoracic malignancies has been reported to be between 3.6% and 13% (4–7). All these series used conventional RT, which typically delivers homogeneous dose distributions across the spinal cord.

    View all citing articles on Scopus

    Dr Cengiz Gemici is a radiation oncologist at the Department of Radiation Oncology, Dr Lutfi Kirdar Kartal Education and Research Hospital, Istanbul Turkey. He received his M.D. in 1990 at the University of Marmara, Istanbul and completed his residency at S.S.K Okmeydani Hospital, Department of Radiation Oncology, between 1990 and 1994. He later worked as a resident first at the Department of Radiation Oncology and then at the Department of Medical Oncology, Tenon Hospital, Paris, France, between 1994 and 1998. He obtained his Medical Oncology specialty diploma from University of Paris XI, Paris-Sud Medical Faculty, Paris, France. He is a member of ASTRO and ASCO. Chemotherapy and radiation interactions and treatment with combination chemoradiotherapy in different types of tumors is his main area of interest.

    The study has not been presented elsewhere, either as a whole or in part.

    View full text