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Neurology. 2013 March 5; 80(10): e104–e107.
PMCID: PMC3653210

Clinical Reasoning: A 57-year-old man with jaw spasms

Zoltan Mari, MD,corresponding author

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  1. (1) Commercial entity: Allergan;(2) Commercial entity: Merz;(3) Commercial entity: Abbott.

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  1. (1) NINDS, 5 U10 NS44474-08, PI, 12/1/08 - 11/30/12;(2) NINDS, 2 P50 NS038377-11, Co-I, 9/30/98 - 7/31/14;(3) NINDS, U10NS44474, PI, 2/15/08 - 11/30/12.

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Liana S. Rosenthal, MD,

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  1. Received funding from Safra Foundation to speak at the 2012 Johns Hopkins Visiting Nurse Faculty training in Parkinson disease, received funding from The Cohen Fund for travel to the 16th International Congress of Parkinson's disease and Movement Disorders.

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  1. Funded as an investigator on NIH grant 5KL2RR025006-05 (2011-present).

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Kristin C. Darwin, BS,

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  1. Johns Hopkins School of Medicine, Research Assistant

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Mark Hallett, MD,

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  1. Clinical Neurophysiology*1999 - present Editor-in-Chief, Western Hemisphere2000 - 2003 Editor-in-Chief (sole)2004 - 2007 Honorary Consulting Editor2008 -- presentBrain1994 - 1998 Associate Editor2006 - 2011Acta Neurologica Scandinavica1994 - presentJournal of Clinical Neurophysiology1995 - presentItalian Journal of Neurological Sciences1997 - presentMedical Problems of Performing Artists1997 - presentAnnals of Neurology2000 - 20052007 - presentNeurology and Clinical Neurophysiology2000 - presentThe Cerebellum2000 - presentNeuroRx2002 - presentCurrent Trends in Neurology2003 - presentFaculty of 1000 Biology2003 - presentEuropean Neurology2005 - presentFaculty of 1000 Medicine2005 - presentBrain Stimulation2007 - present Associate Editor2007 - 2011Journal of Movement Disorders (Korea)2008 - presentWorld Neurology2005 - present Editor in Chief2008 -- 2012

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  1. 2004US 6,780,413 B2 (August 24, 2004) Immunotoxin (MAB-Ricin) for the treatment of focal movement disorders. Inventors: J. Hott, R. Youle, M. Hallett and M. Dalakas. 2000US 7,407,478 (August 5, 2008) Coil for Magnetic Stimulation and methods for using the same. Inventors: A. Zangen, R. Wise, M. Hallett, Y. Roth, P. Miranda. (European Patent Office 01987684.6-2305-US0150737)

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  1. Ariston Pharmaceuticals, a CRADA with NIH to support experimental pharmaceutical therapy for essential tremor (PI, 2007-present); BCN Peptides, a CTA with NIH tosupport experimental pharmaceutical therapy forblepharospasm (PI, 2010-present).

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  1. National Institute of Neurological Disorders and Stroke Intramural Program (1984 to present)US Army, Grant for study of non-invasive brain stimulation for the treatment of Parkinson's disease (PI, 2007-2010)

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and H.A. Jinnah, MD, PhD

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  1. (1) Bachmann-Strauss Dystonia & Parkinson's Foundation, (2) Dystonia Medical Research Foundation, (3) National Spasmodic Torticollis Association, (4) Tyler's Hope

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  1. (1) Psyadon Pharmaceuticals

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  1. PI: NINDS NS067501, NS040470, NS061349, NS033592

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  1. (1) Emory Neurosciences Initiative(2) Atlanta Clinical and Translational Institute

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Abstract

A 57-year-old man presented with spasms of his left jaw. Two years prior, he had developed left-sided facial numbness followed by development of left-sided shock-like pain and then involuntary and repetitive movements of the jaw-closing muscles. Jaw muscle contractions were episodic, interfered with chewing and talking, and led to frequent tongue biting. Individual spasms varied from seconds to minutes and he reported that function in between episodes was normal, although his wife felt he spoke with reduced mouth opening. He sometimes awoke with a bloody tongue, suggesting that contractions occurred during waking and sleep. He had no history of premonitory sensation or relief associated with the spasms. He reported no difficulty swallowing.

SECTION 1

A 57-year-old man presented with spasms of his left jaw. Two years prior, he had developed left-sided facial numbness followed by development of left-sided shock-like pain and then involuntary and repetitive movements of the jaw-closing muscles. Jaw muscle contractions were episodic, interfered with chewing and talking, and led to frequent tongue biting. Individual spasms varied from seconds to minutes and he reported that function in between episodes was normal, although his wife felt he spoke with reduced mouth opening. He sometimes awoke with a bloody tongue, suggesting that contractions occurred during waking and sleep. He had no history of premonitory sensation or relief associated with the spasms. He reported no difficulty swallowing.

Neurologic examination demonstrated decreased sensation in all 3 trigeminal divisions on the left and a reduced corneal reflex on the left. Masseter bulk, tone, and strength were normal bilaterally. Contractions were noted in the jaw-closing muscles. Mouth opening was limited during speech. There was no facial weakness. There was no palatal tremor. Head rotation, shoulder shrugging, and tongue movements were normal. With the exception of mild-to-moderate distal sensory deficit, the remainder of the neurologic examination was unremarkable.

Questions for consideration:

  1. What is the localization?
  2. What are potential etiologies?

GO TO SECTION 2

SECTION 2

This patient's examination revealed 2 localizing abnormalities: decreased left facial sensation with diminished corneal reflex and frequent contractions of the left jaw muscles. Both findings localize to the trigeminal nerve. The sensory fibers of the trigeminal nerve originate at the trigeminal nucleus in the pons and the motor fibers of the trigeminal nerve originate in the pons in an area close to, but not in, the trigeminal nucleus. Sensory fibers project from the trigeminal nucleus to the trigeminal ganglion, at which point they split into 3 branch distributions: ophthalmic (V1), maxillary (V2), and mandibular (V3). Motor fibers project from the pons, decussate, track adjacent to the trigeminal nucleus, and eventually exit the cranium adjacent to V3 through the foramen ovale. From the foramen ovale, motor fibers separate from sensory fibers and radiate to the masseter and other jaw-moving muscles.

The sensory and motor branches of this patient's nerve were both affected. We therefore infer that an intracranial lesion of the trigeminal nerve must be causing the patient's difficulties. Possible etiologies of the lesion include vascular, infectious, autoimmune, trauma, or a mass. The slow onset of this patient's difficulties decreases the likelihood of vascular lesions and there was no history of trauma. A lesion isolated to the trigeminal nerve would be an unusual presentation of an infectious or autoimmune etiology such as multiple sclerosis, Devic disease, or Listeria, and the patient had no current or past symptoms consistent with these illnesses. Considering these factors, the most likely etiology of this patient's isolated trigeminal nerve problem is a central tumor or mass.

Questions for consideration:

  1. What testing will narrow this differential?
  2. What can be done to help the patient's symptoms?

GO TO SECTION 3

SECTION 3

EMG recordings demonstrated fasciculation potentials in the left masseter muscle. Spasms in the left masseter were characterized by increasing frequency of fasciculation discharges, often in bursts, followed by high-frequency discharges of multiple units. Individual motor units fired at frequencies up to 80 Hz (only identifiable with certainty at the beginning and end of spasms). No spasms or abnormal discharges were seen on the right. These EMG results confirmed excess activity of the left trigeminal motor nerve.

Blink reflex was elicited by stimulating the supraorbital nerve, and blink reflex and jaw jerk studies were normal bilaterally. The masseteric inhibitory reflex was elicited by stimulating the right mental nerve at the mental foramen with a 1-cm bipolar stimulator with a 0.2-msec duration stimulus at 28.6 mA. Masseteric inhibitory reflexes were tested during voluntary activation and involuntary spasms. During voluntary activation of the masseter, normal S1 and S2 silent periods were evoked by mental nerve stimulation. During involuntary spasms, when the same stimulation was given, there were no silent periods. Occasionally, high-frequency motor unit potentials could be elicited by voluntary muscle activation.

Brain MRI revealed a hemangioma located adjacent to the left pons in the area of the trigeminal nerve exiting zone (figure, A). The lesion was considered inoperable, but the patient experienced substantial relief with a combination of duloxetine and gabapentin. His jaw twitching almost completely resolved and pain was significantly reduced.

Figure
MRI and schematic representation of the patient's intracranial lesion

GO TO SECTION 4

SECTION 4

Given the history, examination, and these electrophysiologic findings, the patient was given a formal diagnosis of hemimasticatory spasm (HMS) caused by a central lesion. HMS is a rare disorder of involuntary brief contractions of masticatory muscles on one side and is thought to result from irritation of the ipsilateral trigeminal nerve. Our case differs from most reported cases in having a central site of origin.13

It has been suggested that the pathophysiology of HMS lies in ectopic activation of trigeminal motor axons due to their peripheral irritation and subsequent ephaptic transmission.1,4 The notion of peripheral nerve pathology is supported by slowed trigeminal motor nerve conduction as evidenced by delayed jaw jerk reflex latencies,1,4 ipsilateral hemiatrophy in some cases,5,6 presence of fasciculation potentials, and absent silent period during spasms while being present during voluntary activation.1,4 Pathologic involvement of CNS pathways has been suggested in HMS,4,7 but on a functional (i.e., remodeling of brainstem pathways by abnormal sensory input), rather than structural (i.e., a CNS lesion), basis. However, in our case, we observed HMS with confirmed structural central origin.

Similarities and differences in clinical presentation between this case and previously reported cases of HMS can be explained by the central origin of the structural abnormality. Similar to our patient, most previously reported cases of HMS had normal blink reflex studies, indicating that ophthalmic branch (V1) of the trigeminal nerve was not affected.2,4,5,8,9 Our patient was different in that he had clear sensory deficits that are also attributed to trigeminal nerve lesions—specifically the decreased facial sensation and corneal reflex (figure, B).

Our patient differed from most previous cases of HMS in that he had a reduced corneal reflex and normal jaw jerk. This finding reinforces our conclusion of unique HMS etiology in our patient, as the trigeminal nerve is likely unaffected in the periphery. A focal irritation of the central portion of the trigeminal motor axons in the pons by the hemangioma produces neither measurable delay in reflex latency nor loss of axons, as was demonstrated in this patient.

Dull, long-lasting, and aching pain may accompany HMS and is usually correlated with spasms. However, our patient experienced transient, shock-like pain. We hypothesize that dull pain typically reported in HMS is a consequence of spasms and independent of etiology of cases with peripheral origin. The pain reported by our patient was not associated with spasms, and was likely caused by the direct compression of the central trigeminal sensory axons by the tumor.

The MRI confirmed that the patient had a left pontine hemangioma. We conclude that this case of HMS was caused by compression of the fifth nerve at its exit from the pons due to that hemangioma. Although the etiology of HMS commonly relates to trigeminal pathology outside the CNS, central causes should also be suspected in patients with HMS. Brain imaging should be recommended in all cases, with particular attention paid to the posterior fossa.

ACKNOWLEDGMENT

The authors thank Dr. Mary Kay Floeter for planning and supervising the electrophysiologic testing.

AUTHOR CONTRIBUTIONS

Dr. Mari drafted/revised the manuscript for content, acquired data, analyzed and interpreted data, and supervised and coordinated study. Dr. Rosenthal drafted/revised the manuscript for content and analyzed and interpreted data. Ms. Darwin drafted/revised the manuscript for content and analyzed and interpreted data. Dr. Hallett drafted/revised the manuscript for content, analyzed and interpreted data, and supervised and coordinated study. Dr. Jinnah drafted/revised the manuscript for content and analyzed and interpreted data.

STUDY FUNDING

Supported by an NIH intramural grant.

DISCLOSURE

The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

REFERENCES

1. Thompson PD, Carroll WM. Hemimasticatory spasm: a peripheral paroxysmal cranial neuropathy? J Neurol Neurosurg Psychiatry 1983;46:274–276. [PMC free article] [PubMed]
2. Auger RG, Litchy WJ, Cascino TL, Ahlskog JE. Hemimasticatory spasm: clinical and electrophysiologic observations. Neurology 1992;42:2263–2266. [PubMed]
3. Cruccu G, Inghilleri M, Berardelli A, et al. Pathophysiology of hemimasticatory spasm. J Neurol Neurosurg Psychiatry 1994;57:43–50. [PMC free article] [PubMed]
4. Mir P, Gilio F, Edwards M, et al. Alteration of central motor excitability in a patient with hemimasticatory spasm after treatment with botulinum toxin injections. Mov Disord 2006;21:73–78. [PubMed]
5. Ebersbach G, Kabus C, Schelosky L, Terstegge L, Poewe W. Hemimasticatory spasm in hemifacial atrophy: diagnostic and therapeutic aspects in two patients. Mov Disord 1995;10:504–507. [PubMed]
6. Kim HJ, Jeon BS, Lee KW. Hemimasticatory spasm associated with localized scleroderma and facial hemiatrophy. Arch Neurol 2000;57:576–580. [PubMed]
7. Lagueny A, Deliac MM, Julien J, Demotes-Mainard J, Ferrer X. Jaw closing spasm: a form of focal dystonia? An electrophysiological study. J Neurol Neurosurg Psychiatry 1989;52:652–655. [PMC free article] [PubMed]
8. Esteban A, Traba A, Prieto J, Grandas F. Long term follow up of a hemimasticatory spasm. Acta Neurol Scand 2002;105:67–72. [PubMed]
9. Jimenez-Jimenez FJ, Puertas I, Alonso-Navarro H. Hemimasticatory spasm secondary to biopercular syndrome. Eur Neurol 2008;59:276–279. [PubMed]

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