Hemifacial
Spasm
Occurrence in Multiple Sclerosis Fred F. Telischi, MD; Lawrence R. Grobman, MD; William A. Marc Apple, MD; Ram Ayyar, MD
\s=b\ We
present six patients with hemifacial
spasm and
multiple sclerosis. To our knowledge, this association has not been described previously in the North American literature. Magnetic resonance imaging was obtained in all the patients and plaques consistent with multiple sclerosis were identified. In two patients the plaques were seen in the area of the facial nucleus on the involved side. We suggest that hemifacial spasm can be a manifestation of multiple sclerosis. These cases illustrate the utility of magnetic resonance imaging in the investigation of hemifacial spasm. Our findings also support a central (nuclear) origin in multiple sclerosis associated with hemifacial spasm. (Arch Otolaryngol Head Neck Surg. 1991;117:554-556)
(HFS) Hemifacial syndrome of
is a chron¬ spasm intermittent or ic sustained unilateral facial contraction, most commonly resulting from an extramedullary compression of posterior fossa structures by anomalous ectatic Accepted for publication January 2,1991. From the Departments of Otolaryngology (Drs Telischi, Grobman, and Apple) and Neurology (Drs Sheremata and Ayyar), University of Miami (Fla) School of Medicine.
Reprint requests to the Department of Otolaryngology (D-48), University of Miami School of Medicine, 1666 NW 10th Ave, ACC-East, Suite 306, Miami, FL 33136 (Dr Telischi).
Sheremata, MD;
blood vessels. Tumors (including acoustic neuromas, cholesteatomas, and meningiomas) have also been im¬ plicated in producing this clinical pic¬ ture.1 Occasionally HFS is unexplained and referred to as "essential" or "idiopathic." The spasm usually begins in the orbicularis oculi muscle and gradu¬ ally spreads downward to include all the muscles of facial expression.2 Spasms may be triggered by emotional disturbance and eventually mild facial muscle weakness may develop." In id-
iopathic
cases,
long-term
recovery
without treatment is unusual.4 Electromyographically (EMG), HFS consists of repetitive, brief bursts of high-fre¬ quency motor unit discharges.0 These occur irregularly and synkinesis is seen in widely separated muscles uni¬ laterally, particularly in the orbicularis oris and orbicularis oculi muscles. Syn¬ kinesis commonly is associated with
HFS.5'6
Multiple sclerosis (MS) is a disease of the central nervous system charac¬ terized by relapsing and remitting symptoms and signs with evidence of disseminated lesions in the brain and spinal cord. Plaques of demyelination result from focal perivascular accumu¬ lation of lymphocytes and activated macrophages.' The inflammatory pro¬ cess eventually results in the forma-
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
gliotic scar. Treatment with corticotropin or prednisone, which are commonly used, stabilize the mem¬ branes.8 Azathioprine (Imuran) and cytion of
a
may decrease disease such as Anticonvulsants progression.9 phenytoin (Dilantin) and carbamazepine (Tegretol) can control paroxysmal clinical signs such as paroxysmal dystonia and trigeminal neuralgia when
totoxic
agents
they occur.10
REPORT OF CASES Hemifacial spasm was seen in six patients who were diagnosed as having MS. The age of the patients ranged from 24 to 43 years, four were women and two were men. The duration of MS prior to developing HFS var¬ ied from less than 1 year to 10 years (Fig 1). All patients were evaluated by at least two neurologists and the diagnosis of clinically definite MS was established accepted to diag¬ nostic criteria.4 Four patients consented to EMG study. Two patients revealed synkinesis. One pa¬ tient exhibited a longer latency on the side of spasm and another showed waveforms more typical of myokymia. All magnetic resonance imaging (MRI) scans showed multiple areas of increased T2 signal scattered throughout the white matter of the brain, consistent with MS. Two patients had these lesions demon¬ strated in the lower pons on the side of their HFS. Treatment with various regimens of corticotropin, prednisone, and carbamazepine resulted in partial or complete relief of
their spasm. In two patients, the HFS re¬ curred, but to a much lesser degree than originally present. One patient, who had an antecedent history of facial myokymia along with other findings of MS, had episodes of classic HFS interspersed with myokymia. The spasm eventually resolved but intermit¬ tent myokymia persisted. Another patient developed HFS several years after removal of an intracanalicular acoustic neuroma from the opposite side. Summaries of two cases are
presented as examples.
Case 1.—A 47-year-old white woman (pa¬ tient 5, Fig 1) who had been diagnosed with MS 10 years previously presented with fa¬ tigue and decreased ability to walk. Seven months after onset of these symptoms twitches began intermittently at the left cor¬ ner of her mouth. These increased in fre¬ quency and extent, evolving into recurrent spasms, including all of the facial muscles on that side. Her MRI revealed multiple areas of increased signal in the cerebral white mat¬ ter, as well as a prominent area of increased signal in the left lower pons (Fig 2). An EMG revealed synkinesis of the left orbicularis oculi and orbicularis oris muscles. Treatment with corticotropin resulted in relief of her symptoms. Six months later, she developed minimal myokymia on the left side. CASE 2.- Patient 6 (Fig 1), a 34-year-old white woman, had developed a left facial pa¬ ralysis 10 years previously. Four years after that she developed retrobulbar neuritis and later, trigemina] neuralgia. Six years later retrobulbar neuritis developed on the oppo¬ site side and an MRI of her brain at that time showed multiple white matter lesions consis¬ tent with MS. In addition, an intracanalicular acoustic neuroma was found on the right and removed. Ten years after she first developed the left facial paralysis, intermittent contrac¬ tion of the whole left side of her face began abruptly, and a diagnosis of HFS was made. A brain MRI at the time revealed a new area of increased T2 signal in the left lower pons (Fig 3). Carbamazepine administration re¬ sulted in a prompt and marked reduction of her symptoms and she was asymptomatic 1 month later. She did not consent to an EMG.
Patients Duration of multiple sclerosis In years before developing hemifacial spasm in patients studied. Patients 5 and 6 are referred to as cases 1 and 2 in the "Report of Cases" section. The age range of the patients was 23 to 44 years; four of the patients were women and two were men.
Fig 1
—
.
Fig 2. —Magnetic resonance imaging of brain stem of patient 5 demonstrates an area of in¬ creased signal In the left lower pons. The arrow indicates the area of increased T2 signal in the anterior pons.
COMMENT for HFS such as a vertebral aneurysm or mass lesion cannot be identified in all patients. Until recently computed tomographic scanning has been the initial radiologie study of choice.12 However, we have used MRI in patients with HFS to visualize en¬ larged (ectactic) vertebrobasilar ves¬ sels and to identify possible central nervous system causes of the HFS. Magnetic resonance imaging is now A
cause
established as the modality of choice for imaging the MS plaques.13 The visu¬ alized lesions are typically scattered through the cerebral and brain-stem white matter in 90% of patients.1415 The presence of lesions support the diagno¬ sis of MS, but is not required for diagnosis, since the diagnosis rests on clinical grounds.12 Anomalous ectatic vessels have been implicated as the cause of HFS with compression of the seventh nerve at its
Fig 3. —Magnetic resonance imaging of brain stem of patient 6 with previous right acoustic neuroma who developed left-sided hemifacial spasm. The arrow indicates a new area of in¬ creased T2 signal in the left lower pons.
entrance into the brain stem at the
lower
pons.1" Cholesteatomas,
neuromas, and other
mass
acoustic
lesions, in¬
cluding aneurysms, have also been de¬
scribed as causes of HFS.1 In one of our patients, a contralater¬ al intracanalicular acoustic neuroma was present. Nishi et al17 have report¬ ed a case of HFS occurring on the side opposite an acoustic neuroma, but in
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
their
case
the
mass was
larger and
impinging on the brain stem.
Among the movement disorders in the differential diagnosis of HFS, facial myokymia is the one most often associated with MS.18 Myokymia con¬ sists of undulations and flickering of facial muscles that may occur from the frontalis to the platysma. This condi¬ tion is usually unilateral and, unlike HFS, involves individual muscle fasci¬ cles rather than entire muscle groups. Myokymia is characterized by the ap¬ pearance of rhythmic bursts of a single motor unit. When the EMG is recorded from more than one site, the dis¬ charges are not synchronized among different muscles, or even in the same muscle. Synkinesis typically is not present.0 To our knowledge, the occurrence of HFS and myokymia in the same pa¬ tient has not been reported previously. Auger5 and others have presented evi¬ dence that myokymia and HFS are easily distinguished using EMG crite¬ ria, although Horowitz et al19 suggest that the two can coexist. The origin and mechanism of the
electrophysiologic activity underlying HFS is not yet understood. The most common theories include supranuclear stimulation, aberrant regeneration, a
hyperexcitable facial nerve nucleus, and ephaptic spread of the nerve im¬ pulse. Supranuclear and psychogenic causes
of HFS
are
not considered valid
explanations considering
numerous
that have been successfully treated with surgery and the involun¬ tary nature of HFS.3 Explanation of HFS by ephaptic transmission (crosscases
talk)
popularized by Gardner.2" from ephapse, which means to touch or a point of contact. This theory states that mass depolar¬ was
The term
comes
ization
occurs in areas where de¬ creased resistance lowers the firing threshold in damaged or ischemie nerves. Electrophysiologic studies ap¬ pear to disprove ephaptic transmis¬ sion, however, based on intraoperative recording in patients undergoing de¬ compression for HFS.2 Measurements of conduction times showed the cross transmission of antidromic activity oc¬ curs central to the site of vascular compression of the facial nerve. Moller2 proposed an alternative ex¬ planation for HFS. He postulated that the root entry zone is damaged and becomes a trigger where impulses are generated and transmitted orthodromically as well as antidromically. The antidromic impulse would activate fa¬ cial nuclei motor neurons, which in
impulses down the seventh cranial nerve and cause the HFS. He labeled this phenomenon as the kindling effect.
turn would send
Finally, Wartenberg,21 Ferguson,22
and others have proposed dysfunction and reorganization in the facial nucleus to explain the spasm. Our finding of MS plaques in the lower pons at the level of the facial nerve nuclei in two patients with HFS supports the concept that in some cases, such as ours, a mechanism for the origin of facial spasm involves the facial nucleus directly. In the other patients with MS, plaques were found in the brain stem as well as elsewhere in the central white matter. Although it may not be evidenced by imaging, we hypothesize that in MS with HFS the inflammatory lesions associated with the central demyelinating process probably regularly involves the facial nucleus or the proximal portion of the facial nerve. We have described an association of HFS with MS in six patients. We suggest that MRI be used in the evalu¬ ation of cases of HFS. The MRI will identify mass lesions and unusual vasculature as well as other central ner¬ vous system disease such as the plaques of MS.
References 1. Davis WE, Luterman BF, Pullian W. Hemifacial spasm caused by cholesteatoma. Am J Otol.
1981;2:272-273.
2. Moller AR. Hemifacial spasm: ephaptic transmission or hyperexcitability of the facial motor nucleus? Exp Neurol. 1987;98:110-119. 3. Bratzlavsky M, Vander Eken H. Hemifacial spasm: a psychosomatic disease? Acta Neurol Belg.
1982;82:5-11. 4. Digre K, Corbertt JJ. Hemifacial spasm: differential diagnosis, mechanism, and treatment. Adv Neurol. 1988;49:151-176.
Auger RG. Hemifacial spasm: clinical and electrophysiologic observations. Neurology. 5.
1979;29:1261-1272. 6. Kim P, Fukyushima T. Observations on synkinesis in patients with hemifacial spasm. J Neurosurg. 1984;60:821-827. 7. Wisniewski H. Immunopathology of demye-
lination in autoimmune diseases and virus infections. Br Med Bull. 1977;33:54-59. 8. Smith KJ, Blackmore WF, McDonald WI. Central remyelination restores secure conduction. Nature. 1979;280:395-396. 9. Hauser SL, Dawson DM, Lehrich JR. Inten-
sive immunosuppression in progressive multiple sclerosis: a randomized three-arm study of high\x=req-\ dose intravenous cyclophosphamide, plasma exchange and ACTH. N Engl J Med. 1983;308:173\x=req-\ 180. 10. Espir MLE, Millar P. Treatment of paroxysmal disorders in multiple sclerosis with carbamazepine (Tegretol). J Neurol Neurosurg Psychiatry.
1970;33:528-531.
Honig LS, Sheremata WA. Magnetic resoimaging of spinal cord lesions in multiple sclerosis. J Neurol Neurosurg Psychiatry. 1989;52:459-466. 16. Jannetta PJ, Abbasy M, Maroon JC. Etiology and definitive microsurgical treatment of hemifacial spasm. J Neurosurg. 1977;47:321-328. 17. Nishi T, Matsukado Y, Nagahiro S, Fukushma M, Koga K. Hemifacial spasm due to con-
New
ogy. 1987;37:339-342.
CM, Paty DW, Scheinberg L, et al. diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol. 11. Poser
1983;13:227-231. 12. Sobel D, Normal D, York CH, Newton TH. Radiography of trigeminal neuralgia and hemifacial spasm. Am J Radiol. 1980;135:93-95. 13. Paty DW, Hashimoto SA, Hooge H, et al. Magnetic resonance imaging (MRI) in multiple sclerosis (MS): a prospective evaluation of usefulness in diagnosis. Neurology. 1986;36(suppl 1):86. 14. Honig LS, Siddharthan R, Sheremata WA, Sheldon JJ, Sazant A. Multiple sclerosis: correlaof magnetic resonance imaging with cerebrospinal fluid findings. J Neurol Neurosurg Psychiatry. 1988;51:277-280.
tion
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
15.
nance
tralateral acoustic
neuroma: case
report. Neurol-
18. Anderman F, Cosgrove JB, Lloyd-Smith DL, Gloor P, McNaughton FL. Facial myokymia in multiple sclerosis. Brain. 1961;84:31-44. 19. Horowitz SH. Hemifacial spasm and facial
myokymia: electrophysiological findings. Muscle Nerve. 1987;10:422-427.
20. Gardner WJ. Cross talk: the paradoxical transmission of a nerve impulse. Arch Neurol.
1966;14:149-156. 21. Wartenberg R. Hemifacial Spasm: A Clinical and Pathophysiological Study. New York, NY: Oxford University Press Inc; 1952. 22. Ferguson JH. Hemifacial spasm and the facial nucleus. Ann Neurol. 1978;4:97-103.
Spasm
Occurrence in Multiple Sclerosis Fred F. Telischi, MD; Lawrence R. Grobman, MD; William A. Marc Apple, MD; Ram Ayyar, MD
\s=b\ We
present six patients with hemifacial
spasm and
multiple sclerosis. To our knowledge, this association has not been described previously in the North American literature. Magnetic resonance imaging was obtained in all the patients and plaques consistent with multiple sclerosis were identified. In two patients the plaques were seen in the area of the facial nucleus on the involved side. We suggest that hemifacial spasm can be a manifestation of multiple sclerosis. These cases illustrate the utility of magnetic resonance imaging in the investigation of hemifacial spasm. Our findings also support a central (nuclear) origin in multiple sclerosis associated with hemifacial spasm. (Arch Otolaryngol Head Neck Surg. 1991;117:554-556)
(HFS) Hemifacial syndrome of
is a chron¬ spasm intermittent or ic sustained unilateral facial contraction, most commonly resulting from an extramedullary compression of posterior fossa structures by anomalous ectatic Accepted for publication January 2,1991. From the Departments of Otolaryngology (Drs Telischi, Grobman, and Apple) and Neurology (Drs Sheremata and Ayyar), University of Miami (Fla) School of Medicine.
Reprint requests to the Department of Otolaryngology (D-48), University of Miami School of Medicine, 1666 NW 10th Ave, ACC-East, Suite 306, Miami, FL 33136 (Dr Telischi).
Sheremata, MD;
blood vessels. Tumors (including acoustic neuromas, cholesteatomas, and meningiomas) have also been im¬ plicated in producing this clinical pic¬ ture.1 Occasionally HFS is unexplained and referred to as "essential" or "idiopathic." The spasm usually begins in the orbicularis oculi muscle and gradu¬ ally spreads downward to include all the muscles of facial expression.2 Spasms may be triggered by emotional disturbance and eventually mild facial muscle weakness may develop." In id-
iopathic
cases,
long-term
recovery
without treatment is unusual.4 Electromyographically (EMG), HFS consists of repetitive, brief bursts of high-fre¬ quency motor unit discharges.0 These occur irregularly and synkinesis is seen in widely separated muscles uni¬ laterally, particularly in the orbicularis oris and orbicularis oculi muscles. Syn¬ kinesis commonly is associated with
HFS.5'6
Multiple sclerosis (MS) is a disease of the central nervous system charac¬ terized by relapsing and remitting symptoms and signs with evidence of disseminated lesions in the brain and spinal cord. Plaques of demyelination result from focal perivascular accumu¬ lation of lymphocytes and activated macrophages.' The inflammatory pro¬ cess eventually results in the forma-
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
gliotic scar. Treatment with corticotropin or prednisone, which are commonly used, stabilize the mem¬ branes.8 Azathioprine (Imuran) and cytion of
a
may decrease disease such as Anticonvulsants progression.9 phenytoin (Dilantin) and carbamazepine (Tegretol) can control paroxysmal clinical signs such as paroxysmal dystonia and trigeminal neuralgia when
totoxic
agents
they occur.10
REPORT OF CASES Hemifacial spasm was seen in six patients who were diagnosed as having MS. The age of the patients ranged from 24 to 43 years, four were women and two were men. The duration of MS prior to developing HFS var¬ ied from less than 1 year to 10 years (Fig 1). All patients were evaluated by at least two neurologists and the diagnosis of clinically definite MS was established accepted to diag¬ nostic criteria.4 Four patients consented to EMG study. Two patients revealed synkinesis. One pa¬ tient exhibited a longer latency on the side of spasm and another showed waveforms more typical of myokymia. All magnetic resonance imaging (MRI) scans showed multiple areas of increased T2 signal scattered throughout the white matter of the brain, consistent with MS. Two patients had these lesions demon¬ strated in the lower pons on the side of their HFS. Treatment with various regimens of corticotropin, prednisone, and carbamazepine resulted in partial or complete relief of
their spasm. In two patients, the HFS re¬ curred, but to a much lesser degree than originally present. One patient, who had an antecedent history of facial myokymia along with other findings of MS, had episodes of classic HFS interspersed with myokymia. The spasm eventually resolved but intermit¬ tent myokymia persisted. Another patient developed HFS several years after removal of an intracanalicular acoustic neuroma from the opposite side. Summaries of two cases are
presented as examples.
Case 1.—A 47-year-old white woman (pa¬ tient 5, Fig 1) who had been diagnosed with MS 10 years previously presented with fa¬ tigue and decreased ability to walk. Seven months after onset of these symptoms twitches began intermittently at the left cor¬ ner of her mouth. These increased in fre¬ quency and extent, evolving into recurrent spasms, including all of the facial muscles on that side. Her MRI revealed multiple areas of increased signal in the cerebral white mat¬ ter, as well as a prominent area of increased signal in the left lower pons (Fig 2). An EMG revealed synkinesis of the left orbicularis oculi and orbicularis oris muscles. Treatment with corticotropin resulted in relief of her symptoms. Six months later, she developed minimal myokymia on the left side. CASE 2.- Patient 6 (Fig 1), a 34-year-old white woman, had developed a left facial pa¬ ralysis 10 years previously. Four years after that she developed retrobulbar neuritis and later, trigemina] neuralgia. Six years later retrobulbar neuritis developed on the oppo¬ site side and an MRI of her brain at that time showed multiple white matter lesions consis¬ tent with MS. In addition, an intracanalicular acoustic neuroma was found on the right and removed. Ten years after she first developed the left facial paralysis, intermittent contrac¬ tion of the whole left side of her face began abruptly, and a diagnosis of HFS was made. A brain MRI at the time revealed a new area of increased T2 signal in the left lower pons (Fig 3). Carbamazepine administration re¬ sulted in a prompt and marked reduction of her symptoms and she was asymptomatic 1 month later. She did not consent to an EMG.
Patients Duration of multiple sclerosis In years before developing hemifacial spasm in patients studied. Patients 5 and 6 are referred to as cases 1 and 2 in the "Report of Cases" section. The age range of the patients was 23 to 44 years; four of the patients were women and two were men.
Fig 1
—
.
Fig 2. —Magnetic resonance imaging of brain stem of patient 5 demonstrates an area of in¬ creased signal In the left lower pons. The arrow indicates the area of increased T2 signal in the anterior pons.
COMMENT for HFS such as a vertebral aneurysm or mass lesion cannot be identified in all patients. Until recently computed tomographic scanning has been the initial radiologie study of choice.12 However, we have used MRI in patients with HFS to visualize en¬ larged (ectactic) vertebrobasilar ves¬ sels and to identify possible central nervous system causes of the HFS. Magnetic resonance imaging is now A
cause
established as the modality of choice for imaging the MS plaques.13 The visu¬ alized lesions are typically scattered through the cerebral and brain-stem white matter in 90% of patients.1415 The presence of lesions support the diagno¬ sis of MS, but is not required for diagnosis, since the diagnosis rests on clinical grounds.12 Anomalous ectatic vessels have been implicated as the cause of HFS with compression of the seventh nerve at its
Fig 3. —Magnetic resonance imaging of brain stem of patient 6 with previous right acoustic neuroma who developed left-sided hemifacial spasm. The arrow indicates a new area of in¬ creased T2 signal in the left lower pons.
entrance into the brain stem at the
lower
pons.1" Cholesteatomas,
neuromas, and other
mass
acoustic
lesions, in¬
cluding aneurysms, have also been de¬
scribed as causes of HFS.1 In one of our patients, a contralater¬ al intracanalicular acoustic neuroma was present. Nishi et al17 have report¬ ed a case of HFS occurring on the side opposite an acoustic neuroma, but in
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
their
case
the
mass was
larger and
impinging on the brain stem.
Among the movement disorders in the differential diagnosis of HFS, facial myokymia is the one most often associated with MS.18 Myokymia con¬ sists of undulations and flickering of facial muscles that may occur from the frontalis to the platysma. This condi¬ tion is usually unilateral and, unlike HFS, involves individual muscle fasci¬ cles rather than entire muscle groups. Myokymia is characterized by the ap¬ pearance of rhythmic bursts of a single motor unit. When the EMG is recorded from more than one site, the dis¬ charges are not synchronized among different muscles, or even in the same muscle. Synkinesis typically is not present.0 To our knowledge, the occurrence of HFS and myokymia in the same pa¬ tient has not been reported previously. Auger5 and others have presented evi¬ dence that myokymia and HFS are easily distinguished using EMG crite¬ ria, although Horowitz et al19 suggest that the two can coexist. The origin and mechanism of the
electrophysiologic activity underlying HFS is not yet understood. The most common theories include supranuclear stimulation, aberrant regeneration, a
hyperexcitable facial nerve nucleus, and ephaptic spread of the nerve im¬ pulse. Supranuclear and psychogenic causes
of HFS
are
not considered valid
explanations considering
numerous
that have been successfully treated with surgery and the involun¬ tary nature of HFS.3 Explanation of HFS by ephaptic transmission (crosscases
talk)
popularized by Gardner.2" from ephapse, which means to touch or a point of contact. This theory states that mass depolar¬ was
The term
comes
ization
occurs in areas where de¬ creased resistance lowers the firing threshold in damaged or ischemie nerves. Electrophysiologic studies ap¬ pear to disprove ephaptic transmis¬ sion, however, based on intraoperative recording in patients undergoing de¬ compression for HFS.2 Measurements of conduction times showed the cross transmission of antidromic activity oc¬ curs central to the site of vascular compression of the facial nerve. Moller2 proposed an alternative ex¬ planation for HFS. He postulated that the root entry zone is damaged and becomes a trigger where impulses are generated and transmitted orthodromically as well as antidromically. The antidromic impulse would activate fa¬ cial nuclei motor neurons, which in
impulses down the seventh cranial nerve and cause the HFS. He labeled this phenomenon as the kindling effect.
turn would send
Finally, Wartenberg,21 Ferguson,22
and others have proposed dysfunction and reorganization in the facial nucleus to explain the spasm. Our finding of MS plaques in the lower pons at the level of the facial nerve nuclei in two patients with HFS supports the concept that in some cases, such as ours, a mechanism for the origin of facial spasm involves the facial nucleus directly. In the other patients with MS, plaques were found in the brain stem as well as elsewhere in the central white matter. Although it may not be evidenced by imaging, we hypothesize that in MS with HFS the inflammatory lesions associated with the central demyelinating process probably regularly involves the facial nucleus or the proximal portion of the facial nerve. We have described an association of HFS with MS in six patients. We suggest that MRI be used in the evalu¬ ation of cases of HFS. The MRI will identify mass lesions and unusual vasculature as well as other central ner¬ vous system disease such as the plaques of MS.
References 1. Davis WE, Luterman BF, Pullian W. Hemifacial spasm caused by cholesteatoma. Am J Otol.
1981;2:272-273.
2. Moller AR. Hemifacial spasm: ephaptic transmission or hyperexcitability of the facial motor nucleus? Exp Neurol. 1987;98:110-119. 3. Bratzlavsky M, Vander Eken H. Hemifacial spasm: a psychosomatic disease? Acta Neurol Belg.
1982;82:5-11. 4. Digre K, Corbertt JJ. Hemifacial spasm: differential diagnosis, mechanism, and treatment. Adv Neurol. 1988;49:151-176.
Auger RG. Hemifacial spasm: clinical and electrophysiologic observations. Neurology. 5.
1979;29:1261-1272. 6. Kim P, Fukyushima T. Observations on synkinesis in patients with hemifacial spasm. J Neurosurg. 1984;60:821-827. 7. Wisniewski H. Immunopathology of demye-
lination in autoimmune diseases and virus infections. Br Med Bull. 1977;33:54-59. 8. Smith KJ, Blackmore WF, McDonald WI. Central remyelination restores secure conduction. Nature. 1979;280:395-396. 9. Hauser SL, Dawson DM, Lehrich JR. Inten-
sive immunosuppression in progressive multiple sclerosis: a randomized three-arm study of high\x=req-\ dose intravenous cyclophosphamide, plasma exchange and ACTH. N Engl J Med. 1983;308:173\x=req-\ 180. 10. Espir MLE, Millar P. Treatment of paroxysmal disorders in multiple sclerosis with carbamazepine (Tegretol). J Neurol Neurosurg Psychiatry.
1970;33:528-531.
Honig LS, Sheremata WA. Magnetic resoimaging of spinal cord lesions in multiple sclerosis. J Neurol Neurosurg Psychiatry. 1989;52:459-466. 16. Jannetta PJ, Abbasy M, Maroon JC. Etiology and definitive microsurgical treatment of hemifacial spasm. J Neurosurg. 1977;47:321-328. 17. Nishi T, Matsukado Y, Nagahiro S, Fukushma M, Koga K. Hemifacial spasm due to con-
New
ogy. 1987;37:339-342.
CM, Paty DW, Scheinberg L, et al. diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol. 11. Poser
1983;13:227-231. 12. Sobel D, Normal D, York CH, Newton TH. Radiography of trigeminal neuralgia and hemifacial spasm. Am J Radiol. 1980;135:93-95. 13. Paty DW, Hashimoto SA, Hooge H, et al. Magnetic resonance imaging (MRI) in multiple sclerosis (MS): a prospective evaluation of usefulness in diagnosis. Neurology. 1986;36(suppl 1):86. 14. Honig LS, Siddharthan R, Sheremata WA, Sheldon JJ, Sazant A. Multiple sclerosis: correlaof magnetic resonance imaging with cerebrospinal fluid findings. J Neurol Neurosurg Psychiatry. 1988;51:277-280.
tion
Downloaded From: http://archotol.jamanetwork.com/ by a University of Pittsburgh User on 06/18/2015
15.
nance
tralateral acoustic
neuroma: case
report. Neurol-
18. Anderman F, Cosgrove JB, Lloyd-Smith DL, Gloor P, McNaughton FL. Facial myokymia in multiple sclerosis. Brain. 1961;84:31-44. 19. Horowitz SH. Hemifacial spasm and facial
myokymia: electrophysiological findings. Muscle Nerve. 1987;10:422-427.
20. Gardner WJ. Cross talk: the paradoxical transmission of a nerve impulse. Arch Neurol.
1966;14:149-156. 21. Wartenberg R. Hemifacial Spasm: A Clinical and Pathophysiological Study. New York, NY: Oxford University Press Inc; 1952. 22. Ferguson JH. Hemifacial spasm and the facial nucleus. Ann Neurol. 1978;4:97-103.
