Clinical rheurnatology,
1992, 11, N ~ 3
405-407
Case Report
Vestibular Syndrome in Multiple Myeloma Role of Magnetic Resonance Imaging X. C H E V A L I E R ,
N. N. W R O N A ,
B. L A R G E T - P I E T
Summary
The authors report a case of multiple myeloma involving the temporal bone and responsible for a vestibular syndrome associated with hypoacusis. The lesion was best visualised by magnetic resonance imaging.
Key words Multiple Myeloma. Cranial Nerve Paralysis. Magnetic Resonance Imaging.
INTRODUCTION Involvement of the bones of the base of the skull by multiple myeloma has been rarely reported (1). This unusual site may be responsible for cranial nerve compression resulting in various neurological syndromes (2-4). We report the case of a 62-year-old woman, suffering from a secreting multiple myeloma for several years, who presented with a peripheral vestibular syndrome due to a lesion in the petrous pyramid.
rum calcium and creatinine levels were normal. Skeletal X-rays did not reveal any new lesions (spine, long bones, pelvis, skull). Although X-rays centered on the petrous temporal bones were considered to be normal, plain computed tomography revealed a low-density lesion of the apex of the petrous temporal bone. Magnetic resonance imaging provided better visualisation of this lesion which appeared to occupy the petrous bone while sparing the carotid artery and venous sinuses. It presented a low signal intensity on Tl-weighted sequences and a
CASE R E P O R T A 62-year-old female patient was admitted to hospital with recent onset of true vertigo. She had been followed up for 6 years for an IgG ~ multiple myeloma classified as stage III with multiple bone involvement (spine, calvarium, long bones) and an IgG peak of 10 g/l, assayed one month prior to admission to hospital. She had been treated by VAD combination chemotherapy (vincristine, dexamethasone, adramycin). Her rotational vertigo was easily triggered by changes in position and was accompanied by slight left hypoacusis. Clinical examination revealed a harmonious peripheral vestibular syndrome with past pointing and nystagrnus on the left side. The rest of the neurological examination was normal. The audiovestibular examination confirmed the peripheral nature of the vestibular syndrome. The patient presented an obvious tumour relapse with a rise in the monoclonal peak to 20 g/l, haemoglobin of 11 g/100 m! and 35% plasma cell infiltration of the bone marrow. The se1 : Magnetic resonance imaging, transverse scan, postgadolinium Tl-weighted sequences through the petrous temporal bone: high signal intensity over the petrous pyramid.
Fig.
Service de Rhumatologie,H6pital Henri-Mondor, Cr6teil, France.
406
X. Chevalier, N. Wrona, B. Larget-Piet
high signal intensity on T2-weighted sequences and after injection of gadolinium (Fig. 1). This lesion explained the retrocochlear vestibular involvement by compression of the VIIIth cranial nerve during its course through the petrous bone. The patient was treated by local radiotherapy with ocular protection and a new course of VAD chemotherapy. After 3 months of treatment, the vertigo had resolved, but the multiple myeloma had progressed with the appearance of new spinal and pelvic lesions. DISCUSSION Involvement of the cranial nerves is a rare complication of multiple myeloma. It is even more exceptional for such a lesion to be presenting sign of a multiple myeloma (2,5,6). It constitutes the least frequent neurological involvement after nerve root and spinal cord lesions and neuropathies of the lower limbs. Other plasma cell proliferations such as solitary extraosseous plasmacytomas of the upper airways may also induce paralysis of the cranial nerves by local extension to the base of the skull (6). Cranial nerve paralyses, secondary to multiple myeloma, may be due to a variety of mechanisms. Exceptionally, cranial nerve lesions may be due to disseminated specific meningitis (7), but they are more often due to local compression by a plasma cell tumour invading one of the bones of the base of the skull (6), as in our case. In comparison with the frequency of involvement of the calvarium, lesions of the base of the skull are rare in multiple myeloma (1), but when they occur, they generally involve the sphenoid and the petrous temporal bones (1). These two sites are responsible for paralysis of the abducens nerve (4) and neurological syndromes due to compression of cranial nerves at the cerebellopontine angle, such as the VIIth and VIIIth nerves (2,3,5). When the tumour is located in the petrous temporal bone, it may extend towards the middle ear, where it may be visible on otoscopy, as reported by Shone (3). A case presenting with
Collet-Sicard syndrome simulated a jugular glomus tumour (5), while another case presented with a XIIth cranial nerve lesion (8). Our case illustrates the value of computed tomography and magnetic resonance imaging in the investigation of neurological complications of multiple myeloma. X-rays of the base of the skulk including views revealing the petrous temporal bones, are not always easy to interpret and the presence of temporal osteolysis may be due to a number of aetiologies (9). Valvasorri et al. clearly demonstrated the value of MRI in relation to CT-scan in the assessment of retrocochlear vestibular lesions (10). The MRI appearance of bone lesions of multiple myeloma has already been reported, but, to our knowledge, this constitutes the first report of a lesion of the base of the skull studied by MRI. MRI has a very high level of sensitivity for detecting malignant lesions of the bone marrow (11). Under normal conditions, the bone marrow has a high signal intensity on T1weighted sequences, because of its fat content. Lesions affecting cancellus bone can be detected by a reduction in the signal intensity, as in our case. When a bone lesion appears with an increase uptake on bone scan, MRI is able to differentiate between malignant and benign lesions (11). In our case, the lesion presented a low signal intensity on Tl-weighted sequences, which was enhanced by injection of gadolinium, and a high signal intensity on T2-weighted sequences. MRI allows better visualisation of the relations between the neoplastic lesion and the adjacent vessels. It also provides a much better assessment of the extent of the lesion than plain X-rays, particularly in an anatomical region which is difficult to examine, such as the base of the skull. The improvement observed in response to radiotherapy corroborates the results of other cases reported in the literature (2,3), but is was nevertheless incomplete (6). Chemotherapy is frequently also required due to the presence of other bone lesions.
REFERENCES
1. 2. 3. 4.
Clark, E. Cranial and intra-cranial myeloma. Brain 1954, 77, 6181. Marks, P.V., Bookes, G.B. Myelomatosis presenting as an isolated lesion of the mastoid. J Laryngol Otology 1985, 99, 903-906. Shone, G.R. Facial palsy due to myeloma of the temporal bone. J Laryngol Oto 1985, 99, 907-908. Hugon, J., Dibon, J.M., Treves, R., Desproges-Gouteron, R., Gualde, N. Paralysie de deux nerfs moteurs oculaires externes au cours d'un my4lome non excretant. Rev Rhum Mal Osteoartic 1981, 48, 286-287.
5.
6.
7.
8.
Ennouri, A., Malla, M., Hajri, H., Ben-Ayed, H., Marreckchi, H. Atteintes des paires craniennes rdvdlatrices de la maladie de Khaler. Ann Otolaryngol Chir Cerviofac 1990, 107, 141-143. Alexander, M.P., Goodkin, D.E., Poser, C.M. Solitary plasmacytoma producing cranial neuropathy. Arch Neurol !975, 32, 777778. Korine, A., Solal-Celigny, P., Kuentz, N., Farcet, J.P., Ctauvel, J.P. Atteinte mening6e spdcifique au cours du my61ome multiple. Nouv Press Med 1985, 14, 733-736. Lavine, F.R., Matucci, K.F., Merten, C.W. Multiple myeloma of the temporal bone. E N T J 1979, 58, 210-213.
Vestibular syndrome in multiple myelomang
Livingstone, P.A. Differential diagnosis of radiolucent lesion of the temporal bone. Radiol Clin North Am, 1974, 3, 571-583. 10. Valvassori, G.E. Diagnostic of retrocochlear and central vestibular disease by magnetic resonance imaging. Ann Otol Rhino Laryngol 1988, 97, 19-22. 11. Daffner, R.H., Lupentin, A.R., Dash, N., Deeb, Z.L., Sefzeck, R.J., Schapiro, R.L. MRI in the detection of malignant infiltration of bone marrow. Am J Roentgenol 1986, 146, 353-358.
407
9.
Received: 3 June 1991; Revision-accepted: 30 October 1991 Correspondence to: Dr Xavier CHEVALIER CCA, Service de Rhumatologie, HOpital Henri-Mondor, Bd de Lattre de Tassigny, 94010 Crdteil, France.
Book review L'ACTUALITE R H U M A T O L O G I Q U E 1991 P R E S E N T E E AU PRATICIEN.
Les m6decins du Centre Viggo Petersen et de la Clinique Rhumatologique de I'H6pital Bichat, sous la direction de S. de S/~ze, A. Ryckewaert, M.-F. Kahn, D. Kuntz, A. Dryll, Cl. Gu6rin. 1991, pp 440, FF 430, 18 x 23 cm, Expansion Scientifique Fram;aise, For the 28th time the yearly volume of "L'actualit6 rhumatologique" comes off the press. It is a custom to bring together an updating of a number of topics of interest for rheumatologists in the field. The list of the topics is too long to be reproduced here, but the following broad chapters are dealt with : 1. Rhumatismes inflammatoires et connectivites.
2. Pathologie ost6o-articulaire, neurologique et vert6brale. 3. M6tabolisme phosphocalcique. 4. Moyens d'exploration. 5. Biologie et physiopathologie. 6. Th6rapeutiques m6dicales et leurs complications. 7. Chirurgie du rhumatisme. The papers are critical overviews of the literature with extensive references and many illustrations. This yearbook is to be r e c o m m e n d e d to all rheumatologists adept in the French language. It will give them new insights in rheumatology and will also serve as a reference book for consultation when special problems occur. J. D e q u e k e r Leuven - Belgium
1992, 11, N ~ 3
405-407
Case Report
Vestibular Syndrome in Multiple Myeloma Role of Magnetic Resonance Imaging X. C H E V A L I E R ,
N. N. W R O N A ,
B. L A R G E T - P I E T
Summary
The authors report a case of multiple myeloma involving the temporal bone and responsible for a vestibular syndrome associated with hypoacusis. The lesion was best visualised by magnetic resonance imaging.
Key words Multiple Myeloma. Cranial Nerve Paralysis. Magnetic Resonance Imaging.
INTRODUCTION Involvement of the bones of the base of the skull by multiple myeloma has been rarely reported (1). This unusual site may be responsible for cranial nerve compression resulting in various neurological syndromes (2-4). We report the case of a 62-year-old woman, suffering from a secreting multiple myeloma for several years, who presented with a peripheral vestibular syndrome due to a lesion in the petrous pyramid.
rum calcium and creatinine levels were normal. Skeletal X-rays did not reveal any new lesions (spine, long bones, pelvis, skull). Although X-rays centered on the petrous temporal bones were considered to be normal, plain computed tomography revealed a low-density lesion of the apex of the petrous temporal bone. Magnetic resonance imaging provided better visualisation of this lesion which appeared to occupy the petrous bone while sparing the carotid artery and venous sinuses. It presented a low signal intensity on Tl-weighted sequences and a
CASE R E P O R T A 62-year-old female patient was admitted to hospital with recent onset of true vertigo. She had been followed up for 6 years for an IgG ~ multiple myeloma classified as stage III with multiple bone involvement (spine, calvarium, long bones) and an IgG peak of 10 g/l, assayed one month prior to admission to hospital. She had been treated by VAD combination chemotherapy (vincristine, dexamethasone, adramycin). Her rotational vertigo was easily triggered by changes in position and was accompanied by slight left hypoacusis. Clinical examination revealed a harmonious peripheral vestibular syndrome with past pointing and nystagrnus on the left side. The rest of the neurological examination was normal. The audiovestibular examination confirmed the peripheral nature of the vestibular syndrome. The patient presented an obvious tumour relapse with a rise in the monoclonal peak to 20 g/l, haemoglobin of 11 g/100 m! and 35% plasma cell infiltration of the bone marrow. The se1 : Magnetic resonance imaging, transverse scan, postgadolinium Tl-weighted sequences through the petrous temporal bone: high signal intensity over the petrous pyramid.
Fig.
Service de Rhumatologie,H6pital Henri-Mondor, Cr6teil, France.
406
X. Chevalier, N. Wrona, B. Larget-Piet
high signal intensity on T2-weighted sequences and after injection of gadolinium (Fig. 1). This lesion explained the retrocochlear vestibular involvement by compression of the VIIIth cranial nerve during its course through the petrous bone. The patient was treated by local radiotherapy with ocular protection and a new course of VAD chemotherapy. After 3 months of treatment, the vertigo had resolved, but the multiple myeloma had progressed with the appearance of new spinal and pelvic lesions. DISCUSSION Involvement of the cranial nerves is a rare complication of multiple myeloma. It is even more exceptional for such a lesion to be presenting sign of a multiple myeloma (2,5,6). It constitutes the least frequent neurological involvement after nerve root and spinal cord lesions and neuropathies of the lower limbs. Other plasma cell proliferations such as solitary extraosseous plasmacytomas of the upper airways may also induce paralysis of the cranial nerves by local extension to the base of the skull (6). Cranial nerve paralyses, secondary to multiple myeloma, may be due to a variety of mechanisms. Exceptionally, cranial nerve lesions may be due to disseminated specific meningitis (7), but they are more often due to local compression by a plasma cell tumour invading one of the bones of the base of the skull (6), as in our case. In comparison with the frequency of involvement of the calvarium, lesions of the base of the skull are rare in multiple myeloma (1), but when they occur, they generally involve the sphenoid and the petrous temporal bones (1). These two sites are responsible for paralysis of the abducens nerve (4) and neurological syndromes due to compression of cranial nerves at the cerebellopontine angle, such as the VIIth and VIIIth nerves (2,3,5). When the tumour is located in the petrous temporal bone, it may extend towards the middle ear, where it may be visible on otoscopy, as reported by Shone (3). A case presenting with
Collet-Sicard syndrome simulated a jugular glomus tumour (5), while another case presented with a XIIth cranial nerve lesion (8). Our case illustrates the value of computed tomography and magnetic resonance imaging in the investigation of neurological complications of multiple myeloma. X-rays of the base of the skulk including views revealing the petrous temporal bones, are not always easy to interpret and the presence of temporal osteolysis may be due to a number of aetiologies (9). Valvasorri et al. clearly demonstrated the value of MRI in relation to CT-scan in the assessment of retrocochlear vestibular lesions (10). The MRI appearance of bone lesions of multiple myeloma has already been reported, but, to our knowledge, this constitutes the first report of a lesion of the base of the skull studied by MRI. MRI has a very high level of sensitivity for detecting malignant lesions of the bone marrow (11). Under normal conditions, the bone marrow has a high signal intensity on T1weighted sequences, because of its fat content. Lesions affecting cancellus bone can be detected by a reduction in the signal intensity, as in our case. When a bone lesion appears with an increase uptake on bone scan, MRI is able to differentiate between malignant and benign lesions (11). In our case, the lesion presented a low signal intensity on Tl-weighted sequences, which was enhanced by injection of gadolinium, and a high signal intensity on T2-weighted sequences. MRI allows better visualisation of the relations between the neoplastic lesion and the adjacent vessels. It also provides a much better assessment of the extent of the lesion than plain X-rays, particularly in an anatomical region which is difficult to examine, such as the base of the skull. The improvement observed in response to radiotherapy corroborates the results of other cases reported in the literature (2,3), but is was nevertheless incomplete (6). Chemotherapy is frequently also required due to the presence of other bone lesions.
REFERENCES
1. 2. 3. 4.
Clark, E. Cranial and intra-cranial myeloma. Brain 1954, 77, 6181. Marks, P.V., Bookes, G.B. Myelomatosis presenting as an isolated lesion of the mastoid. J Laryngol Otology 1985, 99, 903-906. Shone, G.R. Facial palsy due to myeloma of the temporal bone. J Laryngol Oto 1985, 99, 907-908. Hugon, J., Dibon, J.M., Treves, R., Desproges-Gouteron, R., Gualde, N. Paralysie de deux nerfs moteurs oculaires externes au cours d'un my4lome non excretant. Rev Rhum Mal Osteoartic 1981, 48, 286-287.
5.
6.
7.
8.
Ennouri, A., Malla, M., Hajri, H., Ben-Ayed, H., Marreckchi, H. Atteintes des paires craniennes rdvdlatrices de la maladie de Khaler. Ann Otolaryngol Chir Cerviofac 1990, 107, 141-143. Alexander, M.P., Goodkin, D.E., Poser, C.M. Solitary plasmacytoma producing cranial neuropathy. Arch Neurol !975, 32, 777778. Korine, A., Solal-Celigny, P., Kuentz, N., Farcet, J.P., Ctauvel, J.P. Atteinte mening6e spdcifique au cours du my61ome multiple. Nouv Press Med 1985, 14, 733-736. Lavine, F.R., Matucci, K.F., Merten, C.W. Multiple myeloma of the temporal bone. E N T J 1979, 58, 210-213.
Vestibular syndrome in multiple myelomang
Livingstone, P.A. Differential diagnosis of radiolucent lesion of the temporal bone. Radiol Clin North Am, 1974, 3, 571-583. 10. Valvassori, G.E. Diagnostic of retrocochlear and central vestibular disease by magnetic resonance imaging. Ann Otol Rhino Laryngol 1988, 97, 19-22. 11. Daffner, R.H., Lupentin, A.R., Dash, N., Deeb, Z.L., Sefzeck, R.J., Schapiro, R.L. MRI in the detection of malignant infiltration of bone marrow. Am J Roentgenol 1986, 146, 353-358.
407
9.
Received: 3 June 1991; Revision-accepted: 30 October 1991 Correspondence to: Dr Xavier CHEVALIER CCA, Service de Rhumatologie, HOpital Henri-Mondor, Bd de Lattre de Tassigny, 94010 Crdteil, France.
Book review L'ACTUALITE R H U M A T O L O G I Q U E 1991 P R E S E N T E E AU PRATICIEN.
Les m6decins du Centre Viggo Petersen et de la Clinique Rhumatologique de I'H6pital Bichat, sous la direction de S. de S/~ze, A. Ryckewaert, M.-F. Kahn, D. Kuntz, A. Dryll, Cl. Gu6rin. 1991, pp 440, FF 430, 18 x 23 cm, Expansion Scientifique Fram;aise, For the 28th time the yearly volume of "L'actualit6 rhumatologique" comes off the press. It is a custom to bring together an updating of a number of topics of interest for rheumatologists in the field. The list of the topics is too long to be reproduced here, but the following broad chapters are dealt with : 1. Rhumatismes inflammatoires et connectivites.
2. Pathologie ost6o-articulaire, neurologique et vert6brale. 3. M6tabolisme phosphocalcique. 4. Moyens d'exploration. 5. Biologie et physiopathologie. 6. Th6rapeutiques m6dicales et leurs complications. 7. Chirurgie du rhumatisme. The papers are critical overviews of the literature with extensive references and many illustrations. This yearbook is to be r e c o m m e n d e d to all rheumatologists adept in the French language. It will give them new insights in rheumatology and will also serve as a reference book for consultation when special problems occur. J. D e q u e k e r Leuven - Belgium
