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An unusual cause of hemifacial spasm
Hemifacial spasm
A n u n u s u a l c a u s e of hemifacial spasm Michelle A. Kiley 1 MB BS, Frank Voyvodic 2 MB BS FRACR, Richard J. Burns 1 FRACP FRCP (LOND) Department of Neurology 2Department of Radiology, Flinders Medical Centre, Bedford Park 5042, South Australia
Summary Hemifacial spasm (HFS) is a movement disorder characterized by involuntary paroxysmal chronic contractions of the facial musculature. The usual cause is vascular compression of the seventh cranial nerve, at its exit zone from the brain stem. We report a case of left hemifacial spasm, in a 66-year-old woman, in which the neuroradiological investigation with magnetic resonance imaging showed a hypervascular soft tissue mass arising from the left skull base, in the jugular foramen. This lesion was thought highly likely to be a glomus jugulare tumour. While the usual occurrence of tumour compression causing HFS has been previously recognized, the association of glomus jugulare tumours presenting with HFS has not. The importance of this association is discussed. Journal of Clinical Neuroscience (1999) 6(4), 349~351 © 1999 Harcourt Brace & Co. Ltd
Keywords: hemifacial spasm, management, glomus jugulare tumour Received 23 April 1997 Accepted 15 August 1997 Correspondence to: M. A. Kiley, Tel.: +61 8 8204 4187; Fax: +61 8 8204 5931
INTRODUCTION
Hemifacial spasm is a movement disorder characterized by involuntary paroxysmal clonic contractions of the facial musculature.l,2 The process usually begins in the orbicularis oculi and spreads to involve other facial muscles, including the platysma, on the same side of the face. It may be induced or aggravated by voluntary movements of the face. 2 Hemifacial spasm (HFS) is generally a disease of the middle aged and older populations, and affects women more than men. 2
Fig. 1 Post i.v. contrast (gadolinium-DTPA) T< weighted axial MRI study demonstrating an enhancing soft tissue mass involving the left jugular foramen and adjacent temporal bone (arrow).
© 1999 Harcourt Brace & Co. Ltd
349
For many years, HFS was regarded as idiopathic, however, it is now recognized that vascular compression of the seventh cranial nerve, at its exit zone from the brain stem, is the primary cause2 In most cases this is due to an aberrant or tortuous vessel, less commonly it may be seen with basilar artery aneurysms or arteriovenous malformations. 2,3 A rare, but recognized, cause of HFS is tumour compression; it has been reported in patients with acoustic neuromas, meningiomas and cholesteotomas 1,3,4as well as epidermold tumours s and lipomas. 6 We report a very unusual cause of HFS, a glomus jugulare tumour. As far as we are aware, the association of glomus jugulare tumours with HFS has not been previously reported in the English literature.
CASE REPORT
A 66-year-old woman, in otherwise in good health, presented with a 10 month history of left HFS. She described, brief painless episodes of muscle twitching around her left eye which, over the ensuing months, became more severe, causing complete eye closure, with involvement of the lower facial musculature also. By the time of presentation she was experiencing several episodes daily. She complained of no other symptoms and of particular note there was no facial numbness, hearing deficit, tinnitus, vertigo or speech or swallowing difficulty. Neurological examination failed to reveal any abnormality. There were no palpable neck masses or mastoid bruits. A magnetic resonance imaging (MRI) head scan was performed and revealed a small (2 cm) poorly defined hypervascular soft tissue mass at the left skull base centered on the lateral aspect of the jugular foramen with focal involvement of the jugular vein (Fig. 1). There was no evidence of abnormality in the cerebellopontine angle cistern or internal auditory canal (Fig. 2). A computed tomography (CT) head scan demonstrated permeative bone destruction in the adjacent temporal bone extending to involve the facial canal containing the descending portion of the facial nerve (Fig. 3). There was no involvement of the middle or inner ear. The diagnosis of a small paraganglioma was suggested. In view of the radiology, catecholamine and serotonin metabolities (VMA and HIAA respectively) were screened for. Because of the size and position of the tumour, it was decided to treat her with radiotherapy rather than attempt neurosurgical decompression and/or removal.
Fig. 2 Tzweighted axial MRI study demonstrating normal cerebel]opontine angle cisterns and internal auditory canals (arrow). The vessel visible adjacent to the left middle cerebral peduncle was not in contact with the seventh cranial nerve.
Fig. 3 Axial CT scan presented on bony settings illustrates permeative destruction of the temporal bone with involvement of the facial canal (arrow).
Journal of Clinical Neuroscience (1999) 6(4)
350
Payer et aL
DISCUSSION In a high percentage of cases, HFS is caused by vascular compression of the seventh cranial nerve as it exits the brain stem. This has been demonstrated both radiologically and surgicallyY '8 Jannetta et al. have stated that the vascular compression must be crosscompression at right angles to the nerve, at its root exit zone from the brain stem3; this is the point where central myelin is replaced by peripheral myelin and this may be important in the pathogenesis. 3 It has been postulated that compression of the nerve causes focal demyelination and demyelinated axons can then activate adjacent nerve fibres by ephaptic transmission producing hemifacial spasm? Scoville, however, has reported a case successfully treated by removing a peripheral artery compressing the facial nerve, in the cerebello-pontine angle? Nagata et al. have reported four cases in which a tumour was thought responsible for HFS. No artery was visualized compressing the nerve at the root exit zone, and in three of the four cases, the HFS disappeared after the tumour, which was in contact with the facial nerve, was removed. 4 In our case, both the CT and MRI head scans showed the tumour mass, thought highly likely to be a glomus jugulare tumour, involving the descending portion of the seventh cranial nerve. No vascular compression of the seventh nerve at its root exit zone was seen. We feel, therefore, that this is the most likely cause of our patient's HFS. Glomus jugulare tumours, otherwise termed jugulotympanic paragangliomas, are very uncommon neoplasms, accounting for only 0,6% of all head and neck tumours? ° They are, however, the second commonest turnout to involve the temporal bone al and are the most common turnout in the jugular foramen. These neoplasms arise from the extra-adrenal paraganglia located in the jugular bulb within the temporal bone at the jugular foramenJ 2 Despite locally aggressive behaviour, metastases are rare./2 There is often a long interval between first symptoms and diagnosis, as they tend to be indolent, slow growing tumours. 12-14 The usual presentation of glomus jugulare tumours is otologic. ~ Lower cranial nerve lesions are seen in about one third of cases, n, ~5 Involvement of the seventh cranial nerve has been described ~,~2,15 and was documented in 12 of 57 cases of glomus jugulare tumours reported by Spector et al.l~ All cases, however, report facial paralysis, and none causing HFS have been reported, as far as we are aware. The gross appearance of these tumours is of an encapsulated, lobulated mass which is purple-red in colour. They have a haemorrhagic appearance, spongy texture and bleed readily. They are composed of nests of polyclonal chief cells, and mitoses are few./5 They can be quite aggressive locally, with intracranial extension, most frequently into the posterior cranial fossa, For tumours greater than 2 cm in diameter MRI demonstrates the characteristic 'salt and pepper' appearance of glomus jugulare tumours due to a combination of signal voids from flowing blood ('black') and loci of subacute haemorrhage .()white') on T2-weighted pre-contrast scans./6 MRI also demonstrates the extension of the tumour into the cranial fossa and vascular invasion directly (without contrast)J 7 After contrast injection there is marked enhancement of the lesion, reflecting its highly vascular nature./7 High resolution CT illustrates bone destruction and intracranial extension very well./7 Our case demonstrates the typical ill defined margin and intense contrast enhancement. The mass showed heterogenous signal intensity but did not show the diagnostic 'salt and pepper' appearance presumably due to its smaller size. The radiological differential diagnosis of this lesion includes nerve sheath tumours, meningiomas and malignant tumours (carcinomas, metastases). TM Nerve sheath tumours tend to produce smooth scalloped enlargement of the foramen and are
Journal of Clinical Neuroscience (1999) 6(4)
homogeneous or cystic, Meningiomas rarely arise in the temporal bone and show some of the behavioural patterns of paragangliomas with local bone invasion occurring at the bone margins but tend to be better defined; they tend to be homogeneous and lack the 'salt and pepper' pattern. Carcinoma, metastases and myeloma can all destroy the jugular fossa but most are much less vascular and show much less contrast enhancement and also have a typically homogeneous appearance on MRI. In addition, there was no history of primary malignancy in this case. HFS is sometimes managed non-surgically, especially in milder cases and older patients. Carbamazepine and botulinum toxin injections are two medical treatments which can be employed? In such cases, imaging studies are not routinely obtained. Cranial nerve involvement is often a late manifestation of glomus tumours, 15 when surgical treatment is likely to have a high morbidity? 9 The best clinical practice guidelines for the management of HFS are not clearly stated in most neurological texts. 2,2°,21 A non-invasive investigation such as magnetic resonance angiography would seem reasonable for those cases thought to be due to neurovascular compression, in whom surgical decompression is planned. Despite the low incidence of compressive lesions (other than vascular) we believe that all persons with HFS should have an MRI scan. If the first manifestation of a mass lesion can be HFS then identification of such lesions early is clearly very important.
SUMMARY We report a very unusual cause of HFS, a glomus jugulare tumour, in a patient with no other symptoms or signs. Because of such cases we feel MRI of the brain stem is a reasonable investigation for all patients presenting with HFS.
REFERENCES 1. MaroonJC Hemifacialspasma vascularcause. Archivesof Neurology 1978; 35: 481483. 2. AdamsRD, VictorM, Ropper AH. Principlesof Neurology. 6th ed. New York: McGrawHill, 1997; 1378-1379. 3. JarmettaPJ, AbbanyM, MaroonJC, Ramos FM, Albin MS. Etiologyand definitivemicrosurgicaltreatmentof hemifacialspasm. J Neurosurgery 1977; 47: 321-328. 4. NagataS, MatsushimaT, Fujii K, Fuldn M, KuromatsuC. Hemifacialspasm due to tumour, aneurysmor arteriovenousmalformation.Surg Neurol 1992;38: 204-209. 5. SinghA., Join VK, ChhabraDV, Hong K, KobayashiS. Hemifacialspasmand cerebellopontineangle epidermoid:case report and review.NeurolRes 1994; 16: 321-323. 6. InoueT, MaeyamaR, Ogawa H. Hemifacialspasmresultingfrom a cerebellopontine anglelipoma: case report. Neurosurgery 1995; 36: 846-848. 7. JespersonJH, DupontE, GelineckJ, LtmdorfE. Hemifacialspasm: magnetic resonanceangiography.Acta NeurolStand 1996;93: 35-38. 8. AdlesCH, ZimmermannRA, SavinoPJ, Bemadi B, BosleyTM, SergottRC. Hemifacial spasm: evaluationby magneticresonanceimagingand magnetic resonancetomographicangiography.AnnalNeurol 1992; 32: 502-506. 9. ScovilleWB. Hearingloss followingexplorationof the cerebellopontineangle in the treatmentof hemifacialspasm. J Neurosurg 1969; 31: 4749. 10. ReddyMU, MansfieldLM, HartmanGV. Chemodectomasof the glomus jugulare. Cancer 1983; 52: 337-340. 11. SpectorGJ, GadoM, CiralskyR, Ogura J, MaiseIRH. Neurological implicationsof glomus tumoursin the head and neck. Laryngoscope1975; 85: 1387-1395. 12. JohnstonePAS,Foss RD, DesiletsDJ. Malignantjugulotyrnpanic paraganglioma.Arch Path Lab Med 1990; 114:976-979. 13. GlassockME. The history of glomus tumours:a personalperspective. Laryngoscope 1993; 103: 3-6. 14. BoyleJO, ShimmOS, CoulthardSW. Radiationtherapyfor paragangliomaof the temporalbone. Laryngoscope1990; 100: 896-901. 15. Juliana-GulyaA. The glomustumanr and its biology.Laryngoscope 1993; 103: 7-15.
© 1999 Harcourt Brace & Co. Ltd
Hemifacial spasm~Pituitary abscess
16. OlsenLW, DillonPW, KellyMW. MR imagingof paragangliomas.AJ Roentgerology1987; 148: 201504. 17. AthanassopoulouAC, VlahosLL, GouliamosAD, KallidouED, PapailiouJG, AngeliSS. MRI featuresin a malignantglomusjugulare tumour. The Journal of Laryngologyand Otology 1993; 197:1066 1067. 18. Lo WWM,Solti-BohmanLG. Tumoursof the temporalbone and cerebellopontine angle, In: Headand Neck Imaging,3rd ed. Sore P M, CurtinHD (Eds.) New York:MosbyPress, 1996; 1484-1496. 19. BrammerRE, GrahamMD, KemickJL. Glomustumours of the temporalbone: contemporary evaluationand therapy. OtolaryngolClinicNorthAmerica1984; 17:499 512. 20. JmmettaPJ. Surgicalapproachto hemifacialspasm: microvascular decompression.In: MovementDisorders,Neurology2. C Marsden, S Fahn (Eds). Butterworth-Heinemann,1982, 330-333. 21. BorodicGE. Hemifacialspasm: Evaluationand Management,with emphasis on botulinumtoxintherapy. In: Therapywith BotulinumToxin. J Jankovic,M Hallett (Eds). New York:MarcelDekker, 1994; 331-351.
Pituitary abscess Christine S. Hon 1, Neville K n u c k e y 1 FRACS, Peter Robbins 2 FRCPA, Peter T. Pullan 3 FRACP ~Department of Neurosurgery, The Queen Elizabeth II Medical Centre, Verdun Street, Nedlands, WA, 6009, Australia ~Division of Tissue Pathology, Western Australian Centre for Pathology and Medical Research, Locked Bag 2009, P.O. Nedlands, 6009, Australia "Department of Endocrinology and Diabetes, The Queen Elizabeth fl Medical Centre, Verdun Street, Nedlands, WA, 6009, Australia
S u m m a r y A 28-year-old Caucasian woman presented with a 12 month history of secondary amenorrhoea, polyuria and polydipsia with fatigue and weight loss. Investigations revealed panhypopituitarism, diabetes insipidus, an intrasellar mass and papilloedema, thought to be due to benign intracranial hypertension. She was treated conservatively. However, a repeat magnetic resonance image showed enlargement of the pituitary mass with compression of the optic nerves. The pituitary abscess was drained by a transsphenoidal approach. Postoperatively the patient received antibiotics with no recurrence of the pituitary abscess. Journalof ClinicalNeuroscience(1999)6(4), 351-353© 1999HarcourtBrace& Co. Ltd
351
We review the literature on pituitary abscess, highlight the importance of diabetes insipidus as an important clinical sign and demonstrate the magnetic resonance imaging (MRI) features of this condition. CASE REPORT
The patient is a 28-year-old Caucasian woman who initially presented with a 12 month history of secondary amenorrhoea, polyuria and polydipsia with fatigue and weight loss. She also complained of 4 months, intermittent headache and an episode of blurred vision and diplopia. She had no history of meningitis, rhinorrhoea or systemic sepsis. On examination, she was morbidly obese, her pubic hair was slightly sparse, but she showed no other clinical evidence of hypopituitarism. Clinical examination showed no focal neurological deficit. Visual acuities and visual fields were normal, but the optic fundi showed papilloedema. Initial investigations including blood film, erythrocyte sedimentation rate, serum glucose, calcium, creatinine, electrolytes and liver function tests were normal. Endocrinological investigations revealed that she had panhypopituitarism. Serum oestradiol was 82 pmol/1 (normal: 100-450pmol/1), FSH was 2u/1 (normal: 2-8 u/l) and LH was 1 u/l (normal: 2-13 u/l). The serum free thyroxine was 7pmol/1 (normal: l l - 1 9 p m o l / l ) with TSH 0.30 mud (normal: 0.34-4.80 muff). Following synthetic ACTH stimulation, plasma cortisol peaked at 449 nmolB (normal greater than 550 nmol/l). The serum growth hormone was less than 1 mu/L (normal: <4 muff) and IGF-1 60 ug/1 (normal: 144-360 ug/1). Serum prolactin was mildly elevated at 688 mull (normal: <425 mu/l). A spot plasma osmolality was towards the upper limit of normal at 290 mmol/kg (normal: 275-295 mmol/kg) with an inappropriately low urine osmolality of 190 mmol/kg confirming the clinical diagnosis of diabetes insipidus. Plain lateral skull X-ray demonstrated a slightly enlarged sella. Computerized tomography (CT) scan showed a cystic sellar mass. The initial MRI scan showed a hypointense intrasellar mass on T2-weighted image (Fig. l). The visual evoked potentials were normal. The initial clinical diagnosis was panhypopituitarism due to an intrasellar mass and benign intracranial hypertension. She was treated conservatively with hormone replacement with cortisone acetate (25 mg mane and 12.5 mg nocte), thyroxine (100ug mane) and desmopressin (10 ug intranasally nocte). The papilloedema resolved. However, a repeat MRI showed enlargement of
Keywords: pituitary abscess, diabetes insipidus, hypopituitarism Received 26 May 1997 Accepted 26 June 1997 Correspondence to: N. Knuckey, Tel.: +61 9 346 2865; Fax: +61 9 346 3824; E-mail; [email protected]
INTRODUCTION
Pituitary abscess is rare and approximately 60 cases have been reported in the medical literature. Since pituitary abscess is potentially fatal, early clinical diagnosis is critical. However, the clinical diagnosis is difficult because there are no clinical symptoms or signs characteristic of pituitary abscess and it is frequently indistinguishable clinically from other pituitary lesions. Conservative medical treatment with endocrine replacement therapy or antibiotics has a high mortality. Hence, the treatment of choice is drainage of the pituitary abscess via transsphenoidal surgery and the administration of appropriate antibiotics and endocrine replacement therapy. Since pituitary abscess is rare and potentially fatal if not treated, we report a case to highlight its important clinical and diagnostic features.
© 1999 Harcourt Brace & Co. Ltd
Fig. 1 A T2-weighted MRI of the pituitary fossa showing an enlarged hypointense pituitary lesion.
Journal of Clinical Neuroscience (1999) 6(4)
A n u n u s u a l c a u s e of hemifacial spasm Michelle A. Kiley 1 MB BS, Frank Voyvodic 2 MB BS FRACR, Richard J. Burns 1 FRACP FRCP (LOND) Department of Neurology 2Department of Radiology, Flinders Medical Centre, Bedford Park 5042, South Australia
Summary Hemifacial spasm (HFS) is a movement disorder characterized by involuntary paroxysmal chronic contractions of the facial musculature. The usual cause is vascular compression of the seventh cranial nerve, at its exit zone from the brain stem. We report a case of left hemifacial spasm, in a 66-year-old woman, in which the neuroradiological investigation with magnetic resonance imaging showed a hypervascular soft tissue mass arising from the left skull base, in the jugular foramen. This lesion was thought highly likely to be a glomus jugulare tumour. While the usual occurrence of tumour compression causing HFS has been previously recognized, the association of glomus jugulare tumours presenting with HFS has not. The importance of this association is discussed. Journal of Clinical Neuroscience (1999) 6(4), 349~351 © 1999 Harcourt Brace & Co. Ltd
Keywords: hemifacial spasm, management, glomus jugulare tumour Received 23 April 1997 Accepted 15 August 1997 Correspondence to: M. A. Kiley, Tel.: +61 8 8204 4187; Fax: +61 8 8204 5931
INTRODUCTION
Hemifacial spasm is a movement disorder characterized by involuntary paroxysmal clonic contractions of the facial musculature.l,2 The process usually begins in the orbicularis oculi and spreads to involve other facial muscles, including the platysma, on the same side of the face. It may be induced or aggravated by voluntary movements of the face. 2 Hemifacial spasm (HFS) is generally a disease of the middle aged and older populations, and affects women more than men. 2
Fig. 1 Post i.v. contrast (gadolinium-DTPA) T< weighted axial MRI study demonstrating an enhancing soft tissue mass involving the left jugular foramen and adjacent temporal bone (arrow).
© 1999 Harcourt Brace & Co. Ltd
349
For many years, HFS was regarded as idiopathic, however, it is now recognized that vascular compression of the seventh cranial nerve, at its exit zone from the brain stem, is the primary cause2 In most cases this is due to an aberrant or tortuous vessel, less commonly it may be seen with basilar artery aneurysms or arteriovenous malformations. 2,3 A rare, but recognized, cause of HFS is tumour compression; it has been reported in patients with acoustic neuromas, meningiomas and cholesteotomas 1,3,4as well as epidermold tumours s and lipomas. 6 We report a very unusual cause of HFS, a glomus jugulare tumour. As far as we are aware, the association of glomus jugulare tumours with HFS has not been previously reported in the English literature.
CASE REPORT
A 66-year-old woman, in otherwise in good health, presented with a 10 month history of left HFS. She described, brief painless episodes of muscle twitching around her left eye which, over the ensuing months, became more severe, causing complete eye closure, with involvement of the lower facial musculature also. By the time of presentation she was experiencing several episodes daily. She complained of no other symptoms and of particular note there was no facial numbness, hearing deficit, tinnitus, vertigo or speech or swallowing difficulty. Neurological examination failed to reveal any abnormality. There were no palpable neck masses or mastoid bruits. A magnetic resonance imaging (MRI) head scan was performed and revealed a small (2 cm) poorly defined hypervascular soft tissue mass at the left skull base centered on the lateral aspect of the jugular foramen with focal involvement of the jugular vein (Fig. 1). There was no evidence of abnormality in the cerebellopontine angle cistern or internal auditory canal (Fig. 2). A computed tomography (CT) head scan demonstrated permeative bone destruction in the adjacent temporal bone extending to involve the facial canal containing the descending portion of the facial nerve (Fig. 3). There was no involvement of the middle or inner ear. The diagnosis of a small paraganglioma was suggested. In view of the radiology, catecholamine and serotonin metabolities (VMA and HIAA respectively) were screened for. Because of the size and position of the tumour, it was decided to treat her with radiotherapy rather than attempt neurosurgical decompression and/or removal.
Fig. 2 Tzweighted axial MRI study demonstrating normal cerebel]opontine angle cisterns and internal auditory canals (arrow). The vessel visible adjacent to the left middle cerebral peduncle was not in contact with the seventh cranial nerve.
Fig. 3 Axial CT scan presented on bony settings illustrates permeative destruction of the temporal bone with involvement of the facial canal (arrow).
Journal of Clinical Neuroscience (1999) 6(4)
350
Payer et aL
DISCUSSION In a high percentage of cases, HFS is caused by vascular compression of the seventh cranial nerve as it exits the brain stem. This has been demonstrated both radiologically and surgicallyY '8 Jannetta et al. have stated that the vascular compression must be crosscompression at right angles to the nerve, at its root exit zone from the brain stem3; this is the point where central myelin is replaced by peripheral myelin and this may be important in the pathogenesis. 3 It has been postulated that compression of the nerve causes focal demyelination and demyelinated axons can then activate adjacent nerve fibres by ephaptic transmission producing hemifacial spasm? Scoville, however, has reported a case successfully treated by removing a peripheral artery compressing the facial nerve, in the cerebello-pontine angle? Nagata et al. have reported four cases in which a tumour was thought responsible for HFS. No artery was visualized compressing the nerve at the root exit zone, and in three of the four cases, the HFS disappeared after the tumour, which was in contact with the facial nerve, was removed. 4 In our case, both the CT and MRI head scans showed the tumour mass, thought highly likely to be a glomus jugulare tumour, involving the descending portion of the seventh cranial nerve. No vascular compression of the seventh nerve at its root exit zone was seen. We feel, therefore, that this is the most likely cause of our patient's HFS. Glomus jugulare tumours, otherwise termed jugulotympanic paragangliomas, are very uncommon neoplasms, accounting for only 0,6% of all head and neck tumours? ° They are, however, the second commonest turnout to involve the temporal bone al and are the most common turnout in the jugular foramen. These neoplasms arise from the extra-adrenal paraganglia located in the jugular bulb within the temporal bone at the jugular foramenJ 2 Despite locally aggressive behaviour, metastases are rare./2 There is often a long interval between first symptoms and diagnosis, as they tend to be indolent, slow growing tumours. 12-14 The usual presentation of glomus jugulare tumours is otologic. ~ Lower cranial nerve lesions are seen in about one third of cases, n, ~5 Involvement of the seventh cranial nerve has been described ~,~2,15 and was documented in 12 of 57 cases of glomus jugulare tumours reported by Spector et al.l~ All cases, however, report facial paralysis, and none causing HFS have been reported, as far as we are aware. The gross appearance of these tumours is of an encapsulated, lobulated mass which is purple-red in colour. They have a haemorrhagic appearance, spongy texture and bleed readily. They are composed of nests of polyclonal chief cells, and mitoses are few./5 They can be quite aggressive locally, with intracranial extension, most frequently into the posterior cranial fossa, For tumours greater than 2 cm in diameter MRI demonstrates the characteristic 'salt and pepper' appearance of glomus jugulare tumours due to a combination of signal voids from flowing blood ('black') and loci of subacute haemorrhage .()white') on T2-weighted pre-contrast scans./6 MRI also demonstrates the extension of the tumour into the cranial fossa and vascular invasion directly (without contrast)J 7 After contrast injection there is marked enhancement of the lesion, reflecting its highly vascular nature./7 High resolution CT illustrates bone destruction and intracranial extension very well./7 Our case demonstrates the typical ill defined margin and intense contrast enhancement. The mass showed heterogenous signal intensity but did not show the diagnostic 'salt and pepper' appearance presumably due to its smaller size. The radiological differential diagnosis of this lesion includes nerve sheath tumours, meningiomas and malignant tumours (carcinomas, metastases). TM Nerve sheath tumours tend to produce smooth scalloped enlargement of the foramen and are
Journal of Clinical Neuroscience (1999) 6(4)
homogeneous or cystic, Meningiomas rarely arise in the temporal bone and show some of the behavioural patterns of paragangliomas with local bone invasion occurring at the bone margins but tend to be better defined; they tend to be homogeneous and lack the 'salt and pepper' pattern. Carcinoma, metastases and myeloma can all destroy the jugular fossa but most are much less vascular and show much less contrast enhancement and also have a typically homogeneous appearance on MRI. In addition, there was no history of primary malignancy in this case. HFS is sometimes managed non-surgically, especially in milder cases and older patients. Carbamazepine and botulinum toxin injections are two medical treatments which can be employed? In such cases, imaging studies are not routinely obtained. Cranial nerve involvement is often a late manifestation of glomus tumours, 15 when surgical treatment is likely to have a high morbidity? 9 The best clinical practice guidelines for the management of HFS are not clearly stated in most neurological texts. 2,2°,21 A non-invasive investigation such as magnetic resonance angiography would seem reasonable for those cases thought to be due to neurovascular compression, in whom surgical decompression is planned. Despite the low incidence of compressive lesions (other than vascular) we believe that all persons with HFS should have an MRI scan. If the first manifestation of a mass lesion can be HFS then identification of such lesions early is clearly very important.
SUMMARY We report a very unusual cause of HFS, a glomus jugulare tumour, in a patient with no other symptoms or signs. Because of such cases we feel MRI of the brain stem is a reasonable investigation for all patients presenting with HFS.
REFERENCES 1. MaroonJC Hemifacialspasma vascularcause. Archivesof Neurology 1978; 35: 481483. 2. AdamsRD, VictorM, Ropper AH. Principlesof Neurology. 6th ed. New York: McGrawHill, 1997; 1378-1379. 3. JarmettaPJ, AbbanyM, MaroonJC, Ramos FM, Albin MS. Etiologyand definitivemicrosurgicaltreatmentof hemifacialspasm. J Neurosurgery 1977; 47: 321-328. 4. NagataS, MatsushimaT, Fujii K, Fuldn M, KuromatsuC. Hemifacialspasm due to tumour, aneurysmor arteriovenousmalformation.Surg Neurol 1992;38: 204-209. 5. SinghA., Join VK, ChhabraDV, Hong K, KobayashiS. Hemifacialspasmand cerebellopontineangle epidermoid:case report and review.NeurolRes 1994; 16: 321-323. 6. InoueT, MaeyamaR, Ogawa H. Hemifacialspasmresultingfrom a cerebellopontine anglelipoma: case report. Neurosurgery 1995; 36: 846-848. 7. JespersonJH, DupontE, GelineckJ, LtmdorfE. Hemifacialspasm: magnetic resonanceangiography.Acta NeurolStand 1996;93: 35-38. 8. AdlesCH, ZimmermannRA, SavinoPJ, Bemadi B, BosleyTM, SergottRC. Hemifacial spasm: evaluationby magneticresonanceimagingand magnetic resonancetomographicangiography.AnnalNeurol 1992; 32: 502-506. 9. ScovilleWB. Hearingloss followingexplorationof the cerebellopontineangle in the treatmentof hemifacialspasm. J Neurosurg 1969; 31: 4749. 10. ReddyMU, MansfieldLM, HartmanGV. Chemodectomasof the glomus jugulare. Cancer 1983; 52: 337-340. 11. SpectorGJ, GadoM, CiralskyR, Ogura J, MaiseIRH. Neurological implicationsof glomus tumoursin the head and neck. Laryngoscope1975; 85: 1387-1395. 12. JohnstonePAS,Foss RD, DesiletsDJ. Malignantjugulotyrnpanic paraganglioma.Arch Path Lab Med 1990; 114:976-979. 13. GlassockME. The history of glomus tumours:a personalperspective. Laryngoscope 1993; 103: 3-6. 14. BoyleJO, ShimmOS, CoulthardSW. Radiationtherapyfor paragangliomaof the temporalbone. Laryngoscope1990; 100: 896-901. 15. Juliana-GulyaA. The glomustumanr and its biology.Laryngoscope 1993; 103: 7-15.
© 1999 Harcourt Brace & Co. Ltd
Hemifacial spasm~Pituitary abscess
16. OlsenLW, DillonPW, KellyMW. MR imagingof paragangliomas.AJ Roentgerology1987; 148: 201504. 17. AthanassopoulouAC, VlahosLL, GouliamosAD, KallidouED, PapailiouJG, AngeliSS. MRI featuresin a malignantglomusjugulare tumour. The Journal of Laryngologyand Otology 1993; 197:1066 1067. 18. Lo WWM,Solti-BohmanLG. Tumoursof the temporalbone and cerebellopontine angle, In: Headand Neck Imaging,3rd ed. Sore P M, CurtinHD (Eds.) New York:MosbyPress, 1996; 1484-1496. 19. BrammerRE, GrahamMD, KemickJL. Glomustumours of the temporalbone: contemporary evaluationand therapy. OtolaryngolClinicNorthAmerica1984; 17:499 512. 20. JmmettaPJ. Surgicalapproachto hemifacialspasm: microvascular decompression.In: MovementDisorders,Neurology2. C Marsden, S Fahn (Eds). Butterworth-Heinemann,1982, 330-333. 21. BorodicGE. Hemifacialspasm: Evaluationand Management,with emphasis on botulinumtoxintherapy. In: Therapywith BotulinumToxin. J Jankovic,M Hallett (Eds). New York:MarcelDekker, 1994; 331-351.
Pituitary abscess Christine S. Hon 1, Neville K n u c k e y 1 FRACS, Peter Robbins 2 FRCPA, Peter T. Pullan 3 FRACP ~Department of Neurosurgery, The Queen Elizabeth II Medical Centre, Verdun Street, Nedlands, WA, 6009, Australia ~Division of Tissue Pathology, Western Australian Centre for Pathology and Medical Research, Locked Bag 2009, P.O. Nedlands, 6009, Australia "Department of Endocrinology and Diabetes, The Queen Elizabeth fl Medical Centre, Verdun Street, Nedlands, WA, 6009, Australia
S u m m a r y A 28-year-old Caucasian woman presented with a 12 month history of secondary amenorrhoea, polyuria and polydipsia with fatigue and weight loss. Investigations revealed panhypopituitarism, diabetes insipidus, an intrasellar mass and papilloedema, thought to be due to benign intracranial hypertension. She was treated conservatively. However, a repeat magnetic resonance image showed enlargement of the pituitary mass with compression of the optic nerves. The pituitary abscess was drained by a transsphenoidal approach. Postoperatively the patient received antibiotics with no recurrence of the pituitary abscess. Journalof ClinicalNeuroscience(1999)6(4), 351-353© 1999HarcourtBrace& Co. Ltd
351
We review the literature on pituitary abscess, highlight the importance of diabetes insipidus as an important clinical sign and demonstrate the magnetic resonance imaging (MRI) features of this condition. CASE REPORT
The patient is a 28-year-old Caucasian woman who initially presented with a 12 month history of secondary amenorrhoea, polyuria and polydipsia with fatigue and weight loss. She also complained of 4 months, intermittent headache and an episode of blurred vision and diplopia. She had no history of meningitis, rhinorrhoea or systemic sepsis. On examination, she was morbidly obese, her pubic hair was slightly sparse, but she showed no other clinical evidence of hypopituitarism. Clinical examination showed no focal neurological deficit. Visual acuities and visual fields were normal, but the optic fundi showed papilloedema. Initial investigations including blood film, erythrocyte sedimentation rate, serum glucose, calcium, creatinine, electrolytes and liver function tests were normal. Endocrinological investigations revealed that she had panhypopituitarism. Serum oestradiol was 82 pmol/1 (normal: 100-450pmol/1), FSH was 2u/1 (normal: 2-8 u/l) and LH was 1 u/l (normal: 2-13 u/l). The serum free thyroxine was 7pmol/1 (normal: l l - 1 9 p m o l / l ) with TSH 0.30 mud (normal: 0.34-4.80 muff). Following synthetic ACTH stimulation, plasma cortisol peaked at 449 nmolB (normal greater than 550 nmol/l). The serum growth hormone was less than 1 mu/L (normal: <4 muff) and IGF-1 60 ug/1 (normal: 144-360 ug/1). Serum prolactin was mildly elevated at 688 mull (normal: <425 mu/l). A spot plasma osmolality was towards the upper limit of normal at 290 mmol/kg (normal: 275-295 mmol/kg) with an inappropriately low urine osmolality of 190 mmol/kg confirming the clinical diagnosis of diabetes insipidus. Plain lateral skull X-ray demonstrated a slightly enlarged sella. Computerized tomography (CT) scan showed a cystic sellar mass. The initial MRI scan showed a hypointense intrasellar mass on T2-weighted image (Fig. l). The visual evoked potentials were normal. The initial clinical diagnosis was panhypopituitarism due to an intrasellar mass and benign intracranial hypertension. She was treated conservatively with hormone replacement with cortisone acetate (25 mg mane and 12.5 mg nocte), thyroxine (100ug mane) and desmopressin (10 ug intranasally nocte). The papilloedema resolved. However, a repeat MRI showed enlargement of
Keywords: pituitary abscess, diabetes insipidus, hypopituitarism Received 26 May 1997 Accepted 26 June 1997 Correspondence to: N. Knuckey, Tel.: +61 9 346 2865; Fax: +61 9 346 3824; E-mail; [email protected]
INTRODUCTION
Pituitary abscess is rare and approximately 60 cases have been reported in the medical literature. Since pituitary abscess is potentially fatal, early clinical diagnosis is critical. However, the clinical diagnosis is difficult because there are no clinical symptoms or signs characteristic of pituitary abscess and it is frequently indistinguishable clinically from other pituitary lesions. Conservative medical treatment with endocrine replacement therapy or antibiotics has a high mortality. Hence, the treatment of choice is drainage of the pituitary abscess via transsphenoidal surgery and the administration of appropriate antibiotics and endocrine replacement therapy. Since pituitary abscess is rare and potentially fatal if not treated, we report a case to highlight its important clinical and diagnostic features.
© 1999 Harcourt Brace & Co. Ltd
Fig. 1 A T2-weighted MRI of the pituitary fossa showing an enlarged hypointense pituitary lesion.
Journal of Clinical Neuroscience (1999) 6(4)