247
Clinical Neurology and Neurosurgery, 94 (1992) 247-250 0 1992 Elsevier Science Publishers B.V. All rights reserved 0303-8467/92/S 05.00
CLINEU 00195
Case report
Hyp~rtrophic cranial pachym~nin~itis due to A~~er~i~~~spavu~ H. Murai”, J. Kira”, T. Kobayashi”, I. Gotoa, H. Inoueb and K. HasuoC ‘~eparrment of Neurology, ~eura~ogi~alrns~~~u~e, ‘~epar~rnen~of O~aIaryngo~agy, and ‘department of ~udialagy,Family af ~ed~~~ne, Kyushu University,Fukuoka, Japan (Received 27 January, 1992) (Revised, received 17 February, 1992) (Accepted 17 February, 1992)
Key words:
Hypertrophic
cranial pachymeningitis;
AspergillusJiavus; MRI; Gadolinium-MRI;
Pachymeningitis
Summary A 59-year-old woman suffered from occipital headache and bilateral cranial nerve VII, VIII, IX, X, XI and right XII deficit after developing otitis media. Magnetic resonance imaging (MRI) showed a thickening of the dura mater which was enhanced by gadolinium-DTPA (Gd). Aspergiflusflavus was identified from the culture of otorrhea. She was treated with miconazole, flucytosin and fluconazole, which resulted in an improvement of the clinical symptoms and a thinning of the Gd-enhanced lesions on MRI. This is the first case of hypertrophic cranial pachymeningitis caused by Asp. j?avus infection.
Introduction
Case report
Hypertrophic cranial pachymeningitis is a rare disease that often causes multiple cranial neuropathy. By now, few cases have been reported in the literature, and the greater part of them are still etiologically unclarified. We report a case of hypertrophic cranial pachymeningitis following otitis media, presenting occipital headache and multiple cranial neuropathy. AspergiZlus fIavus was proven to be the etiological agent. To our knowledge, this is the first case of hypertrophic cranial pachymeningitis due to Asp. fzavus infection.
A 59-year-old woman was born deaf in her left ear due to Mondini’s anomaly (hypoplasia of the auris interna). She had suffered from diabetes mellitus since 1968, and has been receiving insulin therapy for the past 2 years. In addition, she developed liver cirrhosis with esophageal varices in 1986. In October 1987, she developed otitis media on the left side. She noticed left occipitalgia a few months later, and developed left facial palsy in April 1988. In October 1988, she noted hoarseness and difficulty in swallowing. In January 1989, she suffered from otitis media on the right side, followed by headache and hearing impai~ent on the right side. In March 1989, a tracheal fenestration was performed as she developed dyspnea because of palsy of the bilateral vocal cords. On April 3, 1989, the date of the admission to our department, physical examination revealed: anemic palpebral conjunctivae and hepatomegaly (4 cm palpable below costal margin). Mental state was normal, and cranial
Correspondence to: H. Murai, Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University 60, 3-l-l Maidashi, Higashi-ku, Fukuoka 812, Japan.
Fig. 1. Sagittal MRI (Gd-enhanced T,-weighted image; TR400/TE20). a: before treatment; was enhanced
by Gd. b: after treatment
with antifungal
agents; Gd enhancement
thickened dura mater in the posterior of the dura mater was markedly reduced.
fossa
nerves I-VI were all normal. Moderate facial palsy on the left side. becoming bilateral one month later. Severe sensorineural hearing impairment on the right side, and
mography (CT) of the upper abdomen indicated liver cirrhosis. A head CT showed right maxillary sinusitis and a contrast enhancement of the dura mater in the
moderate to marked dysphagia and dysarthria. Taste loss and unsteady gait appeared one month later. The soft palate movement was poor and the right vocal cord was immobile. Bilateral sternocleidomastoid muscles
posterior fossa. MRI revealed a thickened dura mater in the posterior fossa and above the clivus, strongly en-
were weak. The tongue deviated to the right on protrusion. Severe pain occurred on passive neck movements. Muscle power, tonus and coordination of the extremities were normal. Gait was slightly unsteady; tandem gait was poor. Mild impairment of vibratory sensation and hyporeflexia, symptoms of diabetic neuropathy, were observed in the lower extremities. The blood count showed mild anemia (Hb 10.2 g/dl). The ESR was 55 mm/h. Serum protein was 8.0 mgldl and albumin 3.3 mg/dl. Liver function tests were: GOT 52 IU/l. y-GTP 204 IUIl, cholinesterase 68 IUIl, and ICG 43.8% (15 min). The fasting glucose level was 163 mgidl and HbAlc level was as high as 7.0%. C-reactive protein level was 1.5 mg/dl (normal, < 0.4 mgidl). The cerebrospinal fluid was clear. showing mild pleocytosis (7/mm’) and 66 mgidl protein. X-ray examinations of skull, chest, and abdomen were unremarkable. Ultrasonography and computed to-
hanced by Gd infusion (Fig. 1a). as well as right maxillary sinusitis. Gallium scintigraphy was negative. Angiography was not performed. Fungal hyphae were microscopically observed in the smear of the surgically evacuated content in the right maxillary sinus. A culture of the otorrhea revealed the formation of yellowish green colonies, which were identified as Asprl-giffus,fl~us (Fig. 2). From May 16, miconazole therapy was started with an initial dose of 200 mgiday up to 1000 mg/day, intravenously. One month later, occipital headache, bilateral facial palsy and dysphagia improved markedly. Furthermore. an MRI taken on June 28 revealed that the Gd enhancement of the dura mater in the posterior fossa was markedly reduced (Fig. 1b). Miconazole was discontinued 1 month later because of the worsening anemia. Flucytosine (4 6 g/day) and then. fluconazole (100 mgi day) was administered orally instead. At the end of August. the facial weakness and dysphagia were hardly noticeable. Soft palate movement and tongue protrusion
249 been
reported.
tuberculosis
Among
them,
[5], Wegener’s
itis nodosa
[7], rheumatoid
arthritis
of methylprednisolone
reported
to cause the disease,
cluded
unclarified.
[2], fungus
granulomatosis
injection
etiologically
syphilis
[3,4],
[6], periarter-
[8,9] and intraspinal
acetate
[lo] have been
but most of the cases are
Fungal
pachymeningitis
in-
Candida tropicalis [3] and Petriellidium boydii [4].
To our knowledge,
the present case is the first case due to
Asp. flaws. Inflammation pergillus infection
skull base, in the presence diabetes
mellitus
by As-
of otitis media caused
easily extends to the dura mater of the of Mondini’s
may have contributed
anomaly.
The
to the persistence
of the fungal infection. Reports
on the MRI findings
pachymeningitis ously reported
of hypertrophic
are still rare. Martin CT and MRI findings
cranial
et al. [l l] previof hypertrophic
cranial pachymeningitis, but Gd enhancement was not performed in their cases. Yuh [9] and Callebaut [5] reported the contrast-enhanced MRI findings of rheumatoid and tuberculous pachymeningitis, respectively. Ishii et al. [ 121 recently reported a case of pachymeningitis which presented Garcin’s syndrome. In their case, after the therapy with antibiotics and prednisolone, decrease
Fig. 2. Spore of AspergillusJlavus identified from the culture of otorrhea.
in thickness of dura mater had been observed on the GdMRI. The etiological agent, however, remained unclear. In our case, brain MRI revealed a thickening of the dura mater and the existence of a chronic inflammation
had returned to almost normal. Hearing difficulty, neck pain, ataxia and right vocal cord paresis, however, re-
in the thickened dura, as determined by Gd enhancement. A reduction of Gd enhancement of the dura after the therapy suggested the inflammation in the dura had subsided and thus the effectiveness of the antifungal
mained.
No surgical
exploration
was performed.
MRI
appears
most useful
for monitoring
the effectiveness of treatment as well as in making accurate diagnosis of hypertrophic pachymeningitis, pecially when Gd enhancement is done.
Discussion In the present case, a marked thickening of the dura mater in the posterior fossa as seen on MRI, indicated hypertrophic cranial pachymeningitis to be the cause of multiple cranial neuropathy. We assumed Aspergihs to be the etiological agent because of the following reasons, although we have not performed any surgical exploration of the dura mater: (1) fungal hyphae were observed in the content of the maxillary sinus, and Asp. Jaws was identified from the culture antifungal agents, clinical edly, and Gd enhancement
drugs. Therefore,
of otorrhea. (2) After using symptoms improved markof the dura mater thinned
remarkably. Since the first case of hypertrophic .pachymeningitis was reported by Charcot in 1869 [l], several cases have
an es-
References Charcot, J.M. and Joffroy, A. (1869) Deux cas d’atrophie musculaire progressive avec l&ions de la substance grise et des faisceaux antkrolateraux de la mo&lle Cpinikre. Arch. Physiol. Norm. Pathol., 2: 354-367. Hassin, G.B. and Zeitlin, H. (1940) Syphilitic cerebral hypertrophic pachymeningitis. Clinicopathologic studies in a case. Arch. Neurol. Psychiat., 43: 362-371. Gorell, J.M., Palutke, W.A. and Chason, J.L. (1979) Candida pachymeningitis with multiple cranial nerve pareses. Arch. Neurol., 36: 719-720. Schiess, R.J., Coscia, M.F. and McClellan, G.A. (1984) Pet-
250 hoydii pachymeningitis treated with miconazole and ketoconazole. Neurosurgery, 14: 220-224. Callebaut, J., Dormont, D., Dubois, B., Chiras, J. and Bories, J. (1990) Contrast-enhanced MR imaging of tuberculous pachymeningitis cranialis hypertrophica: case report. AJNR, 11: 821-822. Eto, F., Shimada, Y., Endo, H. and Mozai, T. (1976) An autopsied case of Wegener’s granulomatosis with remarkable pachymeningitis. Rinsho Shinkeigaku, 16: 3266332. Astrom, K.E. and Lidholm, S.O. (1963) Extensive intracranial lesions in a case of orbital nonspecific granuloma combined with polyarteritis nodosa. J. Clin. Pathol., 16: 137143. Weinstein, G.W., Powell, S.R. and Thrush, W.P. (1987) Chiasmal neuropathy secondary to rheumatoid pachymeningitis. Am. J. Ophthalmol., 104: 439440. riellidium
5
6
7
8
9 Yuh. W.T.C., Drew, J.M., Rizzo, M., Ryals, T.J., Sato, Y. and Bell, W.E. (1990) Evaluation of pachymeningitis by contrast-enhanced MR imaging in a patient with rheumatoid disease. AJNR, 11: 1247-1248. 10 Nelson, D.A. (1988) Dangers from methylprednisolone acetate therapy by intraspinal injection. Arch. Neurol., 45: 804.~ 806. 11 Martin, N., Masson, C., Henin, D., Mompoint, D., Marsault, C. and Nahum, H. (1989) Hypertrophic cranial pachymeningitis: assessment with CT and MR imaging. AJNR, 10: 477484. 12 Ishii, A., Ohkoshi, N., Nagata, H., Mizusawa, H. and Kanazawa, I. (1991) A case of Garcin’s syndrome caused by pachymeningitis secondary to otitis media, responsive to antibiotic therapy. Rinsho Shinkeigaku, 3 1: 837-841.
Clinical Neurology and Neurosurgery, 94 (1992) 247-250 0 1992 Elsevier Science Publishers B.V. All rights reserved 0303-8467/92/S 05.00
CLINEU 00195
Case report
Hyp~rtrophic cranial pachym~nin~itis due to A~~er~i~~~spavu~ H. Murai”, J. Kira”, T. Kobayashi”, I. Gotoa, H. Inoueb and K. HasuoC ‘~eparrment of Neurology, ~eura~ogi~alrns~~~u~e, ‘~epar~rnen~of O~aIaryngo~agy, and ‘department of ~udialagy,Family af ~ed~~~ne, Kyushu University,Fukuoka, Japan (Received 27 January, 1992) (Revised, received 17 February, 1992) (Accepted 17 February, 1992)
Key words:
Hypertrophic
cranial pachymeningitis;
AspergillusJiavus; MRI; Gadolinium-MRI;
Pachymeningitis
Summary A 59-year-old woman suffered from occipital headache and bilateral cranial nerve VII, VIII, IX, X, XI and right XII deficit after developing otitis media. Magnetic resonance imaging (MRI) showed a thickening of the dura mater which was enhanced by gadolinium-DTPA (Gd). Aspergiflusflavus was identified from the culture of otorrhea. She was treated with miconazole, flucytosin and fluconazole, which resulted in an improvement of the clinical symptoms and a thinning of the Gd-enhanced lesions on MRI. This is the first case of hypertrophic cranial pachymeningitis caused by Asp. j?avus infection.
Introduction
Case report
Hypertrophic cranial pachymeningitis is a rare disease that often causes multiple cranial neuropathy. By now, few cases have been reported in the literature, and the greater part of them are still etiologically unclarified. We report a case of hypertrophic cranial pachymeningitis following otitis media, presenting occipital headache and multiple cranial neuropathy. AspergiZlus fIavus was proven to be the etiological agent. To our knowledge, this is the first case of hypertrophic cranial pachymeningitis due to Asp. fzavus infection.
A 59-year-old woman was born deaf in her left ear due to Mondini’s anomaly (hypoplasia of the auris interna). She had suffered from diabetes mellitus since 1968, and has been receiving insulin therapy for the past 2 years. In addition, she developed liver cirrhosis with esophageal varices in 1986. In October 1987, she developed otitis media on the left side. She noticed left occipitalgia a few months later, and developed left facial palsy in April 1988. In October 1988, she noted hoarseness and difficulty in swallowing. In January 1989, she suffered from otitis media on the right side, followed by headache and hearing impai~ent on the right side. In March 1989, a tracheal fenestration was performed as she developed dyspnea because of palsy of the bilateral vocal cords. On April 3, 1989, the date of the admission to our department, physical examination revealed: anemic palpebral conjunctivae and hepatomegaly (4 cm palpable below costal margin). Mental state was normal, and cranial
Correspondence to: H. Murai, Department of Neurology, Neurological Institute, Faculty of Medicine, Kyushu University 60, 3-l-l Maidashi, Higashi-ku, Fukuoka 812, Japan.
Fig. 1. Sagittal MRI (Gd-enhanced T,-weighted image; TR400/TE20). a: before treatment; was enhanced
by Gd. b: after treatment
with antifungal
agents; Gd enhancement
thickened dura mater in the posterior of the dura mater was markedly reduced.
fossa
nerves I-VI were all normal. Moderate facial palsy on the left side. becoming bilateral one month later. Severe sensorineural hearing impairment on the right side, and
mography (CT) of the upper abdomen indicated liver cirrhosis. A head CT showed right maxillary sinusitis and a contrast enhancement of the dura mater in the
moderate to marked dysphagia and dysarthria. Taste loss and unsteady gait appeared one month later. The soft palate movement was poor and the right vocal cord was immobile. Bilateral sternocleidomastoid muscles
posterior fossa. MRI revealed a thickened dura mater in the posterior fossa and above the clivus, strongly en-
were weak. The tongue deviated to the right on protrusion. Severe pain occurred on passive neck movements. Muscle power, tonus and coordination of the extremities were normal. Gait was slightly unsteady; tandem gait was poor. Mild impairment of vibratory sensation and hyporeflexia, symptoms of diabetic neuropathy, were observed in the lower extremities. The blood count showed mild anemia (Hb 10.2 g/dl). The ESR was 55 mm/h. Serum protein was 8.0 mgldl and albumin 3.3 mg/dl. Liver function tests were: GOT 52 IU/l. y-GTP 204 IUIl, cholinesterase 68 IUIl, and ICG 43.8% (15 min). The fasting glucose level was 163 mgidl and HbAlc level was as high as 7.0%. C-reactive protein level was 1.5 mg/dl (normal, < 0.4 mgidl). The cerebrospinal fluid was clear. showing mild pleocytosis (7/mm’) and 66 mgidl protein. X-ray examinations of skull, chest, and abdomen were unremarkable. Ultrasonography and computed to-
hanced by Gd infusion (Fig. 1a). as well as right maxillary sinusitis. Gallium scintigraphy was negative. Angiography was not performed. Fungal hyphae were microscopically observed in the smear of the surgically evacuated content in the right maxillary sinus. A culture of the otorrhea revealed the formation of yellowish green colonies, which were identified as Asprl-giffus,fl~us (Fig. 2). From May 16, miconazole therapy was started with an initial dose of 200 mgiday up to 1000 mg/day, intravenously. One month later, occipital headache, bilateral facial palsy and dysphagia improved markedly. Furthermore. an MRI taken on June 28 revealed that the Gd enhancement of the dura mater in the posterior fossa was markedly reduced (Fig. 1b). Miconazole was discontinued 1 month later because of the worsening anemia. Flucytosine (4 6 g/day) and then. fluconazole (100 mgi day) was administered orally instead. At the end of August. the facial weakness and dysphagia were hardly noticeable. Soft palate movement and tongue protrusion
249 been
reported.
tuberculosis
Among
them,
[5], Wegener’s
itis nodosa
[7], rheumatoid
arthritis
of methylprednisolone
reported
to cause the disease,
cluded
unclarified.
[2], fungus
granulomatosis
injection
etiologically
syphilis
[3,4],
[6], periarter-
[8,9] and intraspinal
acetate
[lo] have been
but most of the cases are
Fungal
pachymeningitis
in-
Candida tropicalis [3] and Petriellidium boydii [4].
To our knowledge,
the present case is the first case due to
Asp. flaws. Inflammation pergillus infection
skull base, in the presence diabetes
mellitus
by As-
of otitis media caused
easily extends to the dura mater of the of Mondini’s
may have contributed
anomaly.
The
to the persistence
of the fungal infection. Reports
on the MRI findings
pachymeningitis ously reported
of hypertrophic
are still rare. Martin CT and MRI findings
cranial
et al. [l l] previof hypertrophic
cranial pachymeningitis, but Gd enhancement was not performed in their cases. Yuh [9] and Callebaut [5] reported the contrast-enhanced MRI findings of rheumatoid and tuberculous pachymeningitis, respectively. Ishii et al. [ 121 recently reported a case of pachymeningitis which presented Garcin’s syndrome. In their case, after the therapy with antibiotics and prednisolone, decrease
Fig. 2. Spore of AspergillusJlavus identified from the culture of otorrhea.
in thickness of dura mater had been observed on the GdMRI. The etiological agent, however, remained unclear. In our case, brain MRI revealed a thickening of the dura mater and the existence of a chronic inflammation
had returned to almost normal. Hearing difficulty, neck pain, ataxia and right vocal cord paresis, however, re-
in the thickened dura, as determined by Gd enhancement. A reduction of Gd enhancement of the dura after the therapy suggested the inflammation in the dura had subsided and thus the effectiveness of the antifungal
mained.
No surgical
exploration
was performed.
MRI
appears
most useful
for monitoring
the effectiveness of treatment as well as in making accurate diagnosis of hypertrophic pachymeningitis, pecially when Gd enhancement is done.
Discussion In the present case, a marked thickening of the dura mater in the posterior fossa as seen on MRI, indicated hypertrophic cranial pachymeningitis to be the cause of multiple cranial neuropathy. We assumed Aspergihs to be the etiological agent because of the following reasons, although we have not performed any surgical exploration of the dura mater: (1) fungal hyphae were observed in the content of the maxillary sinus, and Asp. Jaws was identified from the culture antifungal agents, clinical edly, and Gd enhancement
drugs. Therefore,
of otorrhea. (2) After using symptoms improved markof the dura mater thinned
remarkably. Since the first case of hypertrophic .pachymeningitis was reported by Charcot in 1869 [l], several cases have
an es-
References Charcot, J.M. and Joffroy, A. (1869) Deux cas d’atrophie musculaire progressive avec l&ions de la substance grise et des faisceaux antkrolateraux de la mo&lle Cpinikre. Arch. Physiol. Norm. Pathol., 2: 354-367. Hassin, G.B. and Zeitlin, H. (1940) Syphilitic cerebral hypertrophic pachymeningitis. Clinicopathologic studies in a case. Arch. Neurol. Psychiat., 43: 362-371. Gorell, J.M., Palutke, W.A. and Chason, J.L. (1979) Candida pachymeningitis with multiple cranial nerve pareses. Arch. Neurol., 36: 719-720. Schiess, R.J., Coscia, M.F. and McClellan, G.A. (1984) Pet-
250 hoydii pachymeningitis treated with miconazole and ketoconazole. Neurosurgery, 14: 220-224. Callebaut, J., Dormont, D., Dubois, B., Chiras, J. and Bories, J. (1990) Contrast-enhanced MR imaging of tuberculous pachymeningitis cranialis hypertrophica: case report. AJNR, 11: 821-822. Eto, F., Shimada, Y., Endo, H. and Mozai, T. (1976) An autopsied case of Wegener’s granulomatosis with remarkable pachymeningitis. Rinsho Shinkeigaku, 16: 3266332. Astrom, K.E. and Lidholm, S.O. (1963) Extensive intracranial lesions in a case of orbital nonspecific granuloma combined with polyarteritis nodosa. J. Clin. Pathol., 16: 137143. Weinstein, G.W., Powell, S.R. and Thrush, W.P. (1987) Chiasmal neuropathy secondary to rheumatoid pachymeningitis. Am. J. Ophthalmol., 104: 439440. riellidium
5
6
7
8
9 Yuh. W.T.C., Drew, J.M., Rizzo, M., Ryals, T.J., Sato, Y. and Bell, W.E. (1990) Evaluation of pachymeningitis by contrast-enhanced MR imaging in a patient with rheumatoid disease. AJNR, 11: 1247-1248. 10 Nelson, D.A. (1988) Dangers from methylprednisolone acetate therapy by intraspinal injection. Arch. Neurol., 45: 804.~ 806. 11 Martin, N., Masson, C., Henin, D., Mompoint, D., Marsault, C. and Nahum, H. (1989) Hypertrophic cranial pachymeningitis: assessment with CT and MR imaging. AJNR, 10: 477484. 12 Ishii, A., Ohkoshi, N., Nagata, H., Mizusawa, H. and Kanazawa, I. (1991) A case of Garcin’s syndrome caused by pachymeningitis secondary to otitis media, responsive to antibiotic therapy. Rinsho Shinkeigaku, 3 1: 837-841.
