Clinical Neurology
0
and Neurosurgery, 94 (1992) 261-263
1992 Elsevier Science Publishers
CLINEU
261
B.V. All rights reserved
0303-8467/92/S
05.00
00207
Case report
Pseudotumor
cerebri with familial Mediterranean
fever
Hamit Z. G(ikalp, Mustafa K. Baskaya and Varol Aydin Department
of Neurosurgery,
University of Ankara, (Received (Revised,
Familial Mediterranean
Ankara,
Turkey
1992)
received 4 May, 1992)
(Accepted
Key words~
16 January,
School of Medicine,
4 May, 1992)
fever; Headache; Pseudotumor
cerebri
Summary
Pseudotumor cerebri (PC) is a condition that occurs predominantly in obese women, and long lists of putative causes and associations have been reported. We describe here the case of a woman in whom PC coexisted with familial Mediterranean fever (FMF). A review of the literature revealed no report of an association of these two conditions.
erature, we thought the coexistence of these two conditions interesting.
Introduction
Pseudotumor cerebri (PC) is usually seen in females and is characterised by increased cerebrospinal fluid (CSF) pressure. It is a well defined syndrome of increased intracranial pressure with normal or decreased ventricles and normal CSF content. Increased intracranial pressure causes headache, dizziness, nausea-vomiting, tinnitus, and visual disturbances. The mean annual incidence is l/l00 000 in the general population [1,2]. It is seen frequently in females, especially when obese and in the reproductive period. In this group, the incidence of PC is reported as 19/100 000 [1,2]. Many diseases and conditions are reported to be aetiological factors of PC, but the exact aetiology and pathogenesis are still unknown. In our case, no disease or clinical condition was found that could cause PC. As PC with familial Mediterranean fever (FMF) has not previously been reported in the lit-
Correspondence to: Professor Neurosurgery, Hospital),
University
Dr. Hamit
of Ankara,
06100 Samanpazarl,
Ankara,
2. Gbkalp,
Department
Ibn-i Sina Hastanesi Turkey.
of
(Avicenna
Case report
The patient, a 22-year-old female, had been treated with colchicine for the past 1.5 years for FMF. When admitted to the Neurosurgery Department, she complained of headache, nausea and vomiting. She characterised her headache as generalized, getting worse and worse with time, and as being unresponsive to analgesics. Her history showed no remarkable disease apart from FMF. She did not describe any past infections, menstrual disturbance, pregnancy and lactation or anything else that might cause PC. The diagnosis of FMF was made after the observation of recurrent attacks of abdominal pain, fever, and leucocytosis by the Department of Internal Medicine. She had taken only colchicine 1 g daily for the past 1.5 years. Vital signs were normal and a physical examination revealed no abnormal findings. Her height was 155 cm and her weight 45 kg. Her mind was clear and co-operation and sense of orientation were intact. Fundoscopy
262 revealed
a bilateral
papilloedema.
were intact bilaterally.
nial and spinal nerve functions. laterally
normal.
functions
Visual field and acuity
There was no deficit in other cra-
There
Cerebellar
tests were bi-
was no neck stiffness.
Cortical
were also normal.
On lumbar
puncture,
CSF pressure
was 270 mm HzO,
and clear; analysis
for total protein,
glucose,
gave nor-
chloride,
and im-
munoglobulin. Urinalysis, phosphate), hormone
serum liver
electrolytes and
(Na’,
kidney
levels, and serum vitamin
Complete erythrocyte
blood
count
sedimentation
(CBC),
K’.
function
Ca”,
tests,
Cl-, serum
A level were normal. peripheral
rate were normal.
smear,
and
Serum iron,
iron finding capacity, ferritin level, serum folate and vitamin B,? levels were within normal ranges as was aspirated bone marrow. Cranial CT revealed no space-occupying lesion, oedema, or other abnormal findings. DSA showed no vascular abnormalities or sinus thrombosis. Methyl prednisolone (60 mg) and furosemide (80 mg)
The onset of symptoms
other disease processes,
surgical
tion
or withdrawal
of various
and
diet
In most
[14,6,8].
mechanism
and the CSF was colourless mal values
ual’s weight.
can be related
procedures,
to
administra-
drugs
and medications,
patients
no predisposing
can be found and the syndrome
must be con-
sidered to be idiopathic. Patients with PC require aggressive medical management. Generally. diuretics are the drugs of first choice, such as furosemide
and acetazolamide
production
11. Steroids
[4,5,8-l
lone and dexamethasone bination
with diuretics
remission therapy
within
CSF
such as methyl predniso-
are often used alone or in com[4,5,8810]. Steroids often induce a
a week;
has numerous
which inhibit
however,
long-term
steroid
side effects and may actually
in-
crease the chance of loss of vision by increasing intraocular pressure, and it should therefore be used with caution and only in 2-week periods [8]. Our case was treated with a combination of steroids and diuretics for 10 days. During that period her headache improved. Examination of visual acuity and field. repeated several times during that period. proved to be normal.
were given daily for 10 days. Colchicine was not withdrawn during the treatment of PC. CSF was drained
Repeated lumbar punctures frequently result in the relief of headache [4,5,8810]. In our case lumbar punctures
daily by lumbar puncture for 10 days to relieve headache and intracranial pressure decreased day after day. the last measurement being 120 mm H,O. The CSF was clear and colourless. The bilateral papilloedema improved. During her stay in hospital laboratory analyses were repeated and remained within normal ranges. The patient was discharged from hospital on day 20 without headache and papilloedema and with visual field and acuity
were performed daily to drain off CSF. FMF is a heredofamilial disorder of unknown pathogenesis. probably metabolic, characterised by recurrent episodes of abdominal or chest pain, fever and leucocytosis [12]. It is usually restricted to people of Mediterranean ancestry, primarily Armenians, Sephardic Jews and Arabs, and to some extent to people of Egyptian-Arabic origin living in Turkey. Greece, and Italy [12]. The disease suggests surgical peritonitis, but the acute attacks are recurrent, self-limiting, and not fatal [12,13]. Menin-
intact. On check-up neurological were within the patient
3, 6 and 9 months
later. systemic
and
examinations as well as all laboratory tests normal limits. At the control examination described
no further
attacks
of FMF.
Discussion Pseudotumor
cerebri
is a syndrome
of increased
in-
tracranial pressure without tumour, hydrocephalus or evident brain oedema. Moreover, PC is a diagnosis of exclusion; routine radiological and laboratory tests serve to eliminate other diagnostic possibilities [1,335]. In children and adults. headache, dizziness, nausea-vomiting, tinnitus, and visual disturbances are the symptoms seen most frequently [l-3,6-9]. PC is seen mostly in women. Most patients are obese and the activity of the disease may vary with the individ-
geal involvement has not widely been recognised as a manifestation of FMF. although occasional reports of meningeal irritation, meningism, and open meningitis have appeared in the literature [13]. However, FMF has so far not been reported in association with or as the cause of PC. Many series in the literature involving various numbers of cases have been evaluated to find the aetiological factor of PC and the coincidence of PC with FMF, but no evidence was found for either nor for a relationship that might shed light on the pathogenesis. In our case, despite the lack of an aetiological factor related to FMF, pre-treatment with colchicine for 1.5 years is remarkable. In the literature neuropathy and myoneuropathy have been reported as side effects of colchicine treatment, but so far it has not been suggested as an aetiological factor
of PC
[14,15].
263 References 1 Ireland, B., Corbett, J.J. and Wallace, R.B. (1990) The search for causes of idiopathic intracranial hypertension. A preliminary case-control study. Arch. Neural., 47: 3 15-320. 2 Durcan, F.J., Corbett, J.J. and Wall, M. (1988) The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch. Neurol., 45: 875-877. 3 Digre, K.B. and Corbett, J.J. (1988) Pseudotumor cerebri in man. Arch. Neurol., 45: 866-872. 4 Sklar, F.H. (1985) Pseudotumor cerebri. In: Wilkins, R.H. and Rengachary, S.S. (Eds.), Neurosurgery, vol. I, McGraw- Hill, New York, NY, pp. 350-353. 5 Corbett, J.J. and Thompson, H.S. (1989) The rational management of idiopathic intracranial hypertension. Arch. Neurol., 46: 1049-1051. 6 Sorensen, P.S., Kgogsaa, B. and Gjerris, F. (1988) Clinical course and prognosis of pseudotumor cerebri. A prospective study of 24 patients. Acta Neurol. Stand., 77: 164-172. 7 Round, R. and Keane, J.R. (1988) The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology, 38: 1461-1464. 8 Bowman, M.A. (1987) Pseudotumor cerebri. Am. Fam. Phys., Jan.: 177-182.
9 Greer, M. (1990) Pseudotumor cerebri. In: Youmans, J.R. (Ed.), Neurological Surgery, vol. V, 3rd edn., W.B. Saunders Co., Philadelphia, PA, pp. 35143530. 10 Jefferson, A. and Clark, J. (1976) Treatment of benign intracranial hypertension by dehydrating agents with particular reference to the measurement of blind spot area as a means of recording improvement. J. Neurol. Neurosurg. Psychiat., 39: 627-639. 11 Lyons, M.K. and Meyer, F.B. (1990) Cerebrospinal fluid physiology and the management of increased intracranial pressure. Mayo Clin. Proc., 65: 684707. 12 Pras, M., Bronshpigel, N., Zemer, D. and Gafni, J. (1982) Variable incidence of Mediterranean Fever among different ethnic groups. Johns Hopkins Med. J., 150: 22. 13 Schwabe, A.D. and Monroe, J.B. (1988) Meningitis in Familial Mediterranean Fever. Am. J. Med., 85: 715-717. 14 Rieger, E.H., Halasz, N.A. and Wahlstrom, H.E. (1990) Colchicine neuromyopathy after renal transplantation. Transplantation, 49(6): 11961198. 15 Kuncl, R.W., Cornblath, D.R., Avila, 0. and Duncan, G. (1989) Electrodiagnosis of human colchicine myoneuropathy. Muscle Nerve, 12: 360-364.
0
and Neurosurgery, 94 (1992) 261-263
1992 Elsevier Science Publishers
CLINEU
261
B.V. All rights reserved
0303-8467/92/S
05.00
00207
Case report
Pseudotumor
cerebri with familial Mediterranean
fever
Hamit Z. G(ikalp, Mustafa K. Baskaya and Varol Aydin Department
of Neurosurgery,
University of Ankara, (Received (Revised,
Familial Mediterranean
Ankara,
Turkey
1992)
received 4 May, 1992)
(Accepted
Key words~
16 January,
School of Medicine,
4 May, 1992)
fever; Headache; Pseudotumor
cerebri
Summary
Pseudotumor cerebri (PC) is a condition that occurs predominantly in obese women, and long lists of putative causes and associations have been reported. We describe here the case of a woman in whom PC coexisted with familial Mediterranean fever (FMF). A review of the literature revealed no report of an association of these two conditions.
erature, we thought the coexistence of these two conditions interesting.
Introduction
Pseudotumor cerebri (PC) is usually seen in females and is characterised by increased cerebrospinal fluid (CSF) pressure. It is a well defined syndrome of increased intracranial pressure with normal or decreased ventricles and normal CSF content. Increased intracranial pressure causes headache, dizziness, nausea-vomiting, tinnitus, and visual disturbances. The mean annual incidence is l/l00 000 in the general population [1,2]. It is seen frequently in females, especially when obese and in the reproductive period. In this group, the incidence of PC is reported as 19/100 000 [1,2]. Many diseases and conditions are reported to be aetiological factors of PC, but the exact aetiology and pathogenesis are still unknown. In our case, no disease or clinical condition was found that could cause PC. As PC with familial Mediterranean fever (FMF) has not previously been reported in the lit-
Correspondence to: Professor Neurosurgery, Hospital),
University
Dr. Hamit
of Ankara,
06100 Samanpazarl,
Ankara,
2. Gbkalp,
Department
Ibn-i Sina Hastanesi Turkey.
of
(Avicenna
Case report
The patient, a 22-year-old female, had been treated with colchicine for the past 1.5 years for FMF. When admitted to the Neurosurgery Department, she complained of headache, nausea and vomiting. She characterised her headache as generalized, getting worse and worse with time, and as being unresponsive to analgesics. Her history showed no remarkable disease apart from FMF. She did not describe any past infections, menstrual disturbance, pregnancy and lactation or anything else that might cause PC. The diagnosis of FMF was made after the observation of recurrent attacks of abdominal pain, fever, and leucocytosis by the Department of Internal Medicine. She had taken only colchicine 1 g daily for the past 1.5 years. Vital signs were normal and a physical examination revealed no abnormal findings. Her height was 155 cm and her weight 45 kg. Her mind was clear and co-operation and sense of orientation were intact. Fundoscopy
262 revealed
a bilateral
papilloedema.
were intact bilaterally.
nial and spinal nerve functions. laterally
normal.
functions
Visual field and acuity
There was no deficit in other cra-
There
Cerebellar
tests were bi-
was no neck stiffness.
Cortical
were also normal.
On lumbar
puncture,
CSF pressure
was 270 mm HzO,
and clear; analysis
for total protein,
glucose,
gave nor-
chloride,
and im-
munoglobulin. Urinalysis, phosphate), hormone
serum liver
electrolytes and
(Na’,
kidney
levels, and serum vitamin
Complete erythrocyte
blood
count
sedimentation
(CBC),
K’.
function
Ca”,
tests,
Cl-, serum
A level were normal. peripheral
rate were normal.
smear,
and
Serum iron,
iron finding capacity, ferritin level, serum folate and vitamin B,? levels were within normal ranges as was aspirated bone marrow. Cranial CT revealed no space-occupying lesion, oedema, or other abnormal findings. DSA showed no vascular abnormalities or sinus thrombosis. Methyl prednisolone (60 mg) and furosemide (80 mg)
The onset of symptoms
other disease processes,
surgical
tion
or withdrawal
of various
and
diet
In most
[14,6,8].
mechanism
and the CSF was colourless mal values
ual’s weight.
can be related
procedures,
to
administra-
drugs
and medications,
patients
no predisposing
can be found and the syndrome
must be con-
sidered to be idiopathic. Patients with PC require aggressive medical management. Generally. diuretics are the drugs of first choice, such as furosemide
and acetazolamide
production
11. Steroids
[4,5,8-l
lone and dexamethasone bination
with diuretics
remission therapy
within
CSF
such as methyl predniso-
are often used alone or in com[4,5,8810]. Steroids often induce a
a week;
has numerous
which inhibit
however,
long-term
steroid
side effects and may actually
in-
crease the chance of loss of vision by increasing intraocular pressure, and it should therefore be used with caution and only in 2-week periods [8]. Our case was treated with a combination of steroids and diuretics for 10 days. During that period her headache improved. Examination of visual acuity and field. repeated several times during that period. proved to be normal.
were given daily for 10 days. Colchicine was not withdrawn during the treatment of PC. CSF was drained
Repeated lumbar punctures frequently result in the relief of headache [4,5,8810]. In our case lumbar punctures
daily by lumbar puncture for 10 days to relieve headache and intracranial pressure decreased day after day. the last measurement being 120 mm H,O. The CSF was clear and colourless. The bilateral papilloedema improved. During her stay in hospital laboratory analyses were repeated and remained within normal ranges. The patient was discharged from hospital on day 20 without headache and papilloedema and with visual field and acuity
were performed daily to drain off CSF. FMF is a heredofamilial disorder of unknown pathogenesis. probably metabolic, characterised by recurrent episodes of abdominal or chest pain, fever and leucocytosis [12]. It is usually restricted to people of Mediterranean ancestry, primarily Armenians, Sephardic Jews and Arabs, and to some extent to people of Egyptian-Arabic origin living in Turkey. Greece, and Italy [12]. The disease suggests surgical peritonitis, but the acute attacks are recurrent, self-limiting, and not fatal [12,13]. Menin-
intact. On check-up neurological were within the patient
3, 6 and 9 months
later. systemic
and
examinations as well as all laboratory tests normal limits. At the control examination described
no further
attacks
of FMF.
Discussion Pseudotumor
cerebri
is a syndrome
of increased
in-
tracranial pressure without tumour, hydrocephalus or evident brain oedema. Moreover, PC is a diagnosis of exclusion; routine radiological and laboratory tests serve to eliminate other diagnostic possibilities [1,335]. In children and adults. headache, dizziness, nausea-vomiting, tinnitus, and visual disturbances are the symptoms seen most frequently [l-3,6-9]. PC is seen mostly in women. Most patients are obese and the activity of the disease may vary with the individ-
geal involvement has not widely been recognised as a manifestation of FMF. although occasional reports of meningeal irritation, meningism, and open meningitis have appeared in the literature [13]. However, FMF has so far not been reported in association with or as the cause of PC. Many series in the literature involving various numbers of cases have been evaluated to find the aetiological factor of PC and the coincidence of PC with FMF, but no evidence was found for either nor for a relationship that might shed light on the pathogenesis. In our case, despite the lack of an aetiological factor related to FMF, pre-treatment with colchicine for 1.5 years is remarkable. In the literature neuropathy and myoneuropathy have been reported as side effects of colchicine treatment, but so far it has not been suggested as an aetiological factor
of PC
[14,15].
263 References 1 Ireland, B., Corbett, J.J. and Wallace, R.B. (1990) The search for causes of idiopathic intracranial hypertension. A preliminary case-control study. Arch. Neural., 47: 3 15-320. 2 Durcan, F.J., Corbett, J.J. and Wall, M. (1988) The incidence of pseudotumor cerebri. Population studies in Iowa and Louisiana. Arch. Neurol., 45: 875-877. 3 Digre, K.B. and Corbett, J.J. (1988) Pseudotumor cerebri in man. Arch. Neurol., 45: 866-872. 4 Sklar, F.H. (1985) Pseudotumor cerebri. In: Wilkins, R.H. and Rengachary, S.S. (Eds.), Neurosurgery, vol. I, McGraw- Hill, New York, NY, pp. 350-353. 5 Corbett, J.J. and Thompson, H.S. (1989) The rational management of idiopathic intracranial hypertension. Arch. Neurol., 46: 1049-1051. 6 Sorensen, P.S., Kgogsaa, B. and Gjerris, F. (1988) Clinical course and prognosis of pseudotumor cerebri. A prospective study of 24 patients. Acta Neurol. Stand., 77: 164-172. 7 Round, R. and Keane, J.R. (1988) The minor symptoms of increased intracranial pressure: 101 patients with benign intracranial hypertension. Neurology, 38: 1461-1464. 8 Bowman, M.A. (1987) Pseudotumor cerebri. Am. Fam. Phys., Jan.: 177-182.
9 Greer, M. (1990) Pseudotumor cerebri. In: Youmans, J.R. (Ed.), Neurological Surgery, vol. V, 3rd edn., W.B. Saunders Co., Philadelphia, PA, pp. 35143530. 10 Jefferson, A. and Clark, J. (1976) Treatment of benign intracranial hypertension by dehydrating agents with particular reference to the measurement of blind spot area as a means of recording improvement. J. Neurol. Neurosurg. Psychiat., 39: 627-639. 11 Lyons, M.K. and Meyer, F.B. (1990) Cerebrospinal fluid physiology and the management of increased intracranial pressure. Mayo Clin. Proc., 65: 684707. 12 Pras, M., Bronshpigel, N., Zemer, D. and Gafni, J. (1982) Variable incidence of Mediterranean Fever among different ethnic groups. Johns Hopkins Med. J., 150: 22. 13 Schwabe, A.D. and Monroe, J.B. (1988) Meningitis in Familial Mediterranean Fever. Am. J. Med., 85: 715-717. 14 Rieger, E.H., Halasz, N.A. and Wahlstrom, H.E. (1990) Colchicine neuromyopathy after renal transplantation. Transplantation, 49(6): 11961198. 15 Kuncl, R.W., Cornblath, D.R., Avila, 0. and Duncan, G. (1989) Electrodiagnosis of human colchicine myoneuropathy. Muscle Nerve, 12: 360-364.
