Clinical Neurology


and Neurosurgery, 94 (1992) 261-263

1992 Elsevier Science Publishers



B.V. All rights reserved




Case report


cerebri with familial Mediterranean


Hamit Z. G(ikalp, Mustafa K. Baskaya and Varol Aydin Department

of Neurosurgery,

University of Ankara, (Received (Revised,

Familial Mediterranean




received 4 May, 1992)


Key words~

16 January,

School of Medicine,

4 May, 1992)

fever; Headache; Pseudotumor



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.


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),


Dr. Hamit

of Ankara,

06100 Samanpazarl,


2. Gbkalp,


Ibn-i Sina Hastanesi Turkey.



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


were intact bilaterally.

nial and spinal nerve functions. laterally



Visual field and acuity

There was no deficit in other cra-



tests were bi-

was no neck stiffness.


were also normal.

On lumbar


CSF pressure

was 270 mm HzO,

and clear; analysis

for total protein,


gave nor-


and im-

munoglobulin. Urinalysis, phosphate), hormone

serum liver

electrolytes and



levels, and serum vitamin

Complete erythrocyte









Cl-, serum

A level were normal. peripheral

rate were normal.



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,



or withdrawal

of various



In most



and the CSF was colourless mal values

ual’s weight.

can be related





and medications,


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


11. Steroids


lone and dexamethasone bination

with diuretics

remission therapy



such as methyl predniso-

are often used alone or in com[4,5,8810]. Steroids often induce a

a week;

has numerous

which inhibit




side effects and may actually


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


examinations as well as all laboratory tests normal limits. At the control examination described

no further


of FMF.

Discussion Pseudotumor


is a syndrome

of increased


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


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.

Pseudotumor cerebri with familial Mediterranean fever.

Pseudotumor cerebri (PC) is a condition that occurs predominantly in obese women, and long lists of putative causes and associations have been reporte...
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