SUMMARY

Four cases of surgically verified » primary empty sella » or « intrasellar arachnoidocele » in the black Eastern African, are reported. This four cases are the 3.5 per cent of all sellar and parasellar tumours (113 patients) operated in 25 years time. All four cases were females (45, 16, 31 and 36 ycras old) and all of them had severe visual and visual field impairment. Two patients had radiologically a very enlarged sella and one of them had a severe rhinorrhea. Two cases had a balloon-shaped sella. Two cases have been diagnosed pre-operatively as pituitary tumours, while the other two cases have been diagnosed as arachnoiditis of the chiasma and optic nerves. The C. T. Scan performed in three cases was not informative on the nature of the lesion. The operation was carried out through a right frontal craniotomy and the finding were typical of « primary empty sella ». In two patients the vision lias improved post operatively, has remained unchanged in the other two cases.

Keywords: Africa, Empty Sella, Pituitary, Afrique

RESUME

Les auteurs rapportent 4 cas d’arachnoidocle intra-sellaire ou « selle turcique vide » observes chez des patients noirs originaires d’Afrique de l’Est. Ces 4 cas représentent 3.5 % des 113 tumeurs sellaires et parasellaires opérees en 25 ans. Les 4 patients étaient des femmes agées de 16, 31, 36 el 45 ans. Elles avaient toutes une amblyopie importante et une altération du champ visuel. Chez 2 d’entre elles, dont une avait une rhinorhee importante, la selle turcique était élargie sur les radiographies du crane. Les 2 autres avaient une selle en forme de ballon. Dans 2 cas, le diagnostic pré-opératoire avait été celui d’une tumeur pituitaire, et dans les 2 autres celui d’une arachnoidite du chiasma et des nerfs optiques. Le scanner realise dans 3 cas n’avait fourni aucune information sur la nature de la lésion.Une craniotomie frontale droite a objectivé une « selle turcique vide » typique. Chez 2 patients, la vision a été améliorée après l’intervention. Chez les 2 autres, il n’y eu aucun changement.

Mots cle: Hypophyse, Selle turcique vide

DEFINITION AND CLASSIFICATION

The term « primary empty sella « P.E.S » or « intrasellar arachnoidocele » defines an anatomical condition in which the sella turcica is mainly occupied by an arachnoid diverticulum, containiiig cerebro-spinal fluid whilst the abnormally small pituitary gland appears flattened against the infero-posteriorwall of the sella. An inherent feature is the absenxe or extreme incompetence of the diapliragma sellae.
First described by BUSCH in 1951, it was a chance autopsy finding. A later study of 788 autopsies demonstrated this finding in 5.5 percent of cases. Careful study showed altered volume relationship between pituitary and sella turcica in another 19 per cent of cases. The relationship was normal in only 75 percent of cases.
At the same time other Authors reported similar anatomical conditions in patients operated for pituitary tumours or treated with radiotherapy for pituitary tumours. This condition caused a delayed visual deterioration without recurrence of tumour and has been called « empty sella syndrome ».Other Authors described true cystic formation into the sella, simulating the « empty sella ». Complete different anatomical and pathological conditions were thus grouped under the finition of « empty sella » and therefore, later on, a classification of the various conditions creating an « empty sella » has been suggested. The various types of empty sella can be classified as follows:

A) Primary empty sellar or intrasellar arachnoidocele, partly on a congenital basis.

B) Secondary types of empty sella, due to pituitary infarction, atrophy or spontaneous necrosis of pituitary tumours. Pituitary surgery or radiotherapy.

C) Benign intrasellar cyst, which presents typical anatomo-clinical, radiological and surgical features.
Our report is on four cases of « primary empty sella » or « sellar arachnoidocele » corresponding to the first description of BUSCH, and observed in black Eastern African patient.

PYSIOPATHOLOGY OF THE PRIMARY EMPTY SELLA

The « primary empty sella » or « intrasellar arachnoidocele » represents an abnormal dilatation of the mfradiaphragmatic cistern, detrimental to the pituitary gland. A necessary anatomical prerequisite for its formation is an « incompetent » diaphragma sellae, of BUSCH’S third type which is found in normal subjects
with a frequency varying between 5.5 and 10 per cent. To explain the pathogenetic mechanism causing the formation of the primary empty sella there are various hypothesis. A first hypothesis, BUSCH 1951, is that a congenitally incompetent diaphragm allows the arachnoid to descend below the diaphragm, alongside the pituitary stalk. Communicating freely with the suprasellar cystems it is intensely pulsatile and this pulsations causes a progressive enlargement of the arachnoid diverticulum and consequent damage to the pituitary gland. Two factors argue against this hypothesis: Firstly the frequency of the empty sella in only the half of cases with incompetent diaphragm; secondly the primary empty sella is much more frequent in the females whereas variations in the diameter of the diaphragm are unrelated to sex.

In the second hypothesis, formulated by KAUFMAN in 1968, increased intracranial pressure ( as in the pseudo tumour cerebri or benign intracranial hypertension) causes hemiation of the arachnoid via the congenitally incompetent diaphragm into the sella turcica. Whilst it is more likely that an empty sella may be formed when there are associated incompetent diaphragm and increased intracranial pressure, it is unlikely and statistically improbable that this mechanism explains the majority of the cases of primary empty sella. A third hypothesis, which nowadays seems the more tenable, is related to the developmental alterations of the pituitary and of the sella. After the first structural changes that occur between birth and about four years of age, the sella remains unchanged until puberty, when progressive enlargement take place until the adult shape is reached. Knowing that the primary empty sella is far more frequent in females and that the pituitary in females is larger than in males, and that the pituitary increases distinctly during pregnancies, it is likely that increased pressure within the pituitary fossa, more than pressure above it, can cause the diaphragm to atrophy. With the volume of the pituitary increasing and decreasing in the females, it is understandable that if there is a congenital wide opening of the diaphragma sellae, there will be a larger space into the infradiaphragmatic cystern such to lead, with the successive pregnancies and subsequent athropy of the diaphragmatic orifice, to the primary empty sella formation. Primary empty sella would be therefore not so much an antomica! variant but the outcome of complex, progressive, paraphyssological phenomena involving mainly the pituitary gland and secondarily the structures surrounding ihe pituitary gland and secondarily the structures surronding the pituitary (•sella, diaphragma sellae, arachnoid, cerebro – .spinal fluid)

SYMPTOMATOLOGY

Typicaiiy the clinical syndrome includes headaches, visual disturbances, obesity with or without disfunction
signs in young to middle aged women, more frequently women with several pregnancies.

Neurological signs Headache is the most frequent symptom and is found in about 50 per cent of cases. The
hedaches is usually fontal or retrobulbar, severe, continuous. deep. often intractable. Seizures has been reported together with other neurlogical signs from time to time. but too infrequent to be characteristic of the primary empty sella. Visual disturbances are quite frequent and the decrease in visual acuity are often symmetrical and can occur acutely with sudden blindness. Primary optic atrophy is often present. Visual field defects are more irregular, asymmetric, sometime with concentric peripheral constriction.

Diencephalic signs Obesity is quite common, whilst narcolepsy, PICKWICK syndrome, poliuria and polydipsia are rare.

Pituitary endocrine disfunction . Endocrine manifestation vary from case to case, with variable fequencies of presentation. Panhypopituitarism, thyroid insufficiency, adrenal cortex insufficiency, amenorrhoea and various other hypo or hyper pituitary functions has been described. It has become evident that the fault lies with the hypothalamic-pituitary regulation. This is the only constant finding and there is no other endocrine symptomatology characteristic of the primary empty sella.

CSF rhinorrhoea Rarely some cases present with the serious symptoms of cerebro – spinal rhinorrhoea which may be accompaned by recurrent episodes of meningitis. The CSF fistula is usually located in the antero-inferior wall of the sella, which is both a zone of less resistance and also the area most affected by the pulsatile action of the arachnoid diverticulum.

RADIOLOGICAL FEATURES

It is apparent that the diagnosis of primary empty sella on clinical data alone is rather problematic. The symptoms are too variable to provide a diagnosis and therefore neuroradiological investigations are essential.
Plains films of the skull and tomography of the sella may provide useful informations. Many types of alteration of the sella have been described, the most usual changing being its enlargemet. This is generally mild or moderate and take place in all directions, causing ballooning. It is difficult that bony changes alone are enough to differentiate between an empty sella and a pituitary adenoma. An empty sella of normal size is fairly common (BERNASCONI et al 1972).

The computerized tomography of the head should be helpful in the differentia] diagnosis between empty sella and pituitary tumour: for reliable results a study must include magnified axial and coronal planes, with densilometry of inlrasellar structures. Sometimes the herniation of brain tissue into the sella makes the diagnosis of empty sella difficult also with the CT Scan. The positive contrast eysternography combined with computerized tomography may be the investigation of choice in this cases (HOFMAN et al. 1980).

TREATMENT

Although much progress has been made in understanding (lie various problems associated with the primary empty stella. it is still under discussion which method of treatment produces the best results.

In asymptomatic cases, where the intrasellar arachnoidocele is a chance finding, and in cases with minor clinical signs such as headache and obesity, surgery is contraindicated. At the same time, however, surgery will almost always relieve headaches, by decrasing the intrasellar pulsatile pressure. It may therefore be convenient to operate in cases where the headache becomes unbearable and cannot be relieved by medical treatment. Surgery is indicated when there is either a CSF rhinorrhoea or impaired vision, is debatabel in cases of hypotituitarism. Cerebro spinal fluid rhinorrhoea is an indication for urgent surgery, especially when there have been episodes of recurrent meningitis. The repair of the CSF fistula, usually located in the antero – inferior wall of the sella, is very successful and the operation can be carried out through a frontal approach or through a transphenoidal route.

Visual disturbances are not always reversible, despite effective treatment. Established visual field defects and acuity less rarely improve following surgery, but their progress is usually halted and this of itself is a sufficiently good result to he an indication for surgery.
A similar argument can be made for an empty sella associated with partial or complete hypopituilarism. Surgical removal of the arachnoidoccle not only relieves pituitary compression and stretching of the infundibulum and the hypothalamus. but at the same time, it ends the effects of the pulsatile pressure of CSF on the pituitary. In this case indications lor surgery are not clear cut, because the hormonal disorder may be not progressive and the post operative recovery of pituitary function is not necessarily assured.

Surgery via upper route allows chiasmapexy by filling the sella with muscle or with gelfoam or with silicone. The upper route also allows the optic structures ot be freed from arachnoidal adhesions if present, hi cases with rhinorrhoea. the upper route can be useful for identifying cxtrusellar fistulas. Surgery through the transplienoid.il or lower route aims at chiasmapexy and removal ol the arachnoid divcrliculum by filling in the residual sellu cavity, by cither an intradural or extradural approach.
In agreement with many oilier Authors, it is believed that the traJisphenoulal route is preferable if the primary empty sella can be diagnosed preppcratively, maily because of same results causing less trauma.

CASE REPORTS

Case 1

Z.A. 45 years old female. Had her first period at the age ol’twelve lor only 6 months. No dcvelopmctn of secondary sexual characters. In 1976, 10 years before admission suirtcd compalining of headaches, loss of vision in the left eye. Two yc-.’rs before admission started left sided rhinorrhea with several episodes of meningitis. The neurological examination showed that she was blind in the left eye. Hail a temporal hemianopia in the right eye. Bilateral primary optic atrophy. She was obese, had an infantile breast, infantile genitalia, lack of pubic and axillary hairs. The plain x-rays of the skull showed a very enlarged primary sella. Tlie left carotid angiography was of no diagnostic help. Endocrine evaluation disclosed hypopiluitarism and hypothyroidism. At operation (transfrontal approach) the sella was found filled up with CSF with a dural defect in the anterior – inferior aspect of the size of 1 mm., the site of the CSF leak. With the aid of the microscope the dural defect wa repaired and the sella filled up with muscle.She was dicharged on endocrine replacemnt therapy. She had an improvement of the visual acuity in the right eye persisting one year after surgery when she was last seen.

Case 2

B.M. 16 years old female. At the age of 12 years the normal growth arrested. No development of secondary sexual characteristic. Amenorrhea. Two years before ad- mission she started complaining or retrorbital headache and loss of vision in the left eye. On examination she had an infantile aspect. Infantile breast, infantile genitalia, lack of pubic and axillary hair. Bilateral primary optic atrophy. Can see the movements of the hand with the left eye nasally. Can count the fingers with the right eye, better nasally. Endocrine evaluation disclosed hypopituitrism. The skull x-rays showed mild ballooned sella. The C.T. Scan was positive for pituitary tumour. At operation (transfrontal approach) the sella appeared filled up with CSF, the pituitary flattened postero – inferiorly, brain hemiation into the sella. The sella is filled up with muscle. No post – operative treatment was given. She had no visual improvement and at the check up nineteen months later the visual situation was unchanged.

Case 3

W.N. 31 years old female. Periods at the age of 12. Married with 5 children. Started complaining of headaches and irregularity of her periods followed by loss of vision in the left eye, 2 years before admission. On examination she had primary optic atrophy in the left eye with loss of vision but no visual field defect. Slight obesity. Endocrine evaluatin showed a enlarged primary sella. The CT Scan was not informaive of the diagnosis. At operation (transfrontal approach) the sella appeared filled up with CSF. The sella was filled up with muscle. No post – operative treatment was given. The visual acuity was not changed six years alter surgery.

Case 4

M.M. 36 years old female. Since three years severe progressive headaches followed by loss of vision and amenorrhea. Has had four children. On examination, obese, is blind in the right eye. can see hand movements in the left eye. Primary optic atrophy more in the right eye. Endocrine evaluatin showed hypotituitarism and hypothyroidism. The plains skull xrays showed only slight ballooning of the sella. The C.T. scan was normal. At operation the sella was filled up with CSF with typical findings of primary empty sella. The sella was filled up with muscle. She was discharged on the endocrine replacement therapy.

She had an improvement in the visual acuity immediately after surgery in the left eye that persisted up to one year after surgery when slie was last seen.

DISCUSSION

In all our cases the main symptoms were headache, severe visual disturbances and hypothalamic – pituitary disfunction with obesity and hypotituitarism. CSF rhinorrhoea with recurrent mengingitic episodes was present in one case. No epilepsy has been recorded. Contrary to many other reports of primary empty sella with no visual involvement and often no hypopitutarism or other neurological signs and symptoms, our patient had all the full picture of the sellar lesion .with severe visual and pituitary disfunction. This most probably has been the cause for the referral of this patients with a diagnosis of pituitary tunour. In fact the preoperative diagnosis has been of pituitary tumour. In two cases (case one and case two) and optochiasmatic arachnoiditis in the other two cases (case three and case four).

The plain x-rays showed a primary sella in two cases, and ballooning of the sella in other two cases. The C.T. Scan was normal in two cases and showed a possible pituitary tumour in one case. In the first case, only angiography was performed and was reported normal. Certainly the positive contrast cysternography combined with C.T. Scanning may offer better informations in the P.E.S. cases.
The operative findings were in all cases typical for P.E.S. with almost absent diaphragma sellae with brain hcmiation into the seila in two cases. In one case the CSF fistula was located antero-inferiorly. All cases were explored through a transfrontal approach: the transphenoidal approach may be better in case of correct , preoperative diagnosis.

The post operative course was uneventful in all cases. Two cases had an improvement, but no deterioration following surgery at the follow up one and six years later.
The P.E.S has been found to have an incidence of 3.4 per cent of all cases of sellar and parasellar tumours operated by one of the Authors.

SUMMARY

A review of 139 histologically verified intraspinal tumours in Black Eastern Africans who were operated upon in Nairobi. Kenya in the last 25 years, is presented. Intraspinal tumours in this series are compared with other studies from Africa and elsewhere. Extradural and intramedullary tumours together with cervical spine tumours appear to be more frequent in this series. There is a high incidence ofdumbell tumours in the neurinomas. Sarcomas are the most common type of tumours and mainly affect he thoracic spine

Keywords : Africa , Spinal cord, Spine, Tumours

RESUME

139 cas de tumeurs intrarachidiennes observées chez des noirs de 1’Afrique de l’Est à Nairobi (Kenya), ont été opérées durant 25 ans, et confirmées histologiquement, Les tumeurs intrarachidiennes sont comparées à celles d’autres études publiées en Afrique et dans d’autres pays. Les tumeurs à la fois extradurales et intramédullaires et les tumeurs du rachis cervical sont plus fréquentes dans cette série. Il existe une incidence élevée de tumeurs en forme d’haltère parmi les neurinomes. Les sarcomes sont les tumeurs les plus fréquentes et affectent surtout le rachis dorsal.

Mots cles : Afrique, Tumeurs intra-rachidiennes, Compression médullaire, Rachis

A series of 139 Black Eastern African patients have been treated surgically for intraspinal tumours (I.S.T.) in the last 25 years, in Nairobi, Kenya. This series does not include vascular malformation, parasitic or infectious lesions nor arachnoditis. The ratio between intracranial tumours (605 cases) and I.S.T. (139 cases) in this series is 4.35: 1, with a higher incidence of intraspinal tumours when compared with other larger series, as ofBROAGER, 1953 (10.1) or MALECI, 1954 (8:1). The higher incidence of intraspinal tumours in our series may be due to a higher mortality rate of intracranial tumours before any treatment. (RUBERTI et al. 1976) The relative incidence of various histopathological types of these tumours compared in percentage with other African and Western series was as follows:

Table 1 : Site of Intraspinal Tumours (in percentage)

Our series compares similarly to the one of SOROUR et al, Egypt 1970, and to the overseases series in regard of site ofI.S.T. In the series ofODEKU et al, Nigeria 1976, and GARRIDO et all Zimbabwe 1985, there is a higher percentage ofextradural tumours (ODEKU et al 80.3%, GARRIDO et al 62.3% ) and consequent relative lower incidence of intradural tumours, although the main causes were the same i.e meningiomas and neurinomas. The incidence of intramedullary tumours coincides more or less with all other series.

There is a high incidence ofI.S.T. betweeen the age of 11- 50 years (about a equal distribution in the 2nd, 3rd and 4th decade of life) instead of being between 35-55 years of age as in the large overseas series.

In the gliomas series, the astrocytoma is the most common histopathology found, (18 cases, 82%), with only a few ependymonas (4 cases or 18%). The metastatic tumours are all hystopathologically carcinomas: 2 bronchial, 2 prostate, 1 breast, 1 thyroid, the rest undentified carcinomas. Out of 10 metastasis 9 are located in the thoracic and one in the lumbar region.
The age incidence oflntraspinal tumour in this series is shown in table 2:

Table 2 : Age distribution of intraspinal tumours

In our series there was a high incidence of sarcomas and gliomas in the first two decades of life. In the third and forth decades there was a prevalence of neurinomas and meningiomas, followed by gliomas and sarcomas. In the fifth and sixth decades there was a prevalence of meningiomas and nerurinomas followed by sarcomas and metastatic tumours almost in equal number. Over the age of sixth we fiound only two sarcomas and one metastatic tumour.
There has been a tendency to malignancy (gliomas and sarcomas) in the young age group compared with the middle and old age group were benign (menigiomas and neurinomas) are prevalent.

The sex incidence of I.S.T. in this series as shown in Table 3:

Table 3 : Sex incidence of intraspinal tumours

There is a prevalence of males over females with a ratiomale – female of 1.4:1, Males are 81 cases, 58.2 per cent, females are 58 cases, 41.8 percent, but the difference is not as marked as per mtracranial tumours. It seems that malignant tumours (gliomas, sarcomas and metastatic tumours) affects the male more than the female, while beningn tumours are preferential in the female The preferential site of I.S.T. in the Eastern African patients was as follows:

Table 4 : Preferenctial spinal location of I.S.T.

There is a prevalence of benign tumours in the cervical spine with a high incidence of neurinomas and meningiomas; also the gliomas are frequent at this level The thoracic spine is affected more by sarcomas, with a. lower incidence of benign tumours, hi the lumbar spine there is again a prevalence of benign tumours, 11 between neurinomas and lipomas against 5 sarcomas and metastases.
Two interesting points emerge from these series: the relatively higher incidence of the cervical tumours compared with those of the thoracic tract (in larger series the incidence is largely in favour of tumours of the thoracic tract), and the contrast between the liigh incidence of benign tumours of the cervical spine compared with the high incidence of malignant tumours of the thoracic tract.

DISCUSSION

Tlie incidence ofintraspmal tumours compared with intracranial tumours in higher this series than in larger over seases series Tlie age distribution of I.S.T. is almost equal in the various decades of life from the age of ten to the age of fifty, instead of being 35 and .55 years of age as in other large series: this may be certainly due to tlie large, young population in this part of the world This study correlates with various others in sex incidence and suggest that I.S.T are prevalent in males than in female
The younger age group appear to be affected more by malignant tumours (sarcomas and gliomas) while the middle and older age group seem to be affected more by benign tumours (neurinomas and meningiomas) Intraspinal tumours appear to affect males more than females. Malignant tumours (gliomas, sarcoma, and metastases are more frequent in the males, whilst benign tumours have a higher incidence in the females (meningiomas and neurinomas)
Extradural, intramedullary tumours as well as the cervical spine tumours have a higher incidence in this series. The cervical and lumbar spine are mainly site of benign tumours, while in the thoracic spine there is a high incidence of malignancies . A high percentage. (50 percent ) of dumbell tumours have been found in the neurinomas.
The use of an operating microscope has proven to be very useful in reducing the trauma due to the removal the spinal cord tumours, particularly for the removal of intramedullary gliomas

I – From the President. Professor Dr Armando Basso

Last October, I was honoured with the Presidency of the WFNS. On such an occasion, I stressed that the WFNS would not just be an institution for the organization of Congresses but would be transformed into an organ of expression for all the problems that the performance of our speciality shows us world-wide. To put this project into practice, we agreed that the essential ideas thai we wanted to work with, would be the following:

1. Constitute a solid working team to co-odinate the work of all Committees;

2. Promote frequent meetings of the Administrative Council and the members of other Committees in different parts of the world;

3. Designate more responsibility to the continental Vice-Presidents so that they become actively involved in the problems of their region;

4. Open Ad-Hoc Committees for all neurosurgeons who can contribute new ideas to improve the performance of the Federation;

5. Continue the work that our last President, Lindsay Symon started concerning Education, promoting the achievement of minimum standards and the certifications necessary to practice neurosurgery In different areas of the Federation;

6. Promote, through our Central Office in Geneva, the relations with International Health Institutions so that, with our co-operatin, more information of our speciality can reach (he general public, for instance, campaigns to proven! Craniospinal trauma, Cerebro vascular accidents and many others; and

7. Work together with all (he National and Continental Society members, as well as those of (lie Federation in order to establish standards in subjects such as neuronal transplanation, prosthesis utilization, health costs etc., which should become items to be discussed by the different Committees and supervised by the Administrative Council.
We are already fully concentrating on all of these items, however, it is necessary that all the members of the Societies of Ihe Federation feel that they too, are participating in this Programme and that they can reach US directly or through our Central Office in Geneva, in order that the WFNS, which is everybody’s Federation, can really have a permanent feed-back of ideas and so that we may work together to solve the problems, we neurosurgeons around the world, have in common.

II – Acapuico Congress

The 10th International Congress of the World Federation of Neurosurgical Societies took place in Acapulco, Mexico and was a resounding success. It was outstanding with regard to its scientific content, the number of attendees (which set a new record of 3,154: 1,654 participants, 228 residents, 497 accompanying persons, 482 exhibitors and 293 staff) and the superb social events arranged by the host Society. Sincere congratulations and a great deal of appreciation go to the Mexican Neurosurgical Society, the organizers of this Congress, and everyone who worked so hard to make it such a memorable event in (lie world of Neurosurgery. The Congress was also financially sound, returning more than $150.000 lu the Treasury of the World Federation. These monies are essential for the continuing activities of the Federation, particularly in planning educational courses which will occur during the period before the next Congress.

III – The 11th International Congress of the WFNS in Amsterdam – July 6-11 1997.

The Netherlands Society of Neurosurgeons is very pleased to announce that the XI International Congress of Neurological Surgery will take place under the High Patronage of Her Majesty Queen Beatrix of the Netherlands from 6-11 July 1997.

An outline of the Scientific programme will be included in the Congress First Announcement which will be mailed at the end of 1994. If you would like to receive a copy please contact the Congress Secretariat. Technical exhibits will run in parallel to the Congress. Companies and organizations wishing to participate should contact the Congress Secretarial for more information.

Amsterdam

The Congress Centre is located whithin the city of Amsterdam at a short distance from Schipol International Airport. Amsterdam is famous for its historical inner city criss-crossed by an extensive network of canals lined with picturesque 17lh century buildings . The city boast a wealth of museums and has gained a world-wide reputation as a cultural centre for orchestral music, classical and modern ballet and opera. Not surprisingly, the colourful life-style of Ihe Dutch capital attracts visitors from all over Ihe world.

The Congress Secretariat:

Amsterdam RAI-OBA

PO Box 77777

1070 MS Amsterdam

The Netherlands

Tel: +31(0)205491212

Telefax: +31 (0)206464469

IV – Results of Elections at the Acapuico Congress

a). The officers elected by the Executive Committee of the WFNS at the recent Acapuico Congress consist of the following:

President

Annando Basso (Argentina)

Honorary

Mauro Loyo-Varela (Mexico)

Shozo Ishii (Japan)

Sean Mulian (USA)

Lsndsay Symon (United Kingdom)

First Vice-President

Kintomo Takakura (Japan)

Second Vice Presidents

Giampaola Cantore (Europe)

Kil Soo Choi (Asia-Australasia)

Sayed EI-Gindi (Middle East-Africa)

Gilberto de Almeida (Latin America)

Haroid Hoffman (North America)

Alexander Konovalov (at large)

Peter Jannetta (ai large)

Secretary

Edward Laws (USA)

Treasurer

Jacques Brotchi (Belgium)

Assistant Secretary

Maurice Choux (France)

Assistant Treasurer

Madjid Samii (Germany)

Editor of Federation Publication

John Pickard (United Kingdom)

Historian

Alphons Walder (Nertherlands)

President, XI International Congress August van Alphen (Netherlands)

b). Committee members elected in accordance with the Constitution and Bylaws consist of the following

Nominating Committee:

Chair: Dr F Ismat (Spain)

Vice Chair: Dr Leigh Atkinson (Australia)

Drs L Basauri (Chile). R Fahlbusch (Germany), R Hardy (USA) K Lee (Korea), J Humberto Mateos (Mexico), S Oheaeghulam (Saudi Arabia), J Peter (South Africa), Atos Alves de Sousa (Brazil).

Constitution and Bylaws Committee:

Chair:

Dr J Martin-Rodriquez (Spain)

Drs George Ablin (USA), R Pen-in (Canada), Gianfranco Rossi (Italy).

Liaison and Public Relations Committee:

Chair: Dr M Dujovny (USA)

Drs R Fahlbusch (Germany), M. Lazar (USA).

Audit Committee:

Drs F Rueda-Franco (Mexico), W. Koos (Austria), N de Tribolet (Switzerland).

c). Leadership of the other WFNS Committees for the next four years consists of the followings:

WFNS Education Committee

James Ausman, Chair

WFNS Material Aid Committee

Sean Mulian, Chair

WFNS Spine Committee

Russell Hardy, Chair

Committee on Skull-Base Surgery

Madjid Samii, Chair

Committee on Awards to Young Neurosurgeons

Alber Rhoton, Chair

Neurotraumatology Committee

George Dohrmann, Chair

Medals of Honour Committee

Lindsay Symon, Chair

Pacdiatric Neurosurgery Committee

Harold Hoffman, Chair

Stereotactic and Functional Neurosurgery Committee

Ronald Tasker, Chair

Neuro-oncology Committee

David Thomas, Chair

Scoville Award Committee

Russel Paterson, Chair

Materials, Drugs and Devices Committee

Yves Keravel, Chair

Ethics and Medicolegal Committee

Maximo Poza, Chair

Committee on Standardization of Terminology and Surgical Results

Robert Spetzler, Chair

Neurovascular Committee

Roberto Heros, Chair

Radiosurgery Committee

Landisiau Steiner, Chair

Neurosurgeons seeking more information about the activities of any of these Committees should feel free to
contact the respective Chair.

d). The logo of the WFNS, which you see displayed on this Newsletter, was formally adopted at the meeting in I Acapulco.

V – Call for Nominations

The Nominating Committee, under the Chairmanship of Dr Fabian Isamat of Barcelona, Spain, is listed above. In the Constitution and Bylaws, each Member Society has the privilige of submitting suggested names to be consid- ered for officers to be elected at the 1997 Congress in Amsterdam. These suggestions must be in the hands of the Nominating Committee before its final deliberations, which must be completed one year prior to the election. For that reason, member societies are encouraged to consider suggestions for the offices listed below and to sumit them in a timely fashion to the Chairman of the Nominating Committee or the Office of the WFNS in Geneva.

The absolute deadline for Nominations is May 6 1995.

Suggested are in order and are requested for the following offices.

President

1 st Vice-President

2(at large) 2nd Vice-Presidents

Secretary

Assistant Secretary

Treasurer

Assistant Treasurer

Editor of Federation Publications

Historian

Constitution and Bylaws Committee

Liaison and Public Relations Committee

In addition, each of the five continental organizations, namely the European Association ofNeurosurgical Societies, the Asian-Australasian Society ofNeurlogical Surgeons, the Pan-African Neurosurgical Society, the Federacion Latino Americana deNeurochirurgia. and the American Association of Neurological Surgeons, is asked to submit two nominees from each Continental Society for the position of 2nd Vice-president.

VI – WFNS Liaison Office in Geneva

The Liaison Office for the World Federation of Neurosurgical Societies is located at the World Health Organization and has been run in a most efficient fashion since its inception two years ago by the Executive Secretary, Mrs Jan Joseph. The mailing address for this office, its fax and phone numbers are as follows:

Mrs Jan Joseph, Executive Secretary

WFNS Liaison Office with WHO

Central Office, Room 309

Division of Mental Health – World Health Organisation

CH 1211 Geneva, 27 Switzerland

Tel: 41-22-791-3604. Fax:41-22-788-4329

Copies of the Minutes of the executive Committee and Administrative Council Meetings are available on request from the Central Office, as are reports from the various Committees and of the Acapulco WFNS Newsletter.

VII – Request for Proposals for the Site of the 12th International Neurosurgical Congress to be held in 2001

The selection of the site for the next Congress is an important factor, not only for the planning of WFNS activities, but also with regard to candidacies for President of the Federation. As the number of Neurosurgeons worldwide increases, the size and scope of the meeting will increase as well. Therefore, proposals from various members societies to host the 12th International Congress should be carefully considered and planned. The Executive Committee will formally vote on a site for the Congress at the Interim Meeting to be held in Berlin, Germany in conjunction with the meeting of the European Association of Neurosurgical Societies in May, 1995. proposals from Member Societies need to be submitted in a timely fashion, preferably at least one year in advance of the Berlin Executive Committee Meeting. Examples of previous proposals can be obtained from the Centra! Office of the Federation in Geneva, and Member Societies can use these as guidelines for constructing their own proposals. Please do not hesitate to contact Mrs Joseph in this regard. We look forward to receiving a number of exciting proposals for the site of this important World Congress.

VIII – Interim Meeting of the WFNS Executive Committee

The Interim Meeting of the WFNS Executive Commitee will take place in Berlin, Germany. It is hosted by the European Association ofNeurosurgical Societies with Mario Brock as President. The dates are May 6-12 1995. All delegates and Committee members should plan to attend.

SUMMARY

Seventy-three cases of injury to the spinal column are reviewed. Fifty percent (36) suffered bony injury without neurological damage. Road traffic accidents were the commonest cause. All patients did well. Twenty percent (15) suffered minor neurological damage, again road traffic accidents predominated as the cause by the bony injuries were more severe than in the first group. There was no mortality. Thirty percent (22) suffered bony injury and total paraplegia or quadriplegia., Eleven patients (50%) died in hospital, 9 being quadriplegic. The high mortality in these cases appears related to respiratory problems. It was noted that high cervical injuries (to the C1-C2 levels) rarely result in severe cord damage.

RESUME

Les auteurs ont revu 73 cas de lésions traumatiques de la colonne vertebrale:
– 50% (36) avaient une lésion osseuse, sans lésion nerveuse. Les accidents de la route en étaient la cause la plus fréquente. Tous ces patients étaient asymptomatiques.
– 20% (15) présentaient des troubles neurologiques mineurs. Il s’agissait pour la plupart d’accidents de la circulation. Les lésions osseuses étaient plus graves que dans les premiers groupes, mais il n’y a pas eu de déces.
– 30% (22) avaient une paraplegie ou une quadriplegie secondaire à des lésions osseuses.11 patients soit 50% sont décédés à I’hopital dont 9 etaient quadriplegiques. Cette mortalite élevée étaient liées à des troubles respiratoires. Le niveau lésionnel haut, C1C2 déterminait peu de lésions médullaires.

INTRODUCTION

We are presenting a review of 73 cases of trauma to the spinal column and cord which have been seen by us over the past few years. They fall into three groups, those without nervous system damage, those with minor neurological involvement and those with severe and usually permanent paraplegia or quadriplegia.

CASE ANALYSIS

1. Thirty-six (50%) suffered bony injuries without neurological damage. There were 26 males and 10 females. The ages ranged between 18 and 63 years and averaged 36 years.

Table 1

There was no difference by sex as far as as age distribution or causation was concerned except that both the sack and explosion injuries occurred in men. (Table 1). Most of the bony injuries were of the type with which a better prognosis is associated.

Table 2

The injuries were located as follows:

Table 3

These were treated conservatively or by anterior cervical fusion if the subluxation or minor dislocations appeared unstable. All patients did well.

2. Fifteen patients (20%) (13 males, 2 females) suffered minor neurologival damage. This ranged from weakness of one arm associated with overriding of a facet joint to more severe compressions than we saw in the previous group and are what we can only consider to be, lucky escapes. The average age was 38 years with a spread from 12 years to 60 years.

Table 4

The causes were very similar to those of the “no cord damage” group. However the nature is more severe. There were high cervical injuries.

Table 5

The compression were more severe and the dislocations more obvious than in the former group.
They were located as follows:

Table 6

All the patients with the high cervical lesions and two others had recovered by the time of discharge. Unfortunately we do not have a follow-up on the others at this time.

3. The third group comprises those 22 patients whose injuries were severe enough to render them totally paraplegic and quadriplegic. The average age was 31 years with a spread of 3 years to 70 years. Falls predominated over road traffic accidents

Table 7

There were none of the lifesaving C1-C2 level injuries and the dislocations and compressions were much more serious than in the previous group.

Table 8

(Note: there were no C1-C2 injuries)

They were located as follows:

Table 9

Eleven patients died in hospital, 9 of whom had cervical lesions while one boy who was totally quadriplegic was taken home by his family and undoubtedly demised. The verv high mortality is surprising – there are a considerable number of quadriplegic patient around. However these patients very easily develop respiratory problmes in the early davs after their injury – the aetiology of this, whether it is spinal cord oedema, or pulmonary oedema is uncertain but it has been noted that sometimes they develop severe laryngeal spasm and stridor. It has been necesary to intubate or do a tracheotomy on these patients at times.

SUMMARY

The indications for Nerve Conduction Studies and Electromyography on 642 patients referred to the Neurology Unit over a ten year period are reviewed. Mononeuropathies, peripheral neuropathies and proximal nerve lesions were the main clinical indications for the investigations and diagnosis was substantially aided in mononeuropathies, peripheral neuropathies, traumatic nerve damage, myopathies and myasthenia gravis. Conversely, patients referred with unilateral sensory disturbances, movement disorders and burning feet syndrome did not benefit.
Skilled manpower and the high recurring cost of the tests were the most serious limiting factors threatening the sustainability of (he service. Discretionary referrals and involvement of non specialized medical officers in the performance of tests will improve service delivery, but fiscal contribution from the Health sector to complement cost recovery is vital to sustain and expand this service.

Keywords : Africa, Neuromusculal disorders, Sierra Leone, Afrique, Electrodiagnostic

RESUME

L’étude des vitesses de conductions nerveuses et d’un examen électromyographique realisés chez 642 patients dans I’Unite de Neurologie en 10 ans sont passées en revue. A I’inverse, les patients présentant des troubles unilatéraux de la sensibilité, des troubles dc la motricité ainsi que ceux qui étaient atteints d’un syndrome des « pieds brulants » n’ont retiré aucun bénéfice de ces examens. Le recrutement de personnel qualifié et le prix élevé de ces examens ont été les obstacles les plus sérieux rencontrés par le service. En faisant appel a du personnel medical non spécialisé pour effectuer ces examens, on réduirait ces contraintes, mais la contribution financiere de la santé publique est indispensable pour permettre au service de se maintenir et de se developper.

Mots cles: maladies neuromusculaires, Sierra Leone

INTRODUCTION

Since Galvani discovered electric current in a nerve/muscle preparation of a frog’s leg in the late eighteenth centry, great strides have been made in the study of the role of electricity in nerve/muscle physiology. It was not until 1948 however, that elect ical stimulation of the nerve was put into clinical use (1). Thereafter, nerve conduction studies (NCV), and electomyography (EMG) have become standard procedures in the diagnosis of neuro-muscular disorder.
In Sierra Leone, these facilities became available in 1982 with the setting up of a Neurological service at Connaught Hospital, the main referring hospital in Sierra Leone. Unlike other electrophysiological procedures such as electrocardiography (ECG) and electroencephalography (EEG) that could he competently performed and interpreted by trained technicians, or even by computer, the performance of NCV and EMG is still firmly rooted in the domain of the neurologist or neuro-physiologist. In a developing country such as Sierra Leone, manpower limitations in these specialities may seriously affect the delivery of such services to me community.
The present review often years ol neuroclectrodiagnostic procedures at Connaughl Hospital is an attempt to assess the proper utilization of these faclilities as diagnostic aids in the Sierra Leonean, the constraints encountered in their performance, and to consider ways of improving the efficiency ol the service.

METHODS

Complete records ol all cases ol NCV and EMG done from 1982 to 1992 were available for review. This was made possible by a comprehensive record system containing Oic relevcant clinical, demographic and eleclro-physiological data ot all patients investigated during this time. Excluded were incomplete data due to various reasons, such as electrical failure during examinations, electrode malfunction and patients’ non-cooperation.
Patients ages ranged from six months to eighty five years with a mean of 41 years. The sex ratio was 1.4 females to one male. Investigations were mainly of two types; 1. nerve conduction studies which included measuring the velocities of nerve conduction (NCV) in both sensory and motor nerves, as well as latencies and amplitudes. This investigation was used in diagnosing peripheral nerve disorders like peripheral neuropathies, mononeuropathies, root and plexus abnormalities, as well when repetitive stimulation was also done. 2 electomyography which is the study of muscle fibres by needle electrodes and was the main diagnostic procedure in myopathies, muscular dystrophies and muscular atrophies. Both these procedures were done on a two channel electromyograph machine (Neuropack men – 3102, Nihon-Koden, Japan) according to standard methods (2), in an air-conditioned room at fairly constant temperature, lack of averaging equipment reduced the accuracy and sensitivity of measuring very small sensory potentials.
Cases were referred from all specialties i.e internal medicene, surgery, obstertics and gyneacology, paediatrics as well as from general practitioners. All investigations were done by a neurologist (DRL), assisted by a nurse. A cost revovery fee was levied on most patients, excepting those case where non-affordability was a consideration.

RESULTS

During the period under review, 642 new patients were investigated. Some patients had serial examination but only the initial evaluation was considered for the purpose of this study.
The commonest indication for testing was mononeuropathy accounting for 38% of all cases. Root and plexus abnormalities. and peripheral neuropathy accounted for 22% and 20% respectively. Other indications included traumatic nerve injury, myopathies, and myasthenia gravis.

Table 1 : Clinical indications for clectrodiagnosis

Three Further Categories of patients presenting with unilateral subjective sensory symptoms but no clinical signs, affecting upper and lower limbs, 2. those with burning feet. and 3. a miscellaneous group including patients with tremor, dystonia, dyskinesia, and ataxia.

Table 2 : Details of three commonest indications for NCV and EMG

(Table 2) shows details of the tree commonest categories referred for investigation. Median nerve lesions specially the carpal tunnel syndrome, were by far reprted (3,4). This \as folloed by facial and ulnar nerve lesions. Cervical root and brachial plexus lesions were slightly more prevalent than lumbar root lesions. In the peripheral neuropathies, the mixed sensori-motor type was present in 65% of cases, while pure sensory neuropathy accounted for 19% and motor neuropathy 16%. This distribution is similar to that noted in Nigeria (5). The Motor neuropathy included motor neurone disease and the Gullian-Barre syndrome, the latter accounting for the majority of cases.

In order to assess the usefulness of electrodiagnosis in confirming clinical diagnosis, or in establishing a diagnosis, an attempt has been made to calculate the percentage of cases examined in which the investigation contributed to the establishment of a diagnosis (Table 3).

Table 3: Usefulness of MCV and EMG in diagnosis of neuroniuscular disorders of neuromuscular disorders

This showed that the procedures were of significant diagnostic value in the following conditions; peripheral
neuropathy, mononeuropathies, traumatic nerve injury, myopathies, and myasthenia gravis.
They were of some value in root and plexus injuries and of virtually no use in subjective hemisensory symptoms, burning feet syndrome, and all the movement disorders. In the case of peripheral neuropathy, pure sensory neuropathies were less commonly diagnosed, but classification of neuropathies into axonal and demyelinating types by NCV contributed to the differential diagnosis of underlying causes.

Cost Considerations

Replacing electrodes particularly concentric needle electrodes were the most expensive recurring costs of operations. Other consumable items included electrode gel, ground (earthing) leads, stimulator pads and recording heat sensitive paper. With frequent voltage fluctuations, equipment failure was not uncommon. Assuming that 500 patients paid an average ofLe 3000 ($6) per examination during the study period, the total of $3000 was barely enough to cover these costs. It should also be pointed out that the equipment is now thirteen years old and virtually at the end of its lifespan and that a current replacement cost is in the region of$ 50,000.

DISCUSSION

In pursuing the objectives of the Alma-Ata declaration to provide health for all by the year 2000, great emphasis has been placed on primary health care (PHC). It is becoming clear however that tertiary care cannot be ignored and should be developed to a level capable of supporting and augmenting PHC programmes. This requires the provision of diagnostic and therapeutic facilities in various specialties. In a review of neurological problems in Sierra Leone, neuromuscular diseases accounted for about 15% of all cases seen (6), while in Nigeria over 10% of neurological cases were attributed to neuromuscular conditions (7). Moreover up to 40% of the neuropathies in Nigeria were related to nutritional abnormalities (7), an important PHC considerations. NCV and EMG are considered essential in the investigation of these cases.
The cost profile of the procedure clearly shows that affordable cost recovery is inadequate to sustain the service, hence budgetary provisions must be made to maintain and replace equipments as required. In general, these costs must be offset against large sums spent on individuals for treatment overseas for conditions for which local expertise is available but specialized units poorly equipped. Some of this money should be used to equip our tertiary centres.
Perhaps the major factor limiting the use of the facility is the shortage of skilled manpower. With only one neurologist in practice, the equipment is underutilized. This is.supported by the number of new patients seen during the study period, averaging less than seventy a year. Tests are usually suspended, sometimes for long periods, during his absence. Training of non-specialized medical officers in performing some of the basic investigations is conceivable eventhough a sound knowledge of neurology is essential. This is only possible if medical officers are allowed to spend a longer time at their posts.
Under the present circumstances, efficiency can still be enhanced if optimum use is made of both time and equipment. In this regard, proper selection of cases referred is essential. Patients with movement disorders, burning feet or subjective hemisensory symptoms do not benefit from electrodiagnosis, while those with clinical evidence of peripheral neuropathy, mononeuropathy and myopathy gain the most. Referring physicians should know the indications, usefulness and limitations of NCV and EMG, or otherwise seek neurological opinion before requesting investigations.
In conclusion, electrodiagnosis is an essential aid in the investigation of neuromuscular disoeders in Sierra Leoneans. This service cannot however be sustained by affordable cost recovery and requires financial support from the public health sector for its maintenance and development. Proper selection of cases and training of non-specialized medical personnel will enhance the delivery and efficiency of the service.