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
Les auteurs rapportent 4 cas d’arachnoidocle 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 après 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.
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
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)
Typicaiiy the clinical syndrome includes headaches, visual disturbances, obesity with or without disfunction
Neurological signs Headache is the most frequent symptom and is found in about 50 per cent of cases. The
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.
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.
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).
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.
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.
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.
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.
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.
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.
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 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.
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
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.
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.
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.
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
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:
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.
The Congress Secretariat:
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:
Second Vice Presidents
Editor of Federation Publication
b). Committee members elected in accordance with the Constitution and Bylaws consist of the following
Constitution and Bylaws Committee:
Liaison and Public Relations Committee:
c). Leadership of the other WFNS Committees for the next four years consists of the followings:
WFNS Education Committee
WFNS Material Aid Committee
WFNS Spine Committee
Committee on Skull-Base Surgery
Committee on Awards to Young Neurosurgeons
Medals of Honour Committee
Pacdiatric Neurosurgery Committee
Stereotactic and Functional Neurosurgery Committee
Scoville Award Committee
Materials, Drugs and Devices Committee
Ethics and Medicolegal Committee
Committee on Standardization of Terminology and Surgical Results
Neurosurgeons seeking more information about the activities of any of these Committees should feel free to
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.
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
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.SPINAL COLUMN AND CORD INJURY IN HARARE CENTRAL HOSPITAL
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.
Les auteurs ont revu 73 cas de lésions traumatiques de la colonne vertebrale:
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.
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.
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.
The injuries were located as follows:
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.
The causes were very similar to those of the “no cord damage” group. However the nature is more severe. There were high cervical injuries.
The compression were more severe and the dislocations more obvious than in the former group.
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
There were none of the lifesaving C1-C2 level injuries and the dislocations and compressions were much more serious than in the previous group.
(Note: there were no C1-C2 injuries)
They were located as follows:
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.
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.
Keywords : Africa, Neuromusculal disorders, Sierra Leone, Afrique, Electrodiagnostic
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
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.
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.
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.
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
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.