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ABSTRACT Background and purpose Methods Results Conclusion Key words: Stroke, Clinical profile, computerized tomography scan, Nigerians. RESUME Introduction Objectifs Méthodes Résultats Conclusions Mots Cles : Accident vasculaire cérébral, Afrique, Epidémiologie, Hypertension artérielle, Nigéria, Scanner INTRODUCTION Stroke, defined by the World Health Organization as “rapidly developing signs of focal or global disturbance of cerebral or intracranial neuronal function with symptoms lasting for more than 24 hours or leading to the death of the patient with no apparent cause other than that of vascular origin”17 is a major cause of death worldwide, second only to ischaemic heart disease8. Stroke is the leading cause of neurological disability in adults5, 18. Stroke is also a leading cause of morbidity and mortality in adults in the productive ages that contribute the work force of the society6. The hospital frequency of stroke at the University College Hospital Ibadan in 1969 was between 0.9 and 46% of all hospital admissions and 41% of all neurological admissions10. There appears to be an increase in the hospital frequency of stroke as a recent review at the University College Hospital Ibadan, 30 years later showed that stroke is now the leading cause of adult neurological admissions16. Stroke is increasingly becoming a major cause of morbidity and mortality in Nigeria and other developing countries19. Stroke has thus become a major public health problem in Nigeria. Stroke patients suffer from post stroke depression, a complication which occurs in 30% of cases19 and is associated with increase in morbidity and reduced survival14, 3. Apart from the burden of stroke on the survivors, those who care for them are also prone to emotional distress and a reduced quality of life. In a recent study at out center, the primary care givers of stroke patients were observed with higher rates of anxiety and depression and a lower quality of life when compared with matched controls2. With the increasing availability of computerized tomography scanners (CT scan) in most facilities including ours, the stroke sub-type can now be more accurately determined. The need to do CT scan was further highlighted in a study by Ogun et al7 in which the accuracy of clinical assessment was about 70% even in tertiary centers with many neurologists. The major risk factors for stroke in Nigerians are hypertension, diabetes, and hyperlipidaemia10. Cerebral infarction is the most common type of stroke seen occurring in 48% of patients while intracerebral haemorrhage occurs in 15.7% of patients and subarachnoid haemorrhage in 11.3% of patients11, 1. The mortality and severity of stroke is on the decline in developed countries because of life style modification, increased use of preventive measures such as adequate blood pressure control, increased use of anti- lipid drugs and aspirin15. This is in contrast to the increasing hospital frequency noted in our environment thus having a great burden on the health facilities in Nigerian hospitals. PATIENTS AND METHODS The study was a prospective hospital based study conducted at our centre which is a referral centre for about six out of the thirty-six states in the country. All the patients with diagnosis of stroke presenting to the Neurology unit of the Obafemi Awolowo University Teaching Hospitals Complex, Ile- Ife over a six year period (2000 to 2005) were consecutively recruited. Stroke was diagnosed using both clinical criteria and brain CT scan findings. RESULTS One hundred and thirty five stroke patients were managed during the 6-year study period. Stroke was responsible for 2.9% (135/4583) of all medical admissions and 22% (135/615) of all neurological admissions in our centre. The major predisposing factors noted were hypertension, diabetes mellitus and heart disease (atrial fibrillation) accounting for 73%, 16% and 2% respectively. Over half of the hypertensive patients (53.6%) were non-compliant with their drugs prior to the episode while 7% were previously unaware of their hypertensive status. The duration of symptoms before presentation ranged between 1 and 336 hours with a mean duration of 23 hours (SD 39). The duration of hospital admission ranged between one and 63 days with a mean of 10 days (SD 13). Stroke is undoubtedly an important disease worldwide and emerging as a public health problem in many nations. In our environment, the incidence of stroke in the community and the country is largely unknown since most studies in the country are hospital based. Even then a trend is seen as the hospital data shows progressive rise in the cases of stroke. Most authors believed that the hospital data is just a tip of the iceberg when compared to what may be seen in the community. Studies to determine the epidemiology of stroke in the community including the rural areas are therefore necessary to plan the prevention of stroke as well as manage the scarce resources in the treatment of the disease8. In this study, stroke accounted for about a quarter of the patients managed in the neurology unit of our centre during the six year period. In total, 135 patients that fulfilled the criteria were managed. The mean age was 62years (SD12) with a peak in the 5th and 6th decades of life (Table 1). This is in keeping with the findings of previous workers6, 11 where majority of the patients with stroke are in their fifth and sixth decades of life. We also noted a slight male preponderance (M:F = 1.3:1), also supporting findings in other studies and from community based studies16. Many studies showed that the male patient appear to have enhanced risk, especially for thrombotic strokes4. The higher prevalence of stroke in males might be due to the higher presence of cardiovascular risk factors in them. Also many of the males are in the upper social class and business men constantly exposed to stress and more likely to adopt a western type diet with consumption of refined, high cholesterol foods in combination with a sedentary lifestyle resulting in increased prevalence of cardiovascular risk factors such as hypertension, diabetes and hyperlipidaemia19. Our study like others in the country shows that thrombotic strokes were the most common11, 1. It is important to note that despite the availability of CT scan in our centre; only a third of the patients could afford the test. This clearly shows that cost is a major hindrance to the utilization of CT scan among stroke patients in Nigeria. This is a major problem that needs to be addressed as surgically treatable lesions such as glioma, meningioma, brain abscess and sub-dural haematoma could be mis-diagnosed as stroke in 8.6 to 13.5% of the cases3. At our centre, about twenty patients were excluded from the study as the CT scan showed surgically treatable lesions. This underscores the need for the attending physician to take a very good history of the events at onset of the stroke; however this may not be enough as it is sometimes difficult to differentiate medical from surgical conditions based on the clinical features alone without the aid of imaging modalities. The case of a 46year old long distance driver who slumped while taking his bath and hit his head aptly illustrates this point. He was being managed by the neurosurgical unit for head trauma and was to be taken for exploratory burr hole, when the relations were able to do a CT brain scan which showed massive intracerebral haemorrhage with intraventricular involvement Figure 2. All our patients save one were right handed, two-thirds of them had dominant hemispheric lesion with right hemiparesis. In a recent review of 450 cases in 20years by Onwuekwe et al9, their findings were similar to ours. The male to female ratio was 1.27:1 ant the mean age of their patients was 53.5yrs. They however found that their patients had almost equal distribution of right and left hemiparesis. They also found no significant difference between handedness and the sex of their patients. There were two cases of persistent vegetable state following cerebral infarction. One, a 48year-old woman (Figure 3a & 3b) with multiple infarcts had rapid reduction of her blood pressure at the secondary care center where she subsequently lapsed into coma prior to referral to our centre. This is a common practice by general practitioners with limited knowledge of the pathophysiology and treatment of stroke. Since majority of the patients will be attended to initially by this group of doctors, they need regular updates on the emergency care of stroke so as to improve the outcome of patients. Better still, they should be more actively involved in preventive programmes to detect risk factors and increase awareness generally. In conclusion, stroke is still a major problem in our environment and the major risk factor remains uncontrolled hypertension. The case fatality remains very high with a risk of moderate to severe neurological disability among the survivors. There is sub-optimal utilization of CT scan even when it is available because of financial constraints. We recommend that community based intervention measures such as health education of the community with emphasis on control of the predisposing factors, and education of general practitioners who are usually the first to handle stroke patients be put in place to address the burden of the problem and the inadequate control of blood pressure among hypertensive patients. The primary health workers should also be educated and trained to pass across information to the populace at every available opportunity the advantages of exercise, maintaining a healthy weight, avoid or quit smoking and monitoring of blood pressure and glucose levels regularly. There is also the need for pubic enlightenment campaigns to educate the populace about the need to check their blood pressures regularly and if hypertensive to comply with their medications. Efforts to make radiological imaging test available and affordable should be pursued both by the government and public institutions as well as by non governmental organisations. Table 1: Socio-demographic profile of stroke patients in Ile-Ife (N=135).
Table 2: Activity at onset of stroke patients in Ile-Ife (N= 135)
Others: During an argument (1), coming from night vigil (1), during defecation (1), during sexual intercourse (1), while preaching (2), while fetching water (1), while driving (2), and unknown (6). Table 3: Stroke outcome in Ile-Ife.
* 3 cases discharged against medical advice  Figure 1
Figure 3a
Neurological Development in french speaking African Countries LES HEMATOMES INTRA-CEREBRAUX POST-TRAUMATIQUESConnus des neurochirurgiens depuis très longtemps comme une complication du traumatisme crânien, les hématomes intra-cérébraux post-traumatiques (HICT) sont rares. Facilement diagnostiqués au scanner ou à l’IRM, ils sont trop souvent considérés comme banaux, alors que la littérature fait encore état de nombreux points flous tant sur le plan diagnostique, que sur le plan physio-pathologique ou encore sur celui des traitements à adopter (traitement médical, chirurgical ou mixte). I – DEFINITION DES HEMATOMES INTRA-CEREBRAUX 1 – C’est une définition anatomo-pathologique : Un hématome intra-cérébral post traumatique (HICT) est une collection de sang à l’intérieur d’un foyer lésionnel remplissant plus des 2/3 de ce foyer, le reste étant constitué de tissu cérébral lésé et de zones d’hémorragies microscopiques [3, 4, 13]. C’est ce qui le distingue des contusions parenchymateuses dans lesquelles la quantité de sang reste bien moindre par rapport à la fraction tissulaire et où du tissu nécrotique est centré sur une hémorragie péri-vasculaire [3]. Le scanner a modifié les idées sur l’hématome intra-cérébral : longtemps considéré comme une lésion tardive (48 heures), on sait maintenant que l’HICT se constitue en réalité de façon précoce : dès la 6eme heure pour Ruiz-Sandovall, le plus souvent dans un délai compris entre 8 heures à treize jours voire plus avec une moyenne de deux jours [13, 23]. II – DONNEES GENERALES 1 – Physiopathologie des hématomes intra-cérébraux post-traumatiques Deux mécanismes traumatiques peuvent être à l’origine d’un hématome intra-cérébral : – un choc très violent avec rupture de vaisseaux profonds en regard d’une contusion corticale extensive quelle que soit la région cérébrale touchée . Cela d’autant que l’impact s’est produit sur une petite surface crânienne, dans le cas entre autres des plaies tangentielles par arme à feu ou par arme blanche et des fractures de la voûte crânienne avec enfoncement dans le parenchyme (les plaies crânio-cérébrales sont exclues de cette étude). Si ces mécanismes expliquent la présence des hématomes intra-cérébraux post-traumatiques sur le scanner effectué immédiatement après le traumatisme, il ne peuvent par contre pas rendre compte des hématomes d’apparition secondaire (24 à 72 h) qui pour Ninchoji et col. [20] présentent des aspects particuliers : – les mécanismes sont souvent dynamiques (tête en mouvement), Différents facteurs étiologiques ont donc été évoqués, la plupart expliquant la constitution secondaire d’une plaie vasculaire : – l’hypoxie, souvent présente dans le traumatisme crânien, peut provoquer une accumulation locale CO2 dont le pH acide engendre une hémorragie péri-vasculaire par vasoplégie suivie d’une vasodilatation secondaire avec diapédèse. – la stase veineuse avec dème vasogénique secondaire (Czernickiz), comme dans le cas des hématomes survenant après chirurgie décompressive (évacuation d’un hématome extra-dural, hématome sous-dural ou suite à un volet décompressif). Par contre, l’hypothermie longtemps discutée comme facteur favorisant des HICT , n’est en rien responsable, comme l’a démontré Resnick [21]. Au plan évolutif l’hématome intra-cérébral se développe souvent en deux phases : Une phase initiale brève d’hypertension intra-crânienne proportionnelle au volume et à la rapidité de constitution de l’hématome, suivie au bout d’une semaine environ d’une deuxième phase dite «impansive», correspondant à la mise en jeu des mécanismes tampon de la pression intra-crânienne (compression des structures veineuses et augmentation du débit veineux jugulaire, mise en jeu des villosités arachnoïdiennes de la queue de cheval) au cours de laquelle la pression baisse rapidement. Si donc l’hématome n’est pas massif et n’implique pas un risque vital ou si un traitement palliatif (drainage ventriculaire externe ou traitement médical) a été rapidement mis en route et se révèle efficace sur les chiffres de pression intra-crânienne, le patient franchira la période aiguë et pourra donc éviter une intervention. C’est sans doute ce mécanisme qui rend compte du petit nombre d’opérations nécessaires. Le tableau 1 résume les différents mécanismes physiopathologiques (isolés ou associés) décrits dans la genèse des HICT [14]. Tableau 1 – Mécanismes physiopathologiques
2 – Prévalence des hématomes intra-cérébraux post-traumatiques La survenue d’un hématome intra-cérébral dans les suites d’un traumatisme crânien est relativement rare. Sa manifestation peut être aiguë mais le plus souvent c’est dans les jours qui suivent l’accident que l’on constate l’apparition de l’hématome. La répétition des examens tomodensitométriques est donc capitale. III – FORMES CLINIQUES DES HEMATOMES INTRA-CEREBRAUX POST-TRAUMATIQUES 1 – les aspects évolutifs habituels des HICT Les hématomes intra-cérébraux se distinguent par la diversité des symptômes observés dépendant à la fois de la taille de l’hématome, de sa localisation et de la rapidité de sa formation. Parmi les manifestations cliniques fréquentes on retrouve notamment : – la dégradation progressive de l’état de conscience souvent au dessus de 8 dans la phase initiale exprimée selon le score de Glasgow. Même s’il est classique d’affirmer qu’il peut s’aggraver brutalement par engagement, cette évolution est une éventualité plus rare (3%) que dans les suites d’hématomes extra-duraux ou sous-duraux aigus (82%) selon Andrews et col. [1] et survient surtout dans les localisations temporo-pariétales qui peuvent se compliquer de compression du tronc cérébral. Dans certains cas, la constitution de l’hématome est tardive, au bout de plusieurs semaines et se caractérise alors par l’apparition brutale d’un déficit neurologique, selon le tableau de « Splät apoplexy » décrit par Bollinger [4] en 1891. Quoiqu’il en soit, précoce ou tardif, le diagnostic d’HICT repose sur le scanner et sa répétition selon le protocole actuellement mis en place dans les services de neurochirurgie. 2 – Les aspects particuliers a – étiologiques Toutes les variétés de traumatisme peuvent être à l’origine d’hématomes intra-cérébraux. Les accidents de la circulation avec impact crânien direct prédominent chez les sujets jeunes, alors que les chutes ou les accidents domestiques atteignent plutôt L’âge est un facteur classique, l’hématome intra-cérébral étant une conséquence des traumatismes crâniens survenant après 50 ans. Il existe d’ailleurs à ce propos une relation inversement proportionnelle entre l’importance de l’impact crânien et l’âge du patient. Certaines pathologies peuvent modifier l’aspect clinique et surtout l’évolution spontanée Enfin, le sida peut par son retentissement sur la coagulation avoir une incidence aggravante dans les cas d’HICT. b – topographiques Comme nous l’avons déjà signalé, l’hématome intra-cérébral est généralement situé dan On peut cependant l’observer également dans les régions profondes de la substance blanche de l’encéphale au contact des noyaux gris centraux et dans cette localisation il se rapproche des hémorragies spontanées de l’adulte. Les hémorragies intraventriculaires que l’on pensait rares et systématiquement mortelles avant le scanner sont en réalité présentes dans une proportion de 3 à 10% des traumatismes crâniens mais gardent souvent le pronostic péjoratif qu’on leur prêtait auparavant. Elles surviennent dans les cas de traumatismes crâniens très violents (accidents de moto) et sont souvent associées à d’autres lésions corporelles. Elles sont en général responsables de troubles d’emblée graves de la conscience (score de Glasgow < 8), en rapport avec une hypertension intra-crânienne aiguë par gêne à l'écoulement du liquide céphalorachidien imposant en urgence un monitoring de la PIC et un drainage ventriculaire externe du LCR réalisés en un seul temps opératoire. Si l'HIC est la règle dans 50% des cas, l'hydrocéphalie est par contre peu fréquente, l'HIC étant de trop brève durée pour dilater les cavités ventriculaires. La fosse postérieure est une localisation rare qui se caractérise par une somnolence, des troubles d'idéation, suivis rapidement par des troubles de la conscience qui peuvent s'aggraver brutalement par engagement des amygdales cérébelleuses au niveau du trou occipital. Cette dernière complication impose plus que dans toute autre localisation un monitorage combiné de la PIC et de la PIV.avec drainage ventriculaire externe. c – évolutifs La répétition des examens TDM dans les suites des traumatismes crâniens a permis de vérifier que les H.I.C.T. peuvent exister aux différents moments de l’évolution. Ils peuvent s’observer sur le scanner initial ou au contraire apparaître de façon plus ou moins tardive alors que le premier examen TDM était normal ou ne montrait que des images de contusion. Dans cette deuxième hypothèse le scanner a pu permettre de vérifier que l’H.I.C.T. pouvait se former secondairement par confluence de plusieurs pétéchies, puis évoluer progressivement soit vers la stabilisation, soit vers l’augmentation de volume, généralement sans dème associé. Il semble donc illusoire sur un plan théorique de classer les H.I.C.T. en hématomes immédiats et hématomes retardés selon la classification anglo-saxonne, puisque en réalité, ils peuvent survenir à n’importe quel moment de l’évolution d’un traumatisme crânien (dans les 10 à 12 premiers jours). Fukamachi [14] a cependant proposé une classification qui tient compte de ces problèmes et a séparé les HICT en 4 types basés sur son expérience tomodensitométrique dans 84 cas : – Type I : hématome toujours présent sur le scanner initial (moins de 6 heures) : 39%, Seuls les types III et IV, représentant 50% des cas peuvent être qualifiés d’hématomes tardifs.
Figure 1 – contusion hémorragique temporale gauche au J1 posttraumatique III – TOMODENSITOMETRIE ET EXAMENS COMPLEMENTAIRES Au scanner, les hématomes intra-cérébraux se distinguent de l’attrition par leur topographie en général plus profonde, leur hyperdensité plus nette et mieux limitée, l’absence ou la discrétion initiale de l’dème périphérique. Ils prennent la forme de flaques plus ou moins arrondies, aux bords quelquefois « en carte de géographie » quand l’hématome a pris naissance au sein d’une contusion cérébrale. Dans leur localisation profonde, au contact des noyaux gris, l’aspect est celui de taches hyperdenses à bords nets et précis sans dème périphérique traduisant le « cisaillement » des artères perforantes qui en est la cause. Les hémorragies intraventriculaires ont une densité variable selon la quantité de sang présente dans le LCR et donnent donc des aspects variant de l’hyperdensité modérée à des formations très hyperdenses traduisant la présence de caillots qui déforment la structure ventriculaire tendant à prendre un aspect « soufflé » et arrondi. Au plan évolutif, du fait de la lyse progressive des caillots, ces images varient autant L’IRM n’est pas couramment pratiquée à la période aiguë des traumatismes crâniens. Les autres examens n’apportent aucun élément intéressant dans le diagnostic d’HICT, hormis l’EEG qui doit toujours être pratiqué pour rechercher des crises focales ou un état de mal épileptique, difficilement décelables chez les patients hospitalisés en réanimation. IV – PRISE EN CHARGE THERAPEUTIQUE DES HICT 1 – Les éléments à prendre en compte Il n’existe toujours pas de consensus sur les critères conduisant au traitement chirurgical, en effet de nombreux éléments doivent être pris en compte dans cette décision tels que la profondeur et la localisation de la lésion, l’état clinique initial, l’évolution clinique et radio-scannographique, l’âge du patient, ses pathologies pré-existantes, etc… Il faut cependant souligner que les recommandations chirurgicales fixent une indication opératoire formelle aux hématomes responsables d’un déplacement de la ligne médiane ≥ à 5mm ainsi qu’à ceux entraînant une HIC menaçante pour la vie du patient. 2 – Les méthodes médicales Compte tenu des critères que nous venons de décrire, Il est évident que tous les HICT ne méritent pas une évacuation. Il faut d’ailleurs remarquer à ce propos que l’intervention s’impose beaucoup plus rarement en cas d’hématome intra-cérébral (4 à 5% environ selon les séries) que dans les autres variétés d’hématome intra-crânien traumatiques. Il doit être réalisé sous monitoring de la PIC, des pressions artérielle et veineuse centrale et de la pression de perfusion cérébrale (PPC). Il associe généralement : – Le drainage ventriculaire externe de liquide céphalo-rachidien . 3 – Les méthodes chirurgicales La mesure de la PIC avec drainage externe réalisée au bloc opératoire et par opérateur entraîné à cette pratique constitue un moyen de surveillance inégalable des traumatismes crâniens graves. La ponction stéréotaxique ou par voie endoscopique a été décrite par certains pour évacuer ces lésions mais la craniotomie ouverte reste actuellement la meilleure technique en usage [2, 19]. Un petit volet de craniotomie ou à défaut un trou de tréphine réalisés généralement sous magnification optique et avec l’aide d’écarteurs malléables, en prenant soin de traverser le minimum de substance cérébrale, seront le plus souvent suffisants pour diminuer le volume de l’hématome et son effet de masse. Signalons, à ce propos, la découverte exceptionnelle de malformations artério-veineuses responsables du saignement dans les parois de la cavité opératoire. La décompression sous temporale pour traiter l’HIC, après avoir été abandonnée V – LES INDICATIONS CHIRURGICALES Chaque patient victime d’un traumatisme crânien grave avec présence d’un hématome intra-cérébral post-traumatique sur le scanner, doit être hospitalisé dans une unité de réanimation. Le monitoring de la pression intra-crânien doit être systématique dès que le score de Glasgow < ou = 8.
Lindication d'évacuation d'un hématome intra-cérébral post-traumatique dépend des critères suivants :
Par contre doivent bénéficier d’un traitement médical sous monitoring de la PIC et 1 – les patients présentant des hématomes intra-cérébraux post-traumatiques envahissant les noyaux gris centraux et le thalamus, La lobectomie peut être préférable dans certains cas à l’évacuation d’un hématome ou d’une contusion (à la condition qu’elle intéresse la région traumatisée, siége de l’hémorragie). La crâniectomie décompressive semble être utile à la phase aiguë du traumatisé crânien grave dans des situations extrêmes d’hypertension intracrânienne non contrôlée « refractaire ». VI – PRONOSTIC DES H.C.T. ( RESULTATS) L’évolution et le pronostic des patients victimes d’un hématome intra-cérébral post-traumatique dépendent de plusieurs facteurs et en particulier de : – la taille, la localisation de l’hématome et sa date d’apparition La morbidité est importante de l’ordre de 33 à 35% des cas. Le taux de mortalité moyen est de 25 et 30 % des cas, mais peut atteindre 50 % chez les patients victimes d’un traumatisme crânien sévère et qui développent un hématome intra-cérébral secondaire (Crooks, Hirsch) [11, 17]. Compte tenu de l’absence d’études réellement validées, l’avis des différents auteurs diverge à propos des résultats de la chirurgie comparés à ceux du traitement médical, puisque certains estiment que le recours à une intervention améliore la morbi-mortalité alors que d’autres pensent que les résultats sont identiques [6, 7]. Le tableau 2 résume les différentes études réalisées et leurs principaux résultats.
VII – CONCLUSIONS Au total l’hématome intra-cérébral post-traumatique est une lésion qui demeure rare dans les suites de traumatisme crânien, dont le pronostic est toujours sévère, avec une morbidité de l’ordre de 33 à 35% des cas, une mortalité moyenne de 25 et 30 %, pouvant cependant atteindre les 50% et dont les critères de surveillance sont la réalisation répétée de l’examen clinique, des examens TDM (volume de l’hématome, état des citernes de la base) et le monitoring de la PIC et de la PPC. Une minorité (# 5%) de ces hématomes nécessite une intervention chirurgicale, mais le consensus n’existe pas sur les critères opératoires, d’autant que certains auteurs estiment qu’elle n’améliore pas le pronostic global. SUMMARY A 45 year-old man with periodic muscular weakness is presented. He had 4 similar transient generalized weakness epizodes since the age of 38. His initial evaluation showed mild proximal weakness with no clinical or electrical myotonia. The serum potassium level was 2.7 mEq/lt. He showed dysmorphic features with short stature, a hypoplastic mandible, hypertelorism, low set ears, micrognathia, bilaterally short index fingers, broad forehead. His three children had almost the same dysmorphic features Key words : Potassium, dysmorphic features, weakness, Andersen syndrome INTRODUCTION In 1971, Andersen et al [1] described an 8-year-old boy with a distinct syndrome marked by clinical triad of PP, cardiac dysrhythmia and developmental anomalies named as Andersen syndrome (AS). The attacks are often superimposed on a background of generalized weakness. The inheritance pattern appears to be autosomal dominant with significant phenotypic heterogeneity. The mutations affect a potassium channel, called Kir 2.1-a member of a large family of potassium channels that help regulate the flow of potassium out of muscle cells [5]. REPORT OF CASE A 45-year-old man with periodic, sudden onset, generalized weakness is presented. From his detailed history, it was learned that he had 4 similar transient generalized weakness epizodes since the age of 38. The epizodes were of variable duration from 1 to 3 days and were either spontaneous or induced by prolonged inactivity or following meals and rest after exercise. There was no family history of systemic or neurological illness except his fathers’ death of hearth failure at age 38. Physical examination of the patient revealed prominent dysmorphic features such as hypoplastic mandible, hypertelorism, low-set ears, micrognathia, broad forehead [figure1], bilateral short index fingers [figure 2] and short stature. Also similar dysmorphic features were recognized from his father’s photograph [figure 3]. He had 3 children and their physical examination also revealed almost the same but milder dysmorphic changes [figure 4,5,6]. The patient’s blood pressure and pulse rate were normal. On neurological examination cranial nerves were intact. Generalized proximal muscle weakness with more prominent in lower extremities was found. Tendon reflexes were decreased, sensation was normal. There was no clinical myotonia. Full blood counts and biochemical examinations were performed and no abnormality was seen except the potassium level. His initial potassium level was 2.7 mEq/lt. After infusion of 1000 cc Isolyte M solution (includes 0.15 g potassium) to the patient in 24 hours’ time, potassium level increased to 4.5 mEq/lt. And then his neurological examinations became to normal state. Cardiac examinations including electrocardiography (ECG), echocardiography and Holter monitoring were unremarkable. Brain and spinal cord magnetic resonance imaging were normal. Electromyography (EMG) of limb muscles showed no evidence of myotonia. Muscle biopsy from deltoid muscle, showed mild myopathic findings with chronic denervation. Hypokalemic challenge with intravenous glucose (2 gm/kg) followed 30 minutes later by insulin (0.1 unit/kg intravenously) resulted in no change in muscle strength despite a decrease in serum potassium from 4.3 to 3.7 mEq/lt. Potassium challenge was not performed, because of danger of fatal cardiac complications. DISCUSSION Andersen’s syndrome is characterized by periodic muscle paralysis, cardiac arrhythmia and abnormal growth that includes short stature, and deformations of the spine, fingers, toes and face [6]. Weakness occurs episodic, lasting one hour to days, attacks may be rare though some patients may have persistant generalized background weakness, onset from early childhood to anytime in adulthood. The beginning of the attacks in our patient was at the age of 38. It was slightly a long time for the occurence of the symptoms. Hypokalemia, potassium administration, exercise are the precipitants for attacks or it may be idiopathic. In AS, potassium shifts during attacks of weakness are inconsistent. Recognition of the characteristic face in AS permits an early diagnosis and the detection of the severe systemic manifestations associated with the syndrome. Severity of the facial involvement does not correlate with the the severity of heart or muscle involvement [2]. The patient’s electrophysiological examination included nerve conduction study and electromyography (EMG) exclude the presence of myotonia and muscle biopsy showed mild myopathic findings with chronic denervation. In periodic paralysis muscle biopsy may show a wide range of abnormalities, vacuoles being more specifically linked to the disease. Frequency or severity of attacks did not correlate with the presence of vacuoles but those were more easily found in patients with long term disease [10]. Vacuoles and tubular aggregates are frequent changes in PP and therefore helpful for the diagnosis. Important myopathic findings in the muscle biopsy suggest a permanent myopathy which probably developes after severe crises or long term disease [7]. CONCLUSION The clinical diagnosis of AS is not always apparent and physicians should be aware of the possible physical, clinical manifestations and laboratory findings; because early diagnosis and treatment may reduce the risk of potentially lethal cardiac dysrrhytmias.  Figure 1  Figure 2  Figure 3  Figure 4
ABSTRACT Objects Methods Conclusion Keywords: Ventriculoperitoneal shunt, Hydrocephalus, Endoscopic third ventriculostomy, Shunt catheter. Mots cles: Complication, Hydrocéphalie, Intraventiculaire, Shunt ventriculo-péritonéal INTRODUCTION Cerebrospinal fluid (CSF) shunt operation was first realized in 1908 by Kausch [7] and since then ventriculoperitoneal (VP) shunts have remained the main method of treating hydrocephalus even with the recent increased interest in endoscopic third ventriculostomy. Although VP shunt procedures are easy to perform they are not without complications, adequate management of which may require further surgical procedures. These complications are many ranging from the very minor ones to major complications. Most often, despite appropriate surgical techniques, careful attention to operative details, strict compliance with asepsis and the use of prophylactic antibiotics to prevent or reduce the common complications that may follow VP shunt insertion, some complications still occur, some very unusual and rare [6]. One of these rare complications is complete proximal migration of the entire VP shunt system. Migration of the shunt or its parts have been reported but usually it is the distal or the peritoneal catheter that migrates after breakage or disconnection into many locations such as the scalp, heart, anus/rectum, urethral, knee, umbilicus, chest, pleural cavity, inguinal canal and scrotum [3]. Complete or total migration of the shunt as a whole is very rare and few cases are described in the literature. Distal migration rather than proximal shunt migration is more common and presumed to be due to intestinal peristalsis which may pull down the shunt. We aimed to present in this brief communication an unusual and a rare complication of Ventriculoperitoneal shunt placement which is total upward migration of the entire VP shunt system into the ventricular system of a child. The mechanism, management, and possible preventive methods are discussed. CASE REPORT AO is a 4year-old boy who first presented to our neurosurgical service at the age of five months with progressive hydrocephalus. VP shunt was advised but the parents refused until the child was 3years old due to poor milestone development and gross head enlargement. A right occipital VP shunt was inserted with satisfactory result. A year after, he presented with recurrent symptoms of active hydrocephalus and raised intracranial pressure associated with visual impairment. Examination revealed that the part of the VP shunt proximal to the shunt valve could not be palpated. This was confirmed by the shunt series, shuntogram, and computerized tomography (CT) brain scan which shows shunt disconnection proximal to the shunt valve with the ventricular catheter lying in the right lateral ventricle. There were no clinical features of sepsis and CSF culture did not yield any organism. A new shunt system was inserted with the aid of artery forceps leaving the detached ventricular catheter in situ. During the second shunt surgery, attention was placed on technical details so as to prevent recurrence of the complications, however the patient came back four weeks later with fever, vomiting, gross malnutrition and obtundation. This time no part of the VP shunt could be palpated along its entire length. A repeat shunt series shows the entire VP shunt system in the right lateral ventricle (Figure 1). CT scan was not done due to financial constraints. Sepsis screen did not yield any organisms but the patient was placed on, ceftriaxone. He later had limited craniectomy to retrieve the VP shunt system and the previously detached ventricular catheter. An external ventricular drainage was left in place to monitor and prevent excessive rise in the intracranial pressure until a new shunt could be placed. There was no improvement in his clinical and neurological status until three weeks later when his parent requested for discharge against medical advice. He presented once to the surgical out-patient clinic but had since been lost to follow-up. DISCUSSION Complete proximal migration of the entire VP shunt system is a well known complication though very rare in occurrence as few cases had been reported in the literature [10, 5]. This upward migration involves patient motion that creates a “windlass” effect with no resistance to the movement of the tubing and also requires a potential space such as the subgaleal or the ventricular spaces for the shunt to migrate to. Why this occurs in some patients is not known. A lot of factors had been attributed for the possible mechanism underlying this rare complication. These include the negative sucking intraventricular pressure, the positive pushing intraabdominal pressure and the tortuous subcutaneous track as well as neck movements. Other factors are related to the patient, the surgical technicalities and to the shunt itself. The patient related factors includes the age of the patient, severe and gross hydrocephalus with very thin cortical mantle, malnutrition, anaemia, sepsis, and repeated head movements and rotation (Bobble-head syndrome). Many of these predisposing factors were present in our patient who had a gross head enlargement with very thin cortical mantle. The patient presented also was malnourished and anaemic. The supine position in which the infants are nursed as well as the shorter distance between the ventricular and the peritoneal ends in these children facilitate proximal migration of the shunt. In all the cases reported in the literature all were infants and young children except Eljamel [5] that reported a case in an adult. This may not be unusual as majority of VP shunt are carried out in this age groups. In addition to the predisposing factors in this patient, we found out that the parents were constantly tampering with the shunt system at the region of the shunt valve, and thus likely to dislodge the shunt from the underlying tissues. This complication can be prevented by careful attention to surgical details particularly if performed by an experienced surgeon [1], careful patient selection, use of shunt with bulbous shunt valves and/or reservoirs, use of burr hole cover [4] to prevent the upward migration through the burr hole defect especially when large burr hole is made, and use of frontal burr hole site [3] with a small but preferably a cruciate rather than a linear dural incision to access the ventricle rather than other sites. The parents and/or the guardian of such children should also be educated on the care and handling to avoid undue tampering and dislodgement of the shunt system. The use of endoscopic third ventriculostomy (ETV) should be explored and encouraged for future treatment of hydrocephalus in selected and fit patients as an alternative to shunting procedures in selected patients [2].  Figure 1a  Figure 1b
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