ABSTRACT

We present the case of an 8 year old girl with Guillain Barre Syndrome, managed by our team. She presented with the rapidly progressive form of the disease, with evidence of bulbar involvement and incipient respiratory arrest. She was managed with mechanical ventilation and partial exchange blood transfusion as intravenous immunoglobulin (IVIG) is not available in our centre and there are no facilities for plasmapharesis. She made remarkable clinical improvement following two sessions of partial exchange blood transfusion. She could stand without support by the time of discharge and the total duration of her hospital stay was 21 days. She became ambulant 4 days after discharge and has remained well. We proffer partial exchange blood transfusion as a treatment option for severe Guillain Barre syndrome in areas where IVIG or facilities for plasmapharesis are not available.

Keywords : Blood-exchange, Guillain Barre syndrom, Treatment

RESUME

Nous présentons le cas d’une fille de 8 ans qui a présenté un syndrome de Guillain Barré s’étant manifesté de manière rapidement progressive par une participation bulbaire et un début de détresse respiratoire. Le traitement a consisté à une ventilation sous respirateur associé à un exsanguino – transfusion partielle car les immunoglobulines étaient indisponibles et il n’y avait aucune possibilité de plasmaphérèses. L’état clinique s’est amélioré de manière spectaculaire après deux cures de ce type. Elle a récupéré totalement et la durée d’hospitalisation a été de 21 jours. Elle était autonome 4 jours après sa sortie et cet état perdure.

Nous proposons ce type d’attitude thérapeutique comme option lorsque les immunoglobulines ne peuvent être prescrites et lorsque les plamaphérèses sont impossibles.

Mots cles : Exsanguino-transfusion, Syndrome de Guillain Barré, Traitement

INTRODUCTION

Guillain Barre syndrome (GBS) is an acute demyelinating polyneuropathy, characterised by progressive muscle weakness and areflexia with or without abnormal sensory functions.(9) The disease has been linked to bacterial or viral infections, systemic diseases, neoplasias, pregnancy, traumatic injuries and organ transplant. Serologic studies suggest that 20-45% of patients with GBS have evidence of recent Campylobacter jejuni infection.(5) GBS has also been associated with Human Immunodeficiency virus (HIV) infection and it has been suggested that in the tropics, a diagnosis of GBS should warrant HIV testing.(7)

GBS can be a devastating disease because of its usual, sudden and unexpected onset. With poliomyelitis under control in the developed world, GBS has emerged as the most important cause of acute flaccid paralysis (AFP).(10) In the developing countries of the world, where health facilities are limited, GBS has also been shown to be an important cause of acute flaccid paralysis.(4) There is no specific therapy for GBS but plasma exchange and administration of intravenous immunoglobulin (IVIG) have been shown to temper disease progression, lessen severity of the illness, as well as, accelerate recovery in cases of GBS.(3) About one-third of patients with GBS will require mechanical ventilation and most GBS-related deaths occur as a result of respiratory failure and autonomic dysfunction. In Africa and other developing countries of the world, facilities for paediatric intensive care are greatly limited and IVIG and facilities for plasma exchange are non-existent in most centres.(4,8) These are important, life saving measures which when not available adversely impact the prognosis of GBS in these areas.

We present the case of an eight year old Nigerian girl, who presented with severe GBS, bulbar paralysis and incipient respiratory failure, who was successfully managed with partial exchange blood transfusion and ventilatory support. We suggest that partial exchange blood transfusion can be a life-saving treatment option for GBS in centres where the recognised standard treatment for the condition, namely IVIG and plasma exchange are not available.

CASE REPORT

AO, an eight year old Nigerian girl presented at the Children’s Emergency ward of the University College Hospital, Ibadan, Nigeria with 4 days history of progressive weakness of the limbs and increasing difficulty with walking which culminated in loss of the ability to walk and 2 days history of difficulty with swallowing and breathing. AO was apparently well until about 12 days before presentation when she developed abdominal pain associated with vomiting. The pain was mainly in the peri-umbilical region and there was no associated fever, diarrhoea or passage of bloody stools. She was managed at home with antacids and the pain resolved.

One week later, she noticed sudden weakness of both lower limbs on waking up in the morning. The weakness got progressively worse and by the following day, she became unable to walk. By the end of the second day of the illness, the upper limbs were also noticed to be weak and she became unable to lift her arms. There was associated pain in the lower limbs, urinary and faecal incontinence. By the third day of the illness, AO developed difficulty with breathing and swallowing. She started drooling saliva and was unable to swallow her feeds. Her voice also became weak and was barely audible. She was taken to a general hospital (secondary level of care) but had to be referred to our centre on the fourth day of the illness on account of worsening respiratory distress.

On examination, she was found to be acutely ill, afebrile, acyanosed, not pale, anicteric and the hydration status was satisfactory. She was dyspnoeic and her breathing was shallow with a respiratory rate of 28/min. Her breath sounds were vesicular and she had no added sounds. Cardiovascular and abdominal examination revealed no abnormalities. On neurological examination, she was found to be conscious but restless and apprehensive. She was aphonic and had a very weak cough. Gag reflex was very weak but the other cranial nerves were intact. She had features of flaccid quadriplegia; power was grade 0 in both lower limbs and grade 2 in both upper limbs. Muscle tone was markedly diminished and deep tendon reflexes were lost in all limbs.

Cerebrospinal fluid (CSF) examination showed the typical cyto-albumin dissociation characteristic of GBS. The CSF was clear and colourless, with a white cell count of 5/cmm, protein of 80mg/dl and normal CSF: blood sugar ratio. Stool culture for polio virus was negative. Electromyographic studies were not done as this facility was not available at the time. A diagnosis of GBS with bulbar paralysis and imminent respiratory arrest was made. She was admitted into a general intensive care unit (ICU) of the hospital, placed on a mechanical ventilator via a cuffed endotracheal tube size 6, on 8 MV mode. She was also placed on chest physiotherapy, nil per oris, intravenous fluids, prn suctioning and urethral catheterisation. In view of the unavailability of IVIG and lack of facilities for plasmapharesis, she was offered a partial exchange blood transfusion as a means of removing autoantibodies from the serum. She had two sessions of partial exchange blood transfusion, 24 hours apart. Each session involved withdrawal of 500ml of patient’s blood into a donor bag, followed by infusion of 500ml of fresh whole donor blood. In order to avoid cardiovascular collapse, an infusion of normal saline was put up at another site while the required volume of blood was being removed from the circulation. The child’s vital signs were continuously monitored throughout the duration of the procedure in order to facilitate prompt detection of any signs of cardiovascular dysfunction. (For details see Appendix 1)

AO made remarkable clinical improvement. By the 4th day of admission, she started showing signs of recovery from the illness and limb physiotherapy was commenced. Power in the limbs progressively improved in the cephalo-caudal pattern, her breathing improved and speech became more audible. She was weaned off the ventilator on the 5th day of admission. She remained in the ICU for close observation until the 9th day of admission when she was transferred to the paediatric ward. AO’s condition continued to improve and by the 15th day of admission, she was speaking fluently and audibly, eating solid foods and had power of grade 4 and 3 in the upper and lower limbs respectively. She was discharged home on the 21st day of admission. She walked into the clinic on follow up visit one week after discharge. She is being followed up.

DISCUSSION

GBS is an important cause of acute flaccid paralysis worldwide. With the HIV pandemic and the improvement in immunization practices, GBS is likely to become a more prominent cause of AFP in Africa.(6,7) Full recovery occurs about 75-90% of children with GBS, and the majority enjoy full or functional recovery following resolution of the illness, even with little or no treatment. The decision to use immunomodulatory therapy in the management of GBS is based on the severity of the disease, the rate of disease progression, as well as the length of time between the first symptom and presentation. About one-third of cases of GBS will develop the severe form of the disease with bulbar paralysis, thereby requiring life-saving interventions like mechanical ventilation, IVIG and plasma exchange. These facilities are not available in most developing countries of the world and this is the major reason for the high mortality associated with GBS in these countries. (4,6,8) While the disease is known to be associated with a mortality rate of about 3-5% in the developed world, studies from Africa have reported mortality rates ranging between 9 and 30%, with most deaths resulting from severe forms of disease which cannot be successfully managed in the absence of ventilatory support and immunomodulatory therapy.(2,4,6,8)

The case presented had classical features of severe GBS. In the absence of adequate life-saving measures required for the management of this severe disease, the child had a high risk of mortality. Survival of a case of severe GBS depends largely on the removal or neutralisation of the autoantibodies as well as adequate ventilatory support. The partial exchange blood transfusion, in the absence of the standard recognized therapy, provided a means of mechanical removal of autoantibodies from the child’s serum. The beneficial effect of the procedure is evidenced by the accelerated recovery in this case. Fatunde et al (4) reported 100% mortality in 3 cases of severe GBS who were managed at the UCH, Ibadan with ventilatory support alone but without any other form of intervention with regards to removal or neutralisation of autoantibodies. Similar results have been reported with modified plasmapharesis in centres with limited facilities.(1,11)

CONCLUSION

GBS is an important cause of AFP worldwide. Inadequate health facilities remain a major constraint to the management of severe cases in the developing countries. Although this is a single observation, which needs to be confirmed by a controlled study in future, we propose partial exchange blood transfusion as a treatment option for severe cases of GBS in resource-poor settings where facilities for IVIG and plasmapharesis are not available.

Appendix 1