Venkateswaran S*, Hatkar PA**, Prasad M***.
(* Third year Resident, ** Associate Professor, *** Assistant Professor, Department of Obstetrics and Gynecology, Seth G S Medical College and K E M Hospital, Mumbai, India.)
Vomiting in pregnancy is usually considered to be hyperemesis gravidarum. However, when persistent, it may be the initial manifestation of some sinister diagnosis. A case of Guillain Barré syndrome (GBS) which initially presented as hyperemesis gravidarum is described here. Recovery after ICU care and intravenous immunoglobulin (IvIg) is also described.
GBS is an acute polyradiculoneuropathy with variable presentation. The disease could have life-threatening complications and hence accurate criteria for diagnosis are required. GBS consists of a spectrum of neuropathic disorders that have various underlying pathogenesis and clinical presentations. We present a case of an 18 week G2A1 presenting as hypokalemic paralysis which was later diagnosed as GBS.
A 26 year old G2A1 at 18 weeks of gestation was referred to our center for persistent vomiting for 2 weeks. She also complained of weakness of both lower limbs which was progressive in nature. She was unable to sit up without support. She did not have any complains of pain in abdomen, bleeding or leaking per vaginum. There was no history of fever or loose stools. On examination, she was well oriented and co-operative but had no signs of dehydration. She found difficulty in standing up and getting on to the examination table, for which she needed help. There was no tachycardia, blood pressure was normal, cardiovascular examination and respiratory system was normal. Abdomen was soft and non-tender, uterus was 18 weeks in size and relaxed. On vaginal examination, os was closed.
Owing to late presentation of vomiting, a condition unrelated to pregnancy was considered and she was sent for physician’s evaluation. Ultrasonography showed single live intrauterine gestation, rest abdominal organs were normal. Investigations revealed normal hemogram and liver parameters. However, surprisingly her serum electrolytes revealed hypokalemia with results showing sodium level of 135mEq/L, potassium level of 2.6mEq/L and chloride level of 105 mEq/L.
Detailed neurological examination showed power of 5/5 in both upper limbs, but < 3/5 in both lower limbs. Deep tendon reflexes were blunted in both upper limbs and absent in both lower limbs. Truncal weakness was present. Initial impression by neurologist was hypokalemic paralysis and potassium correction was instituted immediately. Despite this, she complained of breathlessness at rest and arterial blood gas analysis showed carbon dioxide retention. She was transferred to intensive care unit and observed for progression of symptoms. Invasive ventilation was not required. There was a brief episode of glycosuria. Fasting and postprandial blood sugar levels were within normal limits and diabetic ketoacidosis was excluded. Symptomatology persisted despite correction of potassium levels and hence further evaluation was done. Electromyogram and nerve conduction studies were done, and results of these tests was suggestive of early Guillain-Barré syndrome. Intravenous immunoglobulin was started for the same. She reported significant improvement in respiratory symptoms but only marginal improvement in motor activities. Active physiotherapy was instituted after which truncal weakness resolved. Though she could sit up, she required help for activities of daily living, and had to be mobilized on a wheelchair. She was discharged after six days in the hospital. She was following regularly in the outpatient department with a residual amount of weakness of the lower limbs. Apart from medications for usual antenatal care, she was also on multivitamin supplementation and physiotherapy for limb exercises.
At 36 weeks of gestation, She went into labor and delivered a female child of 2.3 kg by emergency lower segment cesarean section that was done for acute fetal distress. The surgery was uneventful.
She recovered well postoperatively and was discharged with the advice to continue physiotherapy.
Guillain Barré Syndrome (GBS) is an acute polyneuropathy. It may lead to severe weakness in many cases. It is an immune mediated, post-infectious disorder. Incidence in general population is 1.2 to 2.3 per 100,000 per year.[1,2]
Despite instituting treatment (consisting of parenteral immunoglobulins and plasma exchange) at the earliest, about 20% patients have difficulty in walking/ performing activities of daily living after 6 months. Thus, GBS is a severe disease with great impact on social life of the affected patients. Such a scenario was seen in our patient. Though the initial episode occurred at around 18 weeks, even at term gestation she had some residual weakness. Hence, any delay in instituting the treatment is unacceptable.
The main clinical feature of GBS is rapidly progressive muscular weakness. Motor symptomatology is rapid, bilaterally symmetrical weakness. This weakness may variably involve cranial nerve innervated muscles, respiratory muscles and sensory disturbances. There may be pain preceding the weakness, and autonomic disturbances may cause diagnostic difficulties. Typical history was seen in our patient also.
Artificial ventilation may be required, due to respiratory muscle weakness. However, our patient did not required ventilation at any point in time.
Patients who are able to walk with support are labeled as “mild” patients, and the ones who are unable to walk are labeled as “severe” patients. Using this definition, our patient could be classified as “severe”.
In our case, the patient presented with extreme lower limb weakness and inability to walk, respiratory difficulty, truncal weakness, absent reflexes in both lower limbs. There was autonomic dysfunction in the form of vomiting which then led to hypokalemia.
Eikoundi et al have described recently a similar case with similar features, including hypokalemia and neurological signs, which was presumed to be GBS, but was eventually detected to be Gitelman syndrome. In their case, the patient presented with similar complains of weakness and vomiting. Serum electrolytes revealed hypokalemia but the diagnostic feature of Gitelman syndrome which is metabolic alkalosis, hypocalcemia, hypomagnesemia in the absence of hypertension were what confirmed their diagnosis. A case of GBS diagnosed in a preexisting case of diabetic ketoacidosis has been described by Affes et al. However, our patient did not have any other preexisting medical conditions.
Our patient however was being treated for hypokalemia with potassium supplementation and supportive care. Ongoing respiratory distress and persistence of autonomic symptoms even after administering potassium supplementation prompted us to ask for an electromyogram (EMG). Absent ‘h’ reflexes bilaterally on EMG clinched the diagnosis of GBS. While a variety of criteria for diagnosis of GBS are available, many include examination of cerebrospinal fluid by a lumbar puncture. However, our patient did not undergo the same. No specific validated criteria in pregnancy appear to exist.
In our patient, intravenous immunoglobulin did not cause any maternal or fetal side effect. The European League Against Rheumatism has summarized the safety and side-effect profile of intravenous immunoglobulins in pregnancy.
The key to management was prompt institution of intensive care which prevented potential mortality. There is a reported maternal mortality of 7 %, which is slightly higher than that of non-pregnant women..
It was possible to institute prompt management due to the availability of specialist neurologist in our tertiary care hospital. Hence, it is advisable to manage such patients with lower limb weakness at a higher centre for adequate deliberation on the differential diagnosis and correct management of the condition. Detailed outcomes regarding obstetric outcomes in GBS is not well known. This case being reported to add to the available literature on GBS in pregnancy.
Successful maternal and fetal outcome can be achieved in GBS with a multidisciplinary approach involving the obstetrician, intensivist, neurologist and physiotherapist.
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Venkateswaran S, Hatkar PA, Prasad M. Successful Management Of Pregnancy Complicated With Guillain Barre Syndrome. JPGO 2018. Volume 5 No.8. Available from: http://www.jpgo.org/2018/08/successful-management-of-pregnancy.html