Spinal anaesthesia for a caesarean section in a patient with paraneoplastic cerebellar ataxia.

Paraneoplastic cerebellar ataxia (PCA) is most frequently observed in gynaecological cancers, small cell lung cancer, breast cancer, Hodgkin's lymphoma, cancer testis or malignant thymoma. In the literature, there is no data related to the effects of PCA during pregnancy or reports on the effects of anaesthesia in patients with PCA. We present management of a pregnant woman with PCA who was suddenly unable to walk with PCA and for whom effective spinal anaesthesia was performed for an elective caesarean section with no complications.

INTRODUCTION

Paraneoplastic cerebellar ataxia (PCA), also known as paraneoplastic cerebellar degeneration (PCD), is a heterogeneous disease group related to specific tumor types and most frequently, with antineural antibodies.[12] It is clinically characterised by the presence of pancerebellar dysfunction, dysarthria, dysphagia and nystagmus, which has subacute onset but rapid progression.[3] Although cerebellar ataxia was first defined in 1919, the relationship between it and cancer was first reported in 1938 by Brauwer and Biemond.[2] PCD is most frequently observed in gynaecological cancers, small cell lung cancer, breast cancer and Hodgkin's lymphoma, and rarely in testis cancer or malignant thymoma.[456]

Literature search did not reveal any data related to the effects of PCA during pregnancy and/or reports on the effects of anaesthesia in patients with PCA. We report a 37-year-old multiparous pregnant woman who was diagnosed with PCA and underwent spinal anaesthesia for elective caesarean section at the 39th week of gestation.

CASE REPORT

An elective caesarean was planned for a 37-year-old multiparous pregnant woman, weighing 80 kg and at 39th week of gestation. The patient initially presented to the hospital with sudden onset headache, dysarthria, somnolence and inability to stand or walk at 34th week of gestation and a neurologist consultation was obtained. There was swelling on the left side of her neck, which she had noticed at approximately 26th gestational week, and which had enlarged after a while. On physical examination, a painless mass measuring approximately 20 × 15 cm in diameter with an irregular borderline was palpated in the left submandibular region. On neurologic examination, there was dysarthria, bilateral intentional tremor, bilateral dysmetria, dysdiadochokinesia and ataxia that impaired the patient's posture and walking. There was loss of balance when the patient was in bed and closed her eyes. The patient was conscious and cooperative, and her place-time orientation was normal. Although she was unable to walk, motor strength was 5/5 in the lower and upper extremities. The sensory, cardiovascular and respiratory system examinations were normal. No pathological finding was detected on the cerebral-diffusion magnetic resonance imaging (MRI) and cranial MRI except for a few nonspecific gliotic foci. As the patient was being followed with the initial diagnosis of PCA, a caesarean section was planned after 39th week of gestation by the obstetrician in consultation with the neurologist.

The preoperative haemogram and biochemical laboratory results for the patient were as follows: haemoglobin 9.4 g dl-1, haematocrit 29.1%, leucocyte count 3,980/mm3. Platelet count, fasting blood glucose, blood urea, creatinine, electrolytes, liver function (including prothrombin time, activated partial thromboplastin time) were within normal limits.

Electrocardiogram, peripheral oxygen saturation and non-invasive blood pressure (BP) monitoring were performed. Preoperative BP was 126/85 mmHg and heart rate (HR) was 83 beats/min. The patient reviewed the case report and gave written permission for the authors to publish the report.

After the patient had been placed in the left decubitus position and the lumbar region had been disinfected, a 27-G (90 mm) pencil-point spinal needle was introduced into the intrathecal space at the L3-L4 intervertebral interspace. After observing the free flow of cerebrospinal fluid (CSF), 1 cc of CSF was obtained using the dropping technique for cytological examination, and 10 mg of hyperbaric bupivacaine HCl (Marcaine Spinal Heavy 0.5% ampule, 4 mL, AstraZeneca) was administered. As soon as the patient was placed in the supine position, the operating table was tilted to the left by approximately 15°. The level of sensory block was assessed using the pinprick test and the surgery was commenced after a T4 level of sensory block was achieved. Five minutes after the skin incision, a male infant weighing 3.3 Kg was extracted with first and fifth minute APGAR scores of 9 and 10 respectively. After clamping the umbilical cord, an infusion of 20 IU of oxytocin (Synpitan Forte ampule, 5 IU/mL, Deva) in 1000 mL of 0.9% NaCl was started. During the 55-minute operation, the patient's HR, BP and SpO2 were between 76 and 102 beats min-1, 142/96 mm Hg and 118/65 mmHg, and 97-99%, respectively. The motor block had regressed by the 2nd postoperative hour.

On the 3rd postoperative day, the patient was transferred to the oncology clinic. During the cancer screening, nasopharyngeal carcinoma was detected and treatment was started.

DISCUSSION

Paraneoplastic neurological syndromes are rarely seen neurological syndromes, and in the presence of cancer, they can develop without direct invasion, metastasis or the side effects of cancer therapy.[7] Paraneoplastic neurological syndromes can cause different neurological pictures, such as Lambert-Eaton myasthenic syndrome, subacute cerebellar ataxia, opsomyoclonus, limbic encephalitis, encephalomyelitis, sensorial neuropathy, retinopathy, etc.,[1] Neurological symptoms develop as a first sign of cancer in 70% of patients with paraneoplastic syndromes.[8] One of the most frequently observed paraneoplastic syndromes is PCA.[8] PCA demonstrates rapid progression and results in severe pancerebellar dysfunction.[7] As PCA can develop clinically several months before the diagnosis of cancer, it is necessary to suspect cancer in these patients.[7] Also, as in the current case, there was sudden-onset PCA, which developed two months before the clinical diagnosis of cancer. During the postoperative cancer screening, we concluded that the PCA had developed in the patient due to nasopharyngeal carcinoma.

Paraneoplastic cerebellar degeneration frequently develops before the diagnosis of cancer, but the paraneoplastic aetiology is confirmed by the detection of antineural antibodies.[5] Nine specific antineural antibodies related to PCD have been defined.[2] In a study investigating 137 patients with high titres of antineural antibodies, and in whom paraneoplastic neurological syndrome had been detected, it was reported that antibody-related PCD was present in 50 patients (36%) and among these, the presence of anti-Yo, anti-Hu, anti-Tr, anti-Ri and anti-mGluR1 antibodies was detected in 19, 16, 7, 6 and 2 patients respectively. It was reported that subacute cerebellar ataxia which progressed over weeks and months was present in all of the patients.[2] In an investigation conducted in patients with unexplained ataxia together with the presence of an underlying tumour, it was reported that the sensitivity of these autoantibodies, which are used in the determination of the paraneoplastic aetiology, was 50%–60%. As the sensitivity of these antibodies is insufficient, PCD might be present in the absence of positivity in even one of them.[9] Also in the current case, there was cerebellar ataxia with subacute onset and anti-Hu, anti-Yo, anti-Ri, anti-PNMA2/Ta, anti-CV2.1 and anti-amphiphysin antibodies were negative on the autoantibody examination, which was performed during the postoperative period to detect malignancy.

In patients with ataxia, due to the risk of aspiration, general anaesthesia is not seen as an acceptable alternative to regional anaesthesia for caesarean sections.[10] Barbiturates and non-depolarising and depolarising muscle relaxants can induce ataxia in Friedreich's ataxia where regional anaesthesia is accepted as more suitable than general anaesthesia.[1011] In the literature, epidural analgesia has been reported to be successful in pregnant patients with spinocerebellar ataxia.[10] There are no data relating to the effects of anaesthetic agents and muscle relaxants in PCA. Furthermore, there are no data demonstrating that spinal or epidural anaesthesia worsens the pathological and clinical course of PCA. Although epidural anaesthesia seems safe in patients with PCA, because of the need to sample CSF for cytological examination and intact motor function, we preferred single-shot spinal anaesthesia. General anaesthesia carries risk of aspiration, atypical response to neuromuscular agents, delay in reversal of airway responses and respiratory depression. No complications related to spinal anaesthesia developed in the intraoperative and postoperative follow-up periods.

CONCLUSION

We could successfully manage a pregnant patient with PCA posted for caesarean section under spinal anaesthesia. Regional anaesthesia is an acceptable choice and safer than general anaesthesia for patients with PCD.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.