Downloaded from http://jnnp.bmj.com/ on December 17, 2014 - Published by group.bmj.com PostScript the other hand, our patient did not show hyper-reflexia, but the Babinski sign was present along with prolongation of the CMCT. In patients of AMAN with hyperreflexia, it is speculated that inflammation in the nerve roots possibly leads to disruption of the bloodeCNS barrier and allows antiganglioside antibodies or other inflammatory substances to access neural structures near the anterior horn cells, especially the intramedullary collateral branches to the inhibitory interneurons resulting in the dysfunction of the inhibitory system via spinal interneurons or of the upper motor neuron.2 Experimental studies showed that anti-GM1 serum injected in the subarachnoid space causes damage to the central axons in the spinal cord as well as the nerve root.5 Thus, we considered that the presence of the Babinski sign and prolongation of the CMCT would be due to direct pyramidal tract involvement and were caused by the immune-mediated process that affected the peripheral nervous system. Since immune-mediated processes associated with anti-GM1 antibody may extend not only to the peripheral nervous system but also to the corticospinal tract, the presence of the Babinski sign does not necessarily exclude the possibility of GuillaineBarré syndrome. M Tomita,1 H Watanabe,1 S Morozumi,1 Y Kawagashira,1 M Iijima,1 T Nakamura,1 M Katsuno,1 H Koike,1 N Hattori,1 M Hirayama,1 S Kusunoki,2 G Sobue1 1 Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan; 2Department of Neurology, Kinki University School of Medicine, Osaka, Japan Correspondence to Professor Gen Sobue, Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan; sobueg@med. nagoya-u.ac.jp Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. Received 2 June 2009 Revised 21 July 2009 Accepted 22 July 2009 J Neurol Neurosurg Psychiatry 2010;81:583e585. doi:10.1136/jnnp.2009.184945 REFERENCES 1. 2. 3. 4. Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for GuillaineBarré syndrome. Ann Neurol 1990;27Suppl):S21e4. Kuwabara S, Ogawara K, Koga M, et al. Hyperreflexia in GuillaineBarré syndrome: relation with acute motor axonal neuropathy and anti-GM1 antibody. J Neurol Neurosurg Psychiatr 1999;67:180e4. Oshima Y, Mitsui T, Yoshino H, et al. Central motor conduction in patients with anti-ganglioside antibody associated neuropathy syndromes and hyperreflexia. J Neurol Neurosurg Psychiatr 2002;73:568e73. Kusunoki S, Morita D, Ohminami S, et al. Binding of immunoglobulin G antibodies in GuillaineBarré syndrome sera to a mixture of GM1 and a phospholipid: possible clinical implications. Muscle Nerve 2003;27:302e6. J Neurol Neurosurg Psychiatry May 2010 Vol 81 No 5 5. Schwerer B, Lassmann H, Kitz K, et al. Ganglioside GM1, a molecular target for immunological and toxic attacks: similarity of neuropathological lesions induced by ganglioside-antiserum and cholera toxin. Acta Neuropathol 1986;72:55e61. Stroke in the puerperium treated with intra-arterial rt-PA Pregnancy and the puerperium are prothrombotic states. Stroke is rare in pregnancy and the puerperium but is the cause of death in 12% of all maternal deaths.1 Pregnancy-related strokes occur late in pregnancy or in the postpartum period, with the highest risk after delivery.2 Hypercoagulopathy, venous stasis, endothelial injury, postpartum angiopathy, cerebral aneurysms, paradox embolism and cerebral venous thrombosis are all known possible aetiologies of pregnancy-associated stroke. One rare cause of acute ischaemic stroke in young women is cardiac thromboembolism caused by peripartum cardiomyopathy (PPCM). Cardiomyopathy is a known cause of cardioembolic stroke. PPCM is a cardiomyopathy that presents in the peripartum period in patients without a known preexisting heart disease. The diagnosis is based on exclusion of other possible causes of cardiomyopathy. PPCM presents most often within the last months of pregnancy and the first 6 months after. Pregnancy and the first 4 weeks after delivery are considered exclusion criteria for thrombolysis following acute stroke. More than 200 patients, however, are reported to have been treated with fibrinolytics in pregnancy. In one case, urokinase was used in the early postpartum period.3 In this case, we report the successful use of intra-arterial alteplasedrtPA (actilyse, Boehringer Ingelheim) for ischaemic stroke in the puerperium in a patient with peripartum cardiomyopathy. CASE REPORT The patient, a 29-year-old primigravid, developed pre-eclampsia in the week 38. Delivery was induced, and she gave birth to a healthy child. During the next 3 days, she developed tachycardia and dyspnoea with orthopnoea as the most prominent feature. She complained of precordial pain and abdominal discomfort. Her blood pressure was 120/70 and heart rate was 130 bpm. She was investigated for pulmonary embolism. Oxygen saturation was 90%, Po2 8.6, Pco2 3.4, haemoglobin 9.8 g/dl, leucocytes 14.63105, CRP 33 mg/l, creatinine 86 mmol/l, albumin 19 g/l and troponin-T 0.17 mg/l. Computer tomography of the chest including pulmonary vessels revealed pleural and pericardial effusion but normal pulmonary vessels. A chest drain was inserted and 1.4 l of transudate pleural fluid drained. Echocardiography 6 days after birth showed a left ventricular ejection fraction of 30%. There was a general hypokinesia, most prominent in the apical part of the left ventricle. A left ventricular thrombus was detected as an echo-dense pendulating mass. She was treated for heart congestion with an ACE inhibitor and a diuretic. To combat thromboembolism, she received unfractionated heparin. She developed an acute onset right-sided hemiplegia with global aphasia. The National Institutes of Health Stroke Scale (NIHSS) score was 14. Non-enhanced CT of the brain showed no haemorrhage or any ischaemic changes. On admission, perfusion CT scans showed a mismatch between mean transit time and cerebral blood volume. CT angiography 2 h and 45 min after stroke onset showed occlusion of the M2 segment of the left middle cerebral artery (MCA). Given recent delivery and anticoagulation therapy, we decided to proceed to intraarterial thrombolysis. Selective intermittent injection of a total of 20 mg of intra-arterial rt-PA via microcatheter resulted in recanalisation of the main trunk of the M2. After treatment, flow was re-established in M2 of the left MCA on CT angiography. Her neurological status improved rapidly over the first few hours, and on examination the next day she had only a mild right facial paralysis, reduced tempo of the right hand, a partial non-fluent aphasia but normal comprehension. A cerebral MRI showed a left-sided infarct in the rostral part of the frontal lobe. There was no sign of haemorrhagic transformation. Diagnostic work up did not reveal any carotid atherosclerosis, coagulopathy or immunological disease. The patient gradually improved, and 4 months after stroke she had an NIHSS of 1 and a modified Rankin score of 1. Echocardiography 8 months after stroke showed normalisation of the ventricular ejection fraction. The safety of thrombolysis in acute stroke in the early puerperium is not proven. The major concern is severe uterine haemorrhage. There are a few case reports that show a beneficial effect of thrombolysis in acute ischaemic stroke in pregnancy.4 5 Only one case has reported the use of thrombolysis in stroke in the puerperium. Mendez and collaborators describe a patient with a good outcome after intra-arterial treatment with urokinase in the immediate postpartum period.3 Pregnancy-related ischaemic stroke is a challenging medical problem. Many of the strokes reported late in pregnancy have large arterial branch occlusions. These strokes are associated with a poor outcome. Taking into account the concerns of intravenous thrombolytic therapy including the risk of haemorrhage and, particularly in large arterial branch occlusions, a decreased probability of recanalisation, one should consider intra-arterial thrombolysis or thrombendarterectomy. This case report is the first to our knowledge that shows a good outcome after intra-arterial thrombolysis with rtPA in stroke in the early puerperium. 585 Downloaded from http://jnnp.bmj.com/ on December 17, 2014 - Published by group.bmj.com PostScript Ole Morten Rønning,1 Arve Dahl,2 Søren Jacob Bakke,3 Amjad Iqbal Hussain,4 Ellen Deilkås1 1 Department of Neurology, Akershus University Hospital, Lørenskog, Norway; 2Department of Neurology, The National Hospital, Oslo, Norway; 3Department of Radiology, The National Hospital, Oslo, Norway; 4 Department of Medicine, Akershus University Hospital, Lørenskog, Norway Correspondence to Professor Ole Morten Rønning, Department of Neurology, Akershus University Hospital, Lørenskog 1478, Norway; omro@online.no Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. Received 23 June 2009 Revised 22 July 2009 Accepted 23 July 2009 J Neurol Neurosurg Psychiatry 2010;81:585e586. doi:10.1136/jnnp.2009.186965 REFERENCES 1. 2. 3. 4. 5. Simolke GA, Cox SA, Cunningham FG. Cerebrovascular accidents complicating pregnancy and the puerperium. Obstet Gynecol 1991;78:27e42. Jaigobin C, Silver FL. Stroke and pregnancy. Stroke 2000;31:2948e51. Mendez JC, Masjuan J, Garcia N, et al. Successful intra-arterial thrombolysis for acute ischemic stroke in the immediate postpartum period: case report. Cardiovasc Intervent Radiol 2008;31;193e5. Dapprich M, Boessenecker W. Fibrinolysis with alteplase in a pregnant woman with stroke. Cerebrovasc Dis 2002;13:290. Murugappan A, Coplin WM, Al-Sadat AN, et al. Thrombolytic therapy of acute ischemic stroke during pregnancy. Neurology 2006;66:768e70. Slowly progressive autonomic neuropathy with antiganglionic acetylcholine receptor antibody The antiganglionic acetylcholine receptor (AChR) antibody is a serological marker for autoimmune autonomic ganglionopathy (AAG).1 2 Typical AAG cases show monophasic progression evolving to peak severity within a few days to weeks.1 3 Before the establishment of AAG as a disease entity, it was called acute pandysautonomia, autoimmune autonomic neuropathy, idiopathic autonomic neuropathy or subacute autonomic neuropathy.1 2 While most cases of AAG show an acute or subacute course to reach its nadir, similar to the somatic counterpart GuillaineBarré syndrome, the nosological position of AAG cases with a chronic course is obscure. In this report, we describe the clinical features and sural nerve biopsy findings in a case with slowly progressive autonomic neuropathy and positive for antiganglionic AChR antibody followed up for 15 years. CASE REPORT A male patient noted faintness upon standing at age 61 (in 1994, 7 years before 586 Figure 1 Transverse section of a sural nerve biopsy from the patient. (A) Light microscopy. Both large and small myelinated fibre densities were preserved. (B) Electron microscopy. A reduction in unmyelinated fibres was evident with an increase in empty Schwann cell subunits and scattered collagen pockets (arrowheads). admission to our hospital). Not long afterwards, he felt palpitation, shortness of breath and dizziness during walking. He sometimes lost consciousness while walking after he felt such symptoms. He suffered from diarrhoea 4 years later. Family history was negative. As the loss of consciousness and diarrhoea gradually became frequent, he was admitted to a hospital at age 67, where hypohidrosis and dry mouth were observed. A biopsy specimen from the rectal mucosa revealed no amyloid deposition. He had loose or watery stool up to 10 times a day at that time. He was subsequently referred to our hospital for further examination in March 2001 (age 68). On admission, he was alert and well oriented. The cranial nerves were intact, except for bilateral mydriasis. Sensation was normal, including pain, light touch, vibration and position. Muscular weakness and atrophy were not noted. Although a slight increase in the patellar tendon J Neurol Neurosurg Psychiatry May 2010 Vol 81 No 5 Downloaded from http://jnnp.bmj.com/ on December 17, 2014 - Published by group.bmj.com Stroke in the puerperium treated with intra-arterial rt-PA Ole Morten Rønning, Arve Dahl, Søren Jacob Bakke, Amjad Iqbal Hussain and Ellen Deilkås J Neurol Neurosurg Psychiatry 2010 81: 585-586 doi: 10.1136/jnnp.2009.186965 Updated information and services can be found at: http://jnnp.bmj.com/content/81/5/585 These include: References Email alerting service This article cites 5 articles, 2 of which you can access for free at: http://jnnp.bmj.com/content/81/5/585#BIBL Receive free email alerts when new articles cite this article. Sign up in the box at the top right corner of the online article. 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