Clinical Neurology and Neurosurgery 116 (2014) 99–100
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Limbic encephalitis and Lambert Eaton myasthenic syndrome – An immunological profile of a new syndrome S. Kalra a,∗ , P. Gozzard b , S. Jacob c , A. Leonard a , P. Maddison b a b c
Neurology Department, University Hospital North Staffordshire, Stoke-on-Trent, UK Neurology Department, Queen’s Medical Centre, Nottingham, UK Neurology Department, Queen Elizabeth Neuroscience Centre, University Hospitals of Birmingham, Birmingham, UK
a r t i c l e
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Article history: Received 18 January 2013 Received in revised form 25 August 2013 Accepted 19 October 2013 Available online 31 October 2013 Keywords: Lambert Eaton myasthenic syndrome Limbic encephalitis VGKC VGCC SOX antibodies
1. Introduction We report a case of limbic encephalitis and Lambert Eaton myasthenic Syndrome with a novel onco-neural profile: Voltage Gated Calcium Channel antibodies, high titre Voltage Gated Potassium Channel complex antibodies of non-LGI1 non-Caspr2 type and Srylike high mobility box (SOX2) antibodies with probable small cell lung carcinoma. 2. Case report A 76-year-old heavy cigarette smoker presented with a 6-month history of dry mouth, dry cough, anorexia, significant weight loss, dysarthria and dysphagia, dizziness, limb weakness, and reduced mobility. Examination showed postural hypotension with systolic blood pressure drop of >40 mmHg. He had bilateral ptosis and facial weakness, bulbar palsy, proximal limb wasting and weakness, and diminished deep tendon reflexes with distinct post-exercise potentiation suggestive of Lambert–Eaton myasthenic syndrome (LEMS). Over the course of 6 weeks he developed fluctuating confusion, behavioural change, disorientation, hypersomnolence, and complex partial seizures suggestive of limbic encephalitis (LE). Autonomic dysfunction worsened with onset of excessive sweating, itching, urinary retention and constipation.
∗ Corresponding author. Tel.: +44 7973479585. E-mail address: [email protected] (S. Kalra). 0303-8467/$ – see front matter © 2013 Published by Elsevier B.V. http://dx.doi.org/10.1016/j.clineuro.2013.10.014
Electromyography confirmed LEMS with 40 Hz repetitive stimulation producing >300% rise in amplitude of the compound muscle action potential from the first dorsal interosseous muscle. There were no features of neuromyotonia. Magnetic Resonance Imaging of the brain was normal. Electroencephalography showed generalised slowing with a suspicion of epileptiform discharges from temporal lobes. Extensive laboratory investigations including creatine kinase, lactate dehydrogenase, angiotensin converting enzyme, antinuclear antibody, anti-neutrophilic cytoplasmic antibody, extractable nuclear antigen antibody and tumour markers were all negative. Computerised Tomography chest, abdomen and pelvis imaging revealed sub-mental, sub-clavicular, hilar, para-aortic and portocaval lymphadenopathy. Positron Emission Tomography (PET) showed increased uptake in lymph nodes. Attempts at needle aspiration and open biopsy were non-diagnostic and tissue diagnosis could not be made. Multiple pharmacological interventions to treat postural hypotension were ineffective and his neurological symptoms failed to respond to 3,4-diaminopyridine, intravenous steroids, and intravenous immunoglobulin. He declined lumbar puncture and further investigations, deteriorated further, and later died of sepsis. Postmortem was not performed. Voltage gated potassium channel complex (VGKC) antibodies were detected at very high titres of 2337 pM (normal
Contents lists available at ScienceDirect
Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro
Case report
Limbic encephalitis and Lambert Eaton myasthenic syndrome – An immunological profile of a new syndrome S. Kalra a,∗ , P. Gozzard b , S. Jacob c , A. Leonard a , P. Maddison b a b c
Neurology Department, University Hospital North Staffordshire, Stoke-on-Trent, UK Neurology Department, Queen’s Medical Centre, Nottingham, UK Neurology Department, Queen Elizabeth Neuroscience Centre, University Hospitals of Birmingham, Birmingham, UK
a r t i c l e
i n f o
Article history: Received 18 January 2013 Received in revised form 25 August 2013 Accepted 19 October 2013 Available online 31 October 2013 Keywords: Lambert Eaton myasthenic syndrome Limbic encephalitis VGKC VGCC SOX antibodies
1. Introduction We report a case of limbic encephalitis and Lambert Eaton myasthenic Syndrome with a novel onco-neural profile: Voltage Gated Calcium Channel antibodies, high titre Voltage Gated Potassium Channel complex antibodies of non-LGI1 non-Caspr2 type and Srylike high mobility box (SOX2) antibodies with probable small cell lung carcinoma. 2. Case report A 76-year-old heavy cigarette smoker presented with a 6-month history of dry mouth, dry cough, anorexia, significant weight loss, dysarthria and dysphagia, dizziness, limb weakness, and reduced mobility. Examination showed postural hypotension with systolic blood pressure drop of >40 mmHg. He had bilateral ptosis and facial weakness, bulbar palsy, proximal limb wasting and weakness, and diminished deep tendon reflexes with distinct post-exercise potentiation suggestive of Lambert–Eaton myasthenic syndrome (LEMS). Over the course of 6 weeks he developed fluctuating confusion, behavioural change, disorientation, hypersomnolence, and complex partial seizures suggestive of limbic encephalitis (LE). Autonomic dysfunction worsened with onset of excessive sweating, itching, urinary retention and constipation.
∗ Corresponding author. Tel.: +44 7973479585. E-mail address: [email protected] (S. Kalra). 0303-8467/$ – see front matter © 2013 Published by Elsevier B.V. http://dx.doi.org/10.1016/j.clineuro.2013.10.014
Electromyography confirmed LEMS with 40 Hz repetitive stimulation producing >300% rise in amplitude of the compound muscle action potential from the first dorsal interosseous muscle. There were no features of neuromyotonia. Magnetic Resonance Imaging of the brain was normal. Electroencephalography showed generalised slowing with a suspicion of epileptiform discharges from temporal lobes. Extensive laboratory investigations including creatine kinase, lactate dehydrogenase, angiotensin converting enzyme, antinuclear antibody, anti-neutrophilic cytoplasmic antibody, extractable nuclear antigen antibody and tumour markers were all negative. Computerised Tomography chest, abdomen and pelvis imaging revealed sub-mental, sub-clavicular, hilar, para-aortic and portocaval lymphadenopathy. Positron Emission Tomography (PET) showed increased uptake in lymph nodes. Attempts at needle aspiration and open biopsy were non-diagnostic and tissue diagnosis could not be made. Multiple pharmacological interventions to treat postural hypotension were ineffective and his neurological symptoms failed to respond to 3,4-diaminopyridine, intravenous steroids, and intravenous immunoglobulin. He declined lumbar puncture and further investigations, deteriorated further, and later died of sepsis. Postmortem was not performed. Voltage gated potassium channel complex (VGKC) antibodies were detected at very high titres of 2337 pM (normal
