Paraneoplastic cerebellar syndrome and sensory ganglionopathy with papillary thyroid carcinoma James P. Gratwicke, Adebayo Alli, Matthew Rollin, Francis Vaz, Jeremy Rees, Angela Vincent, Mark Edwards PII: DOI: Reference:
S0022-510X(14)00201-9 doi: 10.1016/j.jns.2014.03.042 JNS 13120
To appear in:
Journal of the Neurological Sciences
Received date: Revised date: Accepted date:
19 January 2014 1 March 2014 21 March 2014
Please cite this article as: Gratwicke James P., Alli Adebayo, Rollin Matthew, Vaz Francis, Rees Jeremy, Vincent Angela, Edwards Mark, Paraneoplastic cerebellar syndrome and sensory ganglionopathy with papillary thyroid carcinoma, Journal of the Neurological Sciences (2014), doi: 10.1016/j.jns.2014.03.042
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Paraneoplastic cerebellar syndrome and sensory
PT
ganglionopathy with papillary thyroid carcinoma James P. Gratwicke MA(Oxon) BMBCh MRCP1*, Adebayo Alli MA(Oxon) MRCS(DO-HNS)2,
RI
Matthew Rollin FRCS(ORL-HNS)2, Francis Vaz FRCS(ORL-HNS)2, Jeremy Rees BSc MBBS PhD FRCP1, Angela Vincent MBBS PhD FRCPath FMedSci3 and Mark Edwards BSc MBBS PhD1
SC
_____________________________________________________________________ The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK
2.
Department of Ear, Nose & Throat Surgery, University College London Hospital, 235 Euston Road, London,
NU
1.
NW12BU, UK 3.
Neurosciences Group, Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital,
MA
Oxford, OX3 9DS, UK
* Corresponding Author: Dr. James Gratwicke, Sobell Department of Motor Neuroscience and Movement disorders, UCL Institute of Neurology, 33 Queen Square, London, WC1N 3BG, UK
D
Email: [email protected], Tel: +44 203 448 8736, Fax: +44 207 419 1860.
TE
Keywords: paraneoplastic neurological syndromes, thyroid carcinoma, cerebellar syndrome, sensory
AC CE P
ganglionopathy, VGKC-complex antibodies, CRMP-5 antibodies Word Count: (case report) 755 words
Dear Sir,
Paraneoplastic neurological disorders (PND) are syndromes caused by an autoimmune pathogenesis against ‘onconeural antigens’ shared between a tumour and the nervous system [1]. They are rare but important and can cause severe neurologic disability if not recognised and treated early. In addition, their clinical presentation often precedes identification of the associated cancer and alerts the physician to perform a thorough search for malignancy [2]. Here we describe the first reported case of cerebellar syndrome and sensory ganglionopathy as paraneoplastic phenomena in association with papillary thyroid carcinoma.
1
ACCEPTED MANUSCRIPT Case Report A seventy-one year-old man presented with a four-month history of progressive unsteadiness descending stairs and numbness in his fingers. His gait
PT
was broad-based, there was bilateral dysdiadochokinesia and dysmetria and gazeevoked nystagmus. Tone and power were normal. Reflexes were present in the
RI
upper limbs but absent at the knees and ankles bilaterally. Plantar responses were
SC
equivocal. Vibration perception was decreased to his clavicles and proprioception to the medial malleoli. There were no signs of autonomic involvement. He was a mild
NU
asthmatic but took no regular medications.
MR imaging of the brain and spinal cord were normal. Nerve conduction
MA
studies showed absent sensory nerve action potentials (SNAPs) in the lower limbs and reduced SNAP amplitudes in right radial and ulnar nerves. Motor nerve conduction was normal. These findings indicated a pure sensory ganglionopathy.
D
Lumbar puncture revealed a raised protein (0.75g/L) but oligoclonal bands were
TE
negative in serum and CSF. A whole body PET/CT scan showed a 1.1 x 0.9 cm FDGavid nodule in the right lobe of the thyroid. Fine-needle aspiration yielded a cellular
AC CE P
specimen consistent with papillary thyroid carcinoma (Fig. 1). Two distinct neural autoantibodies were detected in the serum: collapsin-response mediator protein 5 (CRMP-5) IgG was weakly positive on immunoblot, and voltage-gated potassium channel (VGKC)-complex antibodies were positive on immunoprecipitation with a titre of 1070 pM/L (normal range
S0022-510X(14)00201-9 doi: 10.1016/j.jns.2014.03.042 JNS 13120
To appear in:
Journal of the Neurological Sciences
Received date: Revised date: Accepted date:
19 January 2014 1 March 2014 21 March 2014
Please cite this article as: Gratwicke James P., Alli Adebayo, Rollin Matthew, Vaz Francis, Rees Jeremy, Vincent Angela, Edwards Mark, Paraneoplastic cerebellar syndrome and sensory ganglionopathy with papillary thyroid carcinoma, Journal of the Neurological Sciences (2014), doi: 10.1016/j.jns.2014.03.042
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Paraneoplastic cerebellar syndrome and sensory
PT
ganglionopathy with papillary thyroid carcinoma James P. Gratwicke MA(Oxon) BMBCh MRCP1*, Adebayo Alli MA(Oxon) MRCS(DO-HNS)2,
RI
Matthew Rollin FRCS(ORL-HNS)2, Francis Vaz FRCS(ORL-HNS)2, Jeremy Rees BSc MBBS PhD FRCP1, Angela Vincent MBBS PhD FRCPath FMedSci3 and Mark Edwards BSc MBBS PhD1
SC
_____________________________________________________________________ The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N 3BG, UK
2.
Department of Ear, Nose & Throat Surgery, University College London Hospital, 235 Euston Road, London,
NU
1.
NW12BU, UK 3.
Neurosciences Group, Department of Clinical Neurology, University of Oxford, John Radcliffe Hospital,
MA
Oxford, OX3 9DS, UK
* Corresponding Author: Dr. James Gratwicke, Sobell Department of Motor Neuroscience and Movement disorders, UCL Institute of Neurology, 33 Queen Square, London, WC1N 3BG, UK
D
Email: [email protected], Tel: +44 203 448 8736, Fax: +44 207 419 1860.
TE
Keywords: paraneoplastic neurological syndromes, thyroid carcinoma, cerebellar syndrome, sensory
AC CE P
ganglionopathy, VGKC-complex antibodies, CRMP-5 antibodies Word Count: (case report) 755 words
Dear Sir,
Paraneoplastic neurological disorders (PND) are syndromes caused by an autoimmune pathogenesis against ‘onconeural antigens’ shared between a tumour and the nervous system [1]. They are rare but important and can cause severe neurologic disability if not recognised and treated early. In addition, their clinical presentation often precedes identification of the associated cancer and alerts the physician to perform a thorough search for malignancy [2]. Here we describe the first reported case of cerebellar syndrome and sensory ganglionopathy as paraneoplastic phenomena in association with papillary thyroid carcinoma.
1
ACCEPTED MANUSCRIPT Case Report A seventy-one year-old man presented with a four-month history of progressive unsteadiness descending stairs and numbness in his fingers. His gait
PT
was broad-based, there was bilateral dysdiadochokinesia and dysmetria and gazeevoked nystagmus. Tone and power were normal. Reflexes were present in the
RI
upper limbs but absent at the knees and ankles bilaterally. Plantar responses were
SC
equivocal. Vibration perception was decreased to his clavicles and proprioception to the medial malleoli. There were no signs of autonomic involvement. He was a mild
NU
asthmatic but took no regular medications.
MR imaging of the brain and spinal cord were normal. Nerve conduction
MA
studies showed absent sensory nerve action potentials (SNAPs) in the lower limbs and reduced SNAP amplitudes in right radial and ulnar nerves. Motor nerve conduction was normal. These findings indicated a pure sensory ganglionopathy.
D
Lumbar puncture revealed a raised protein (0.75g/L) but oligoclonal bands were
TE
negative in serum and CSF. A whole body PET/CT scan showed a 1.1 x 0.9 cm FDGavid nodule in the right lobe of the thyroid. Fine-needle aspiration yielded a cellular
AC CE P
specimen consistent with papillary thyroid carcinoma (Fig. 1). Two distinct neural autoantibodies were detected in the serum: collapsin-response mediator protein 5 (CRMP-5) IgG was weakly positive on immunoblot, and voltage-gated potassium channel (VGKC)-complex antibodies were positive on immunoprecipitation with a titre of 1070 pM/L (normal range
