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|LETTER TO EDITOR
|Year : 2013 | Volume
| Issue : 5 | Page : 534-535
Paraneoplastic CRMP-5 basal ganglionitis and limbic encephalitis in an elderly Indian lady
Boby Varkey Maramattom
Department of Neurology, Lourdes Hospital, Pachalam, Kochi, Kerala, India
|Date of Submission||22-Jul-2013|
|Date of Decision||31-Jul-2013|
|Date of Acceptance||13-Oct-2013|
|Date of Web Publication||22-Nov-2013|
Boby Varkey Maramattom
Department of Neurology, Lourdes Hospital, Pachalam, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Maramattom BV. Paraneoplastic CRMP-5 basal ganglionitis and limbic encephalitis in an elderly Indian lady. Neurol India 2013;61:534-5
Collapsin response mediator proteins (CRMP) are a group of five phosphoproteins that play a vital role in neurite outgrowth and neuronal polarity.  Anti-CRMP-5 antibodies react with a 66 kDa protein found in the brain and the peripheral nerve.  Of these, paraneoplastic syndromes are well described with CRMP-5. We describe the first case of CRMP-5 associated basal ganglionitis and limbic encephalitis in an Indian lady with a primary lung tumor.
A 66-year-old housewife presented with a 3 month history of abnormal behavior, logorrhea, and visual and olfactory hallucinations. She was initially evaluated by a psychiatrist for psychosis and was started on atypical neuroleptics. During our evaluation, she also complained of patchy numbness in her arms, right hip, right side of the face and tongue, and recent memory impairment. She had recently lost 12 kg of weight. On examination, she scored 19/30 on the mini mental scale examination. She had mild choreic movements involving limbs and tongue. She had impaired position and vibration sense till her ankles bilaterally with diminished reflexes in lower limb. Plantar reflexes were flexor. She also had a wide based gait, but no Rhomberg's sign or cerebellar signs. Routine blood tests, X-ray chest, and ultrasound abdomen were normal.
A repeat magnetic resonance imaging (MRI) brain showed a symmetric T2/fluid attenuated inversion recovery (FLAIR) hyperintensity of the putamina and caudate heads and bodies without contrast enhancement as well as bilateral mesial temporal hyperintensities on FLAIR images [Figure 1]. Cerebrospinal fluid examination showed an opening pressure of 15.5 cm of water, 6 WBCs, glucose of 62 mg, and protein of 39 mg. Polymerase chain reaction (PCR) for John Cunningham (JC) virus, acid-fast Bacillus (AFB) stains, bacterial serology, and cytology for malignant cells were negative. Vasculitic work up and urine for heavy metals were negative. Nerve conduction studies showed low amplitude compound motor action potentials (CMAPs) with normal velocities and normal F-wave latencies. Needle examination showed polyphasic, short duration potentials, and scattered fibrillation potentials. An electroencephalogram (EEG) was normal. Computed tomography (CT) chest and abdomen were normal. An 18-F fluoro-deoxyglucose positron emission tomography (FDG-18 PET) scan demonstrated multiple foci of increased FDG-18 uptake in mildly enlarged lymph nodes within the mediastinum consistent with malignancy. The family was unwilling for further measures and she was discharged to domiciliary care with 40 mg/day of oral prednisolone. At 1 month, results of serum antibodies were positive for CRMP-5 (Oxford University, Immunology lab). Other antibodies including leucine-rich glioma inactivated 1 (LGi1), contactin associated protein-2 (CASPR2), Hu, Ri, Yo, Tr, Ma/Tr, glutamic acid decarboxylase (GAD), amphiphysin, aquaporin-4 (Aqp4), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA), and N-methyl-D-aspartate (NMDA) were negative. After 4 months, she expired at a local hospital of bronchogenic carcinoma.
|Figure 1: Magnetic resonance (MR) images. Panel (a and b) T1WI plain and contrast MRI are normal. Panel (c and d) T2WI and fluid attenuated inversion recovery (FLAIR) images show bilateral symmetrical basal ganglia hyperintensities. Panel (e) FLAIR image shows bilateral mesial temporal hyperintensities. Panel (f) diffusion weighted image is normal|
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CRMP-5 immunoglobulin (IgG) antibodies have been described predominantly in association with small cell lung carcinoma (SCLC; 77%) and thymoma (5%).  Paraneoplastic neurological associations include limbic encephalitis, chorea, cerebellar ataxia, myelopathy, radiculopathy, neuropathy, optic neuropathy, uveoretinal syndromes, myasthenia gravis, and Lambert-Eaton syndrome.  Late-onset chorea with basal ganglia hyperintensities may signal CRMP-5 encephalitis. , Our patient had a primary lung tumor. Plasmapheresis, intravenous Ig (IVIg), corticosteroids have been used in the treatment, but the most effective treatment is eradication of the underlying tumor. In conclusion, we present probably the first case of paraneoplastic CRMP-5 related basal ganglionitis and limbic encephalitis in an Indian lady.
| » References|| |
|1.||Brot S, Rogemond V, Perrot V, Chounlamountri N, Auger C, Honnorat J, et al. CRMP5 interacts with tubulin to inhibit neurite outgrowth, thereby modulating the function of CRMP2. J Neurosci 2010;30:10639-54. |
|2.||Stich O, Rauer S. Antigen-specific oligoclonal bands in cerebrospinal fluid and serum from patients with anti-amphiphysin- and anti-CV2/CRMP5 associated paraneoplastic neurological syndromes. Eur J Neurol 2007;14:650-3. |
|3.||Yu Z, Kryzer TJ, Griesmann GE, Kim K, Benarroch EE, Lennon VA. CRMP-5 neuronal autoantibody: Marker of lung cancer and thymoma-related autoimmunity. Ann Neurol 2001;49:146-54. |
|4.||McKeon A, Pittock SJ. Paraneoplastic encephalomyelopathies: Pathology and mechanisms. Acta Neuropathol 2011;122:381-400. |
|5.||Kinirons P, Fulton A, Keoghan M, Brennan P, Farrell MA, Moroney JT. Paraneoplastic limbic encephalitis (PLE) and chorea associated with CRMP-5 neuronal antibody. Neurology 2003;61:1623-4. |
|6.||O'Toole O, Lennon VA, Ahlskog JE, Matsumoto JY, Pittock SJ, Bower J, et al. Autoimmune chorea in adults. Neurology 2013;80:1133-44. |
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