Anti-Hu-associated paraneoplastic limbic encephalitis presenting as rapidly progressive non-convulsive status epilepticus. 2006

Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
The Neuroscience Institute, 4503 Medical Sciences Building, 231 Albert Sabin Way, Cincinnati, OH 45267-0525, USA. Alberto.espay@uc.edu

We report a 68-year-old woman who developed refractory non-convulsive generalized status epilepticus secondary to anti-Hu antibodies, detected by immunofluorescence and confirmed by Western immunoblotting. The patient presented with rapidly evolving impairment in consciousness and electroencephalographic evidence of lateralized pseudoperiodic sharp-wave discharges. Ataxia and sensory neuropathy developed within the first two weeks. To our knowledge, this is the first description of a very rapidly progressive non-convulsive status epilepticus of paraneoplastic origin. Serum anti-Hu antibodies deserve to be considered among the investigations required in the evaluation of rapidly progressive epileptic syndromes even when little or no imaging abnormalities are found.

UI MeSH Term Description Entries
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D004829 Epilepsy, Generalized Recurrent conditions characterized by epileptic seizures which arise diffusely and simultaneously from both hemispheres of the brain. Classification is generally based upon motor manifestations of the seizure (e.g., convulsive, nonconvulsive, akinetic, atonic, etc.) or etiology (e.g., idiopathic, cryptogenic, and symptomatic). (From Mayo Clin Proc, 1996 Apr;71(4):405-14) Convulsive Generalized Seizure Disorder,Epilepsy, Tonic,Generalized Nonconvulsive Seizure Disorder,Seizure Disorder, Generalized,Convulsive Seizure Disorder, Generalized,Epilepsy, Akinetic,Epilepsy, Atonic,Generalized Convulsive Epilepsy,Generalized Nonconvulsive Epilepsy,Generalized Onset Seizure Disorder,Generalized Seizure Disorder, Convulsive,Generalized Seizure Disorder, Nonconvulsive,Nonconvulsive Generalized Seizure Disorder,Nonconvulsive Seizure Disorder, Generalized,Seizure Disorder, Convulsive, Generalized,Seizure Disorder, Generalized Nonconvulsive,Seizure Disorder, Generalized Onset,Seizure Disorder, Generalized, Convulsive,Seizure Disorder, Nonconvulsive Generalized,Symptomatic Generalized Epilepsy,Akinetic Epilepsies,Akinetic Epilepsy,Atonic Epilepsies,Atonic Epilepsy,Convulsive Epilepsies, Generalized,Convulsive Epilepsy, Generalized,Epilepsies, Akinetic,Epilepsies, Atonic,Epilepsies, Generalized,Epilepsies, Generalized Convulsive,Epilepsies, Tonic,Epilepsy, Generalized Convulsive,Epilepsy, Generalized Nonconvulsive,Epilepsy, Symptomatic Generalized,Generalized Convulsive Epilepsies,Generalized Epilepsies,Generalized Epilepsy,Generalized Epilepsy, Symptomatic,Generalized Seizure Disorder,Generalized Seizure Disorders,Nonconvulsive Epilepsy, Generalized,Seizure Disorders, Generalized,Tonic Epilepsies,Tonic Epilepsy
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012907 Smoking Willful or deliberate act of inhaling and exhaling SMOKE from burning substances or agents held by hand. Smoking Behaviors,Smoking Habit,Behavior, Smoking,Behaviors, Smoking,Habit, Smoking,Habits, Smoking,Smoking Behavior,Smoking Habits
D013226 Status Epilepticus A prolonged seizure or seizures repeated frequently enough to prevent recovery between episodes occurring over a period of 20-30 minutes. The most common subtype is generalized tonic-clonic status epilepticus, a potentially fatal condition associated with neuronal injury and respiratory and metabolic dysfunction. Nonconvulsive forms include petit mal status and complex partial status, which may manifest as behavioral disturbances. Simple partial status epilepticus consists of persistent motor, sensory, or autonomic seizures that do not impair cognition (see also EPILEPSIA PARTIALIS CONTINUA). Subclinical status epilepticus generally refers to seizures occurring in an unresponsive or comatose individual in the absence of overt signs of seizure activity. (From N Engl J Med 1998 Apr 2;338(14):970-6; Neurologia 1997 Dec;12 Suppl 6:25-30) Absence Status,Complex Partial Status Epilepticus,Generalized Convulsive Status Epilepticus,Non-Convulsive Status Epilepticus,Petit Mal Status,Simple Partial Status Epilepticus,Grand Mal Status Epilepticus,Status Epilepticus, Complex Partial,Status Epilepticus, Electrographic,Status Epilepticus, Generalized,Status Epilepticus, Generalized Convulsive,Status Epilepticus, Grand Mal,Status Epilepticus, Non-Convulsive,Status Epilepticus, Simple Partial,Status Epilepticus, Subclinical,Electrographic Status Epilepticus,Generalized Status Epilepticus,Non Convulsive Status Epilepticus,Status Epilepticus, Non Convulsive,Status, Absence,Status, Petit Mal,Subclinical Status Epilepticus
D051959 ELAV Proteins A family of RRM PROTEINS that are homologues of ELAV protein, Drosophila. They were initially identified in humans as the targets of autoantibodies in patients with PARANEOPLASTIC ENCEPHALOMYELITIS. They regulate GENE EXPRESSION at the post-transcriptional level. Hu Paraneoplastic Encephalomyelitis Antigens,ELAV Protein,Embryonic Lethal, Abnormal Vision-Like Proteins,Hu Antigens,Antigens, Hu,Embryonic Lethal, Abnormal Vision Like Proteins,Protein, ELAV,Proteins, ELAV

Related Publications

Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
July 2012, Leukemia & lymphoma,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
September 2012, Developmental medicine and child neurology,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
July 2007, Clinical EEG and neuroscience,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
September 2003, Journal of the neurological sciences,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
October 2011, Neuropediatrics,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
March 2008, Acta neurologica Belgica,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
January 2010, The Journal of neuropsychiatry and clinical neurosciences,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
November 2019, Autoimmunity reviews,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
November 2005, Journal of neuro-oncology,
Alberto J Espay, and Vijay Kumar, and Günseli Sarpel
August 1992, Lancet (London, England),
Copied contents to your clipboard!