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Sign In or Create an Account. Sign In. Advanced Search. Search Menu. Article Navigation. Close mobile search navigation Article Navigation. Volume Article Contents Abstract. Design and methods. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Humayun Gultekin , S. Humayun Gultekin. Oxford Academic. Google Scholar. Myrna R. Raymond Voltz. Joseph Eichen. Jerome B. Josep Dalmau. Revision received:.
Cite Cite S. Select Format Select format. Permissions Icon Permissions. Abstract Paraneoplastic limbic encephalitis PLE is a rare disorder characterized by personality changes, irritability, depression, seizures, memory loss and sometimes dementia. Tumour type. Neurological improvement with treatment type. Henson et al. Open in new tab. Table 2 Tumours associated with paraneoplastic limbic encephalitis. Type of tumour. Current series 50 patients. English literature 72 patients. Table 3 Clinical features.
Table 4 Diagnostic tests. Paraneoplastic antibodies. Three patients without temporal—limbic MRI findings had white matter abnormalities in other regions: 1 multifocal, 1 diffuse, and 1 perithalamic and deep white matter changes. These include diffuse, non-specific slowing 3 and posterior triphasic waves 1. Table 5 Immunological subsets of paraneoplastic limbic encephalitis.
Clinical features. Anti-Hu 18 patients. Anti-Ta 10 patients. Table 6 Neurological outcome in 34 patients with PLE. Treatment of the tumour only 11 2 1 Immune modulation only 0 0 4 Treatment of the tumour and immune modulation 4 6 4 No treatment 0 0 2. Open in new tab Download slide.
Rev Neurol Paris. South Med J. J Neurol Neurosurg Psychiatry. Arch Neurol. J Neurooncol. J Urol. Am J Med. Brain Behav Immun. N Engl J Med. Br Med J. Ann Neurol. Biol Psychiatry. Ann Thorac Surg. Medicine Baltimore. Brain Pathol. Acta Neuropathol Berl. Acta Neuropathol Suppl Berl. Gen Hosp Psychiatry. Can J Neurol Sci. Clin Electroencephalogr. S Afr Med J. Am J Reprod Immunol Microbiol. J Neurol. Acta Neurol Scand. Psychiatry Clin Neurosci. Can Assoc Radiol J.
Folia Neuropathol. Acta Pathol Jpn. J Neuropathol Exp Neurol. J Neuroimmunol. J Comput Assist Tomogr. Ann Intern Med. J Int Neuropsychol Soc. Pediatr Radiol. J Exp Med. Schweiz Arch Neurol Neurochir Psychiatry. Br J Haematol. Ital J Neurol Sci. Prognosis Prognosis. The prognosis for limbic encephalitis varies from person to person and is based on the underlying cause of the condition.
For example, individuals that have small cell lung carcinoma SCLC and test positive for an antibody called anti-Hu, tend to respond poorly to treatment, whereas individuals that have an ovarian teratoma and test positive for the NMDAR antibody respond well to treatment. Organizations Organizations. Organizations Supporting this Disease. Do you know of an organization? Learn More Learn More. Where to Start The Encephalitis Society offers a factsheet on Limbic encephalitis The International Autoimmune Encephalitis Society offers patients and families detailed information on diagnosis and treatment.
In-Depth Information Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free. The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health.
Visit the website to explore the biology of this condition. Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge. PubMed is a searchable database of medical literature and lists journal articles that discuss Limbic encephalitis. Click on the link to view a sample search on this topic. Lancet Neurol. J Neurol Neurosurg Psychiatry.
Cleveland Clinic Journal of Medicine. Have a question? References References. Limbic encephalitis - a review. Journal of Clinical Neuroscience. Asztely F, Kumlien E.. The diagnosis and treatment of limbic encephalitis. Acta Neurol Scand. Dec ; 6 Limbic Encephalitis. The Encephalitis Society. Etiologies include limbic encephalitis which is a result of autoimmune-induced inflammation of the limbic system, which encompasses the thalamus, amygdala, hypothalamus, and mammillary bodies and is critical in memory, behavior, and emotion.
Limbic encephalitis presents most commonly with memory loss with onset over the course of weeks to months. Other symptoms include confusion, seizures, and psychogenic features such as anxiety, depression, hallucinations, and personality changes [ 1 ]. Limbic encephalitis can be caused by autoimmune process, which may be paraneoplastic or nonparaneoplastic.
Paraneoplastic limbic encephalitis is usually associated with small cell lung cancer, testicular tumors, and thymomas [ 2 ], whereas nonparaneoplastic limbic encephalitis has been attributed to antibodies against different channel proteins or receptors in the nervous system. Characterization of these antibodies is relatively recent, with anti-voltage-gated potassium channel VGKC antibodies being reported in [ 3 ].
Unfortunately, these specialized antibody assays are only available in some research laboratories, and therefore diagnosis is often missed or delayed when finally made. We present a case of a middle-aged female who was brought in by her family for acute onset memory deficits and ataxia, complicated by in-hospital seizures, with the only finding of syndrome of inappropriate antidiuretic hormone SIADH on initial evaluation which did not improve despite correction of sodium levels.
She met clinical criteria for limbic encephalitis and received immunotherapy only to be later diagnosed with anti-VGKC limbic encephalitis. A year-old female with a known medical history of rheumatoid arthritis and chronic pain syndrome was brought to the hospital by her family for a 2-week history of intermittent episodes of confusion, short-term memory loss, slurred speech, increased somnolence, and gait instability.
A complete review of systems was also positive for intermittent dizziness, minor falls, and pain at multiple locations. Her medications included methotrexate, golimumab, folic acid, tramadol, and diclofenac as needed for pain.
Family history was significant for coronary artery disease in her father. She worked as a cashier and was a lifetime nonsmoker and denied alcohol and illicit drug use. Initial physical exam revealed good personal hygiene, normal vital signs, mild swelling of both her wrist joints without any bony deformities, and an unremarkable heart, lung, and abdominal exam.
No rash on skin exam. Initial laboratory investigations were unremarkable except hyponatremia details in Table 1. A serum pregnancy test was negative. CT scan of the head without contrast did not reveal any evidence of intracranial hemorrhage. An MRI of the brain and cervical spine with and without contrast did not reveal any obvious abnormalities except minimal cervical spondylosis at C4—C6 levels.
The patient was admitted for altered mental status in the setting of syndrome of inappropriate antidiuretic hormone SIADH and started on fluid restriction and eventually urea tablets. Her methotrexate, golimumab, and diclofenac were held. On hospital day 2, the patient was noted to have twitching of her right upper extremity without any other focal neurological deficits.
She also did not remember the events of the prior day. Neurology service was consulted; the patient was loaded with levetiracetam and placed on a hour video EEG monitoring. Twelve hours into EEG monitoring, she was noted to have left-sided periodic lateralized epileptiform discharges PLEDs and generalized slowing of her background waves. A lumbar puncture was performed and was remarkable for minimally elevated opening pressure and a high IgG index Table 1.
Due to lack of improvement in clinical status and EEG findings of PLEDs, a brain MRI was repeated with coronal sections and revealed T2 hyperintensity with mild swelling of the left hippocampus with abnormal contrast enhancement Figure 1.
A CT scan of her head, neck, chest, abdomen, and pelvis was performed next to look for primary tumors that could be causing a paraneoplastic encephalitis, but these were negative for any abnormalities. A repeat video EEG did not reveal any seizures or epileptiform activities this time but did show mild to moderate slowing of the background. At this time, the patient was noted to be walking around the unit without ataxia and her memory had improved mildly.
The patient was discharged with close outpatient neurology follow-up. Therefore, a retrospective diagnosis of anti-VGKC limbic encephalitis was made. Antibodies to leucine-rich glioma-inactivated protein-1 anti-LGl1 and contactin-associated protein-2 Caspr2 were not tested. Patient returned to the hospital 1 month after discharge with recurrent seizures, short-term memory loss, and ataxia.
She was treated with a 5-day course of plasmapheresis followed by improvement in her neurological status and discharged home with outpatient neurology follow-up. She will continue to follow-up with Neurology every month. Alteration in mental status is a common patient presentation, and the differential diagnosis includes primary neurological as well as secondary causes like metabolic, toxic, hypoxic, vascular, psychiatric, and infectious.
Basic evaluation includes cardiac monitoring, measuring serum glucose, electrolytes, alcohol and drug levels, arterial blood gas, thyroid function tests, urine analysis with toxicology, lumbar puncture, and imaging studies. Autoimmune encephalitis, once considered rare, is now being diagnosed more frequently because of the identification of specific neuronal antibodies. As per a recent study, the incidence of autoimmune encephalitis has increased from 0.
Limbic encephalitis is usually a paraneoplastic phenomenon but may also occur without an associated cancer. In fact, the neurologic syndrome may present weeks to months before diagnosis of the primary tumor [ 6 ]. Even though serum and cerebrospinal fluid assays for auto-antibodies are now available in a limited number of laboratories mostly in Europe and United States, the diagnosis will often be delayed if reliance is placed solely on antibody testing.
Early diagnosis and immunotherapy is paramount to achieve neurological improvement and prevent relapses [ 2 ]. Therefore, Graus et al. Diagnosis is usually suspected in patients presenting with subacute onset of short-term memory loss, altered mental status, or psychiatric symptoms. EEG findings are usually nonspecific but may be used to unveil subclinical seizures or nonconvulsive status epilepticus. Anti-Hu antibodies have shown association with small cell lung cancer in younger patients, whereas young patients with anti-Ma2 antibodies should be evaluated for testicular cancer [ 2 ].
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