Uncommon Epiloptogenic Lesions Affecting the Temporal Lobe (original) (raw)

Limbic encephalitis – a report of four cases

Central European Journal of Immunology, 2017

Usually limbic encephalitis (LE) is a paraneoplastic neurologic syndrome. LE symptoms can precede cancer even by a few years. Almost 50% of LE cases are connected with small cell lung carcinoma. Testis and breast cancers, granulomatous disease, thymoma, and teratomas are also often connected with LE. Other cases have infectious and autoimmunological aetiology. In LE limbic system dysfunction is observed, and it is accompanied by cerebellum and brain stem abnormalities as well as polyneuropathy. Paraneoplastic limbic encephalitis is sometimes a part of larger syndrome in which brain stem and spinal cord are involved in an inflammatory process called paraneoplastic encephalomyelitis. The main LE symptoms are: impairment of cognitive functions with subacute beginning, partial and generalised seizures, mental distress, disturbances of consciousness, and limb paresis. In MRI study hyperintensive lesions in the medial part of the temporal lobes in T2 and FLAIR sequences are present. Sharp and slow waves in electroencephalography in the temporal area are also frequent. In cerebrospinal fluid pleocytosis, elevation of protein level, intensification of immunoglobulin synthesis, and oligoclonal bands can be detected. The majority of patients with paraneoplastic LE have onconeural antibodies in the blood. The presented study is a description of the clinical course of the disease in four patients diagnosed with LE.

Excellent outcome after prolonged status epilepticus due to non-paraneoplastic limbic encephalitis

Acta neurologica Belgica, 2008

Limbic encephalitis (LE) is frequently associated with malignancy. Non-paraneoplastic LE is less common and in this form, voltage-gated potassium channel (VGKC) antibodies are usually found. However in 2007 the spectrum was further extended by a report on four patients with presumed non-paraneoplastic LE in whom neither VGKC-antibodies nor other antibodies could be found (Samarasekera et al. 2007). Despite immunmodulatory treatment all these patients had severe neurological residual symptoms. Here we describe a further patient in whom extensive diagnostic procedures suggested non-paraneoplastic antibody-negative limbic encephalitis. Although this woman had prolonged status epilepticus during seven weeks, her outcome was excellent.

Pitfalls in diagnosing limbic encephalitis - a case report

Acta Neurologica Scandinavica, 2008

The syndrome of limbic encephalitis (LE) is characterized by subacute onset of temporal lobe epilepsy, loss of short-term memory, cognitive confusion and psychiatric symptoms. We report a patient with pharmacoresistant epilepsy who underwent presurgical video-electroencephalogram (EEG)-monitoring with normal psychiatric and neuropsychological findings. Magnetic resonance imaging (MRI) revealed a hyperintense lesion within the right amygdala but no contrast enhancement. Analysis of cerebrospinal fluid (CSF) showed pleocytosis and positive oligoclonal bands, but all tests for neurotropic viruses or borrelia antibodies were negative. Presurgical evaluation identified a right mesiotemporal focus. As a tumour was the most likely differential diagnosis, we performed selective amygdalohippocampectomy of the right hemisphere. Subsequent histopathological examination revealed the surprising diagnosis of LE. As a consequence, tumour screening was initiated and a testicular carcinoma with high anti-Ma2-antibody titres was detected. Following surgical and chemotherapeutical treatment, the patient was seizure-free and Ma2-antibodies decreased below detection limits. Conclusion - This case report highlights that LE has to be considered even in patients with atypical clinical presentation, i.e. without neuropsychological deficits, if CSF analysis reveals an inflammatory response. When LE is diagnosed, extensive tumour search is mandatory to detect and treat the paraneoplastic origin of LE. Therapeutic strategies of LE include surgical treatment as well as early immunosuppression.

Clinical variants of limbic encephalitis

SAGE Open Medical Case Reports

The clinical picture of immunomediator disorders of the central nervous system resulting from autoimmune or paraneoplastic processes is often represented by the limbic symptom complex or limbic encephalitis. The article gives a brief description of these conditions, allocated to a separate nosological group in 2007. The symptoms of limbic encephalitis include mental disorders and epileptic seizures of both convulsive and non-convulsive spectrum, up to epileptic status. Four clinical cases representative of different variants of limbic encephalitis are presented in this study, along with the discussion of epidemiology, differential diagnostics, and generally accepted patient management strategies. The diagnosis of limbic encephalitis was made on clinical grounds alone in three cases and on the presence of antibodies to N-Methyl-d-aspartic acid receptors in one case. A combination of glucocorticoid pulse therapy with prolonged use of valproic acid was successfully applied for the trea...

A Boy with Non-Herpes Simplex Acute Limbic Encephalitis and Antiglutamate Receptor Antibodies

Clinical Medicine Insights: Case Reports, 2011

This report concerns a 12-year-old male with intractable seizures over a long period. The case fulfilled the diagnostic criteria for nonherpetic acute limbic encephalitis. He had frequent convulsions starting with a partial seizure at the left angle of the mouth and progressing to secondary generalized seizures. He was treated with several anticonvulsants, combined with methylprednisolone and γ-globulin under mechanical ventilation. However, his convulsions reappeared after tapering of the barbiturate. His magnetic resonance imaging showed a high intensity area in the hippocampus by FLAIR and diffusion. After five months he recovered without serious sequelae. Virological studies, including for herpes simplex virus, were all negative. He was transiently positive for antiglutamate receptor antibodies in cerebrospinal fluid and serum.

Pilomotor seizures: An autonomic semiology of limbic encephalitis?

Seizure, 2014

Ictal piloerection (''goose bumps'') is an infrequent form of autonomic seizures and is commonly overlooked as an ictal epileptic manifestation. Piloerection is principally considered to be an expression of ictal temporal lobe activity 1 although other seizure origins such as frontal or hypothalamic have been reported. 2 The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas, and cryptogenic epilepsies. 1 Limbic encephalitis (LE) is an autoimmune disorder clinically characterized by the presence of impaired memory, behavioral/ psychiatric abnormalities, and epileptic seizures. LE is often associated with antibodies against intracellular/nuclear antigens (mainly paraneoplastic), being small-cell lung cancer (SCLC) the most commonly associated. LE associated with antibodies against surface antigens (mainly non-paraneoplastic) is less frequent and presents a better prognosis of recovery after immunotherapy. We report here a series of five patients who debuted with pilomotor seizures of autoimmune origin that led to the diagnosis of limbic encephalitis.