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Hyponatremia causing seizure is a common cause for admission in the critical care unit. Here, we describe a peculiar case of seizure due to hyponatremia, associated with anti-voltage-gated potassium channel antibody syndrome. This case emphasizes that how a proper workup can unveil unusual but potentially treatable causes of hyponatremia. The hallmark of this syndrome is that neurological symptoms may relapse or progress if the disorder is not recognized in time. This case report emphasizes the point that how a keen observation may decode subtle signs of the grave but potentially treatable pathologies.

Temporal lobe epilepsy is considered to be the most frequent of all epileptic syndromes. Recently, several retrospective studies suggest that limbic encephalitis (LE) may be a cause for adult onset unexplained seizure disorders in patients. This report describes two cases of adult onset epilepsy with voltage-gated potassium channel antibodies (VGKC-abs)-associated LE that responded well to levetiracetam (LEV). As demonstrated by these two cases and reviewing previous reports, we propose that the therapeutic regimen for VGKC-abs associated seizures still needs to be determined and LEV may be effective in treating this kind of disorders.

Objective: To illustrate the neuropsychiatric and imaging findings in a confirmed case of voltage-gated potassium channel antibody limbic encephalitis. Method: Case report and review of the literature. Results: A 64-year-old man presented with several months’ history of obsessive thoughts and compulsions associated with faciobrachial dystonic seizures. He had no significant past medical and psychiatric history. Physical examinations revealed only mildly increased tone in the left upper limb. Bedside cognitive testing was normal. Positron-emission tomography showed intense symmetrical uptake in the corpus striatum. No underlying malignancy was identified on whole body imaging. Magnetic resonance imaging, lumbar puncture and electroencephalogram were normal. Serum voltage-gated potassium channel antibodies were strongly positive. The patient had a favourable response to antiepileptic drugs, oral steroids and immunotherapy. Conclusions: Voltage-gated potassium channel limbic encephalitis characteristically presents with neuropsychiatric symptoms and temporal lobe seizures. Positron-emission tomography–computed tomography can be a useful adjunct to the clinical and biochemical work-up.

We are emphasising the importance of considering a rare diagnosis, voltage-gated Potassium channel antibody-associated limbic encephalitis, in an 80-year-old gentleman who presented with memory impairment, seizure and hyponatraemia. He was found to have high titre of voltage-gated potassium channel antibodies in his serum. He was given high-dose steroids and he responded biochemically and clinically with marked improvement in symptomatology.

Voltage-gated potassium channels (VGKCs) are a family of membrane proteins responsible for controlling cell membrane potential. The presence of antibodies (Ab) against neuronal VGKC complexes aids in the diagnosis of idiopathic and paraneoplastic autoimmune neurologic disorders. The diagnosis of VGKC Ab-associated encephalopathy (VCKC Ab syndrome) should be suspected in patients with subacute onset of disorientation, confusion, and memory loss in the presence of seizures or a movement disorder. VGKC Ab syndrome may present with sleep-related symptoms, and the purpose of this communication is to alert sleep and neurology clinicians of this still-under-recognized condition. In this case, we are presenting the VGKC Ab syndrome which improved after treatment with solumedrol. The prompt recognition and treatment of this condition may prevent the morbidity associated with cerebral atrophy and the mortality associated with intractable seizures and electrolyte disturbances.

Voltage-gated potassium channel antibody-associated limbic encephalitis (VGKC-LE) is a rare disease that is a diagnostic and therapeutic challenge for medical practitioners. Two patients with VGKC-LE, both developing dementia are presented. Following treatment, both patients showed remarkable cognitive and functional improvement enabling them to leave the psychogeriatric nursing homes they both were admitted to. Patients with VGKC-LE can have a major cognitive and functional improvement even after a diagnostic delay of more than 1 year. Medical practitioners who treat patients with unexplained cognitive decline, epileptic seizures, or psychiatric symptoms should be aware of LE as an underlying rare cause.

A 65-year-old woman presenting with an acute onset ofconsciousness disturbance was referred to our emergencydepartment. During the examination, she was comatose andhad clonic seizures. Her brain MRI showed symmetric ab-normal signal intensities localized in the bilateral medialtemporal lobes and claustrum (Picture). The serum anti-voltage-gated potassium channel (VGKC) antibody was ele-vated (1,207 pM; normal range

Limbic encephalitis may occur as an infectious, paraneoplastic, or autoimmune phenomenon. One such cause of limbic encephalitis is voltage gated potassium channel antibodies (VKGC). Hyponatremia with new cognitive decline may be one of the presenting symptoms. The exact mechanism of hyponatremia is unknown though findings consistent with syndrome of inappropriate antidiuretic hormone (SIADH) are observed. We retrospectively reviewed all cases admitted to an academic medical center with a diagnosis of limbic encephalitis (848 adults between 2004 to 2010) and found six cases of VGKC antibody associated limbic encephalitis. Three of the six cases had SIADH that completely or partially resolved with a combination of water restriction and immunotherapy. The reversibility of hyponatremia and limbic encephalitis with immunomodulation suggests an antibody-mediated cause. We further review available literature for association of hyponatremia and VGKC limbic encephalitis and propose mechanisms of for the hyponatremia in autoimmune encephalitis

Recently, limbic encephalitis (LE) associated with Voltage-gated potassium channel antibody (VGKC-Ab) has been postulated as a new autoimmune disorder. Most previously reported cases of VGKC-Ab-associated LE were non-paraneoplastic, and reports of a paraneoplastic type are rare. Here we describe a 59-year-old woman with paraneoplastic VGKC-Ab-associated LE preceding the recurrence of invasive thymoma. There was a close temporal relationship between the clinical course and the changes of the VGKC-Ab titer. Unlike many of the non-paraneoplastic VGKC-Ab-associated LE cases, our cases showed the more extensive high intensity lesions on MRI and the absence of seizure and hyponatremia.

WCN 2013 No: 1540 Topic: 5 — Dementia Voltage-gated potassium channel antibody encephalopathy mimicking Creutzfeldt–Jakob disease T. Santos, G. Oliveira, A.S. Countinho, P. Barros, H. Felgueiras, T. Soares-Silva, P. Carneiro. Neurology, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal; Neuroradiology, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal; Psychiatry, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de

LE defines the subacute onset of amnesia and seizures. However, there are few reliable clinical features distinguishing VGKC-antibody associated LE from the many other forms of LE. Identification of such features may aid early diagnosis and immunotherapy administration. We describe 26 patients with median age at onset of 64.5 years (36–73) who developed characteristic, brief (few seconds), frequent (mean of 65 per day) dystonic seizures involving the arm (in 100%) and face (in 73%). All patients had high VGKC-antibody levels (mean 2403 pM, 238–6129; normal values