Voltage-gated Potassium Channel Antibody Autoimmune Encephalopathy Presenting With Isolated Psychosis in an Adolescent

2017 ◽  
Vol 23 (6) ◽  
pp. 441-445 ◽  
Author(s):  
NATALIE C. PON ◽  
KIMBERLY M. HOUCK ◽  
EYAL MUSCAL ◽  
SINDHU A. IDICULA
2018 ◽  
Vol 89 (10) ◽  
pp. A18.2-A18
Author(s):  
Joyutpal Das ◽  
Vanisha Chauhan ◽  
Ryan Keh ◽  
Daniel Mills ◽  
Johal Nicholas ◽  
...  

Voltage-gated potassium channel (VGKC) complex antibodies have been associated with a spectrum of presentations including peripheral nerve hyperexcitability (PNH), Morvan’s syndrome, autoimmune encephalopathy, epilepsy and recently psychosis.We retrospectively reviewed the medical records of 70 patients from the Greater Manchester Neuroscience Centre, who had tested positive for VGKC-complex antibodies between 2012 and 2015 to identify the clinical relevance of positive results.The majority were diagnosed with autoimmune encephalopathy(19) followed by epilepsy(14), psychosis(10) and PNH(6). The remaining fifteen had other neurological presentations and six had no primary neurological disorder. 39/70 patients who had antibody titres>400 pM, were diagnosed with autoimmune encephalopathy(19), epilepsy(9), psychosis(4), PNH(3) and other disorders(4). 24/39 patients, who received treatment with one or a combination of corticosteroids, intravenous immunoglobulins, cyclophosphamide, plasma exchange, azathioprine or rituximab, had a diagnosis of autoimmune encephalopathy(18), epilepsy(2), psychosis(2) and malignancy(2). 16/24 were treatment responsive. 3/31 patients with lower titres were also treated, but only one with the classic phenotype (PNH) responded to treatment.The classic phenotype often had a titre >400 pM. PNH may have a titre ≤400 pM. The patients without classic presentations typically had titres≤400 pM. Consistent with previous studies, clinical phenotyping and antibody titre helped to determine the relevance of VGKC-complex antibodies.


Author(s):  
Eoin P. Flanagan ◽  
Richard J. Caselli

The historical term “Hashimoto’s encephalopathy” describes a serologically diverse spectrum of autoimmune encephalopathies that tend to be highly steroid responsive and unassociated with cancer. Patients typically present with subacute cognitive decline and serological evidence of autoimmunity that includes but is not limited to thyroid antibodies. An inflammatory spinal fluid and, in a subset of patients, MRI signal abnormalities are supportive. Patients often respond promptly to corticosteroids but relapses are typical. The continued discovery of novel neural-specific-autoantibodies such as voltage-gated-potassium-channel-autoantibodies associated with autoimmune encephalopathies highlights why the term “Hashimoto’s” is misleading. The authors advocate the more general term “autoimmune encephalopathy.”


2008 ◽  
Vol 39 (01) ◽  
Author(s):  
E Haberlandt ◽  
CG Bien ◽  
A Reiter ◽  
B Simma ◽  
R Crazzolara ◽  
...  

2020 ◽  
Vol 13 (12) ◽  
pp. e233179
Author(s):  
Eric Garrels ◽  
Fawziya Huq ◽  
Gavin McKay

Limbic encephalitis is often reported to present as seizures and impaired cognition with little focus on psychiatric presentations. In this case report, we present a 49-year-old man who initially presented to the Psychiatric Liaison Service with a several month history of confusion with the additional emergence of visual hallucinations and delusions. Due to the inconsistent nature of the symptoms in the context of a major financial stressor, a provisional functional cognitive impairment diagnosis was made. Investigations later revealed a positive titre of voltage-gated potassium channel (VGKC) antibodies, subtype leucine-rich glioma inactivated 1 accounting for his symptoms which dramatically resolved with steroids and immunoglobulins. This case highlighted the need for maintaining broad differential diagnoses in a patient presenting with unusual psychiatric symptoms.


2014 ◽  
Vol 106 (2) ◽  
pp. 15a-16a
Author(s):  
Elise Faure ◽  
Christine Thompson ◽  
Rikard Blunck

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