Journal of Neurology, Neurological Science and Disorders
Department of Medicine, University of the Region of Joinville, Santa Catarina, Joinville, 89202-207, Brazil
Cite this as
Merkle AR, Machado Schlindwein MA, Breis LC, Magno Gonçalves MV (2020) Acute psychosis in elderly: do not forget the CASPR2 spectrum as a possible cause. J Neurol Neurol Sci Disord. 2020; 6(1): 008-009. Available from: 10.17352/jnnsd.000037
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© 2020 Merkle AR, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Antibodies against contactin-associated protein 2 (CASPR2), a protein associated with the Voltage-Gated Potassium Channel Complex (VGKC) [1], represent an emerging cause of some old neurological manifestations; such as Morvan’s and Isaacs’ syndromes, as well as a variety of phenotypes encompassing limbic encephalitis, neuropathic pain, late onset epilepsy and dysautonomia [2,3].
Antibodies against contactin-associated protein 2 (CASPR2), a protein associated with the Voltage-Gated Potassium Channel Complex (VGKC) [1], represent an emerging cause of some old neurological manifestations; such as Morvan’s and Isaacs’ syndromes, as well as a variety of phenotypes encompassing limbic encephalitis, neuropathic pain, late onset epilepsy and dysautonomia [2,3].
Although the prevalence of anti-neuronal surface antibodies and their impact on clinical practice is not well-established [4-7], they represent an important etiology of autoimmune encephalitis and a curable cause of dementia and acute psychosis [8-10].
In a study by Baumgartner, et al. 34% of the patients with autoimmune encephalitis were initially admitted to the psychiatry department. In addition, psychiatry disturbances represent the second most common presentation of autoimmune encephalitis after seizures [11].
When it comes to anti-CASPR2 psychiatric phenotypes, symptoms tend to present in a progressive manner (5-7 months); mostly with no fluctuations, and a tumor is present in 19% of the patients [2,10]. It affects mostly elderly man (Figure 1) [2,12] and clinical presentation ranges from cognitive decline, sleep disorders, depressive mood, global amnesia - especially when associated with anti-Leucine-rich, glioma inactivated 1 (anti-LGI1) antibodies - and psychotic episodes12. Other symptoms, such as seizures, peripheral nerve symptoms and dysautonomia may appear later in the disease, possibly being misdiagnosed as dementia or psychiatric disease [2].
It is important to highlight that most of the patients with anti-CASPR2 have either normal MRI image or hippocampal atrophy, which holds and association with anti-LGI1 antibodies; and therefore the diagnosis may be challenging when facing a case with this suspected etiology [13].
In conclusion, antibodies against CASPR2 should be remembered as a cause of cognitive imbalance or psychosis, especially in elderly men, even with a normal MRI image. Also, if a tumor is not yet known, it is important to make a screening. Finally, anti-CASPR2 disorders respond well to immunotherapy, but may acquire a poor prognosis, especially when treatment is delayed due a misdiagnosis [12].
Performed data acquisition and data analysis as well helping with the writing in the text: André Ricardo Merkle, Letícia Caroline Breis, Marco Antônio Machado Schlindwein.
Made substantial contributions to conception and design of the study, as well as provided technical support: Marcus Vinicius Magno Gonçalves MD PhD.
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