LETTER TO THE EDITOR
Acute demyelinating encephalomyelitis presenting with psychiatric symptoms Acute demyelinating encephalomyelitis presenting with psychiatric symptoms Acute demyelinating encephalomyelitis (ADEM) is a rare disease of the white matter, and sometimes the grey matter, of the central nervous system. Most cases have onset in childhood, but adult onset cases have been reported (Parrish and Yeh, 2012). ADEM usually, but not always, follows a viral, bacterial or parasitic infection. The typical initial presentation consists of confusion, cognitive impairment, and multifocal neurological deficits. Psychiatric symptoms can be part of the initial presentation even prior the development of neurological signs obscuring the ADEM diagnosis especially in psychiatric patients (Patel and Friedman, 1997). The psychiatric manifestations of acute demyelinating encephalitis have been described in case reports (Patel and Friedman, 1997). The initial presentation consists of mood lability, lethargy, disorientation, and confusion followed by delusions, hallucinations, agitation, disturbed sleep, personality changes, and disorganized behavior. Symptoms usually start acutely and may or may not be accompanied by neurologic findings. Psychiatric symptoms and cognitive impairment may persist after the resolution of neurologic signs in some cases (Patel and Friedman, 1997). We recently treated a 60-year-old woman with long history of bipolar disorder who developed ADEM with symptoms resembling her usual manic episodes but accompanied by delirium. She had responded poorly to lithium and risperidone. She had no focal neurological signs, but a brain MRI revealed a demyelinating process with large (≥1 cm) multifocal hyperintense lesions, most pronounced within the frontal and temporal lobes, right greater than left. Her delirium subsided after treatment with intravenous steroids. Diagnosing acute demyelinating encephalitis in psychiatric patients is challenging. First, it can present with only psychiatric symptoms. Habek et al. reported five patients who presented with psychosis, depression and anxiety, an unremarkable neurological examination, and an MRI with classical ADEM findings (Habek et al., 2006). Second, it can mimic a relapse of psychiatric illness. Cupina et al reported a case of a 31-year-old patient with bipolar disorder who was initially diagnosed as a manic relapse (Cupina and Short, 2012). This patient had some atypical features, including changing Copyright # 2013 John Wiley & Sons, Ltd.
presentation, poor response to antimanic agents, and high sensitivity to drug side effects. The location of neuroanatomic involvement may determine the presenting psychiatric symptoms. Habek et al. suggested that the mechanism of depression in his cases was related to cortical–subcortical disconnection compromising the frontolimbic networks (Habek et al., 2006). Consistent with literature on bipolar disorder, right frontotemporal involvement in our patient may have contributed to mania (Starkstein et al., 1990). Clinicians should be aware of the possibility of ADEM in patients with atypical psychiatric symptoms, symptoms of delirium, and atypical neurological findings, including er adults and with lifelong psychiatric disorders. These patients are often admitted to psychiatric services and are misdiagnosed as having a relapse of their psychiatric illness. Demyelinating lesions on brain MRI may assist the diagnosis. Early identification of ADEM and treatment with psychotropic agents along with steroids, intravenous immune globulin, or in some cases plasmapheresis may improve the prognosis. Conflict of interest None declared. References Cupina D, Short B. 2012. A case of acute disseminated encephalomyelitis disguised as mania in a woman with known bipolar disorder. J Neuropsychiatry Clin Neurosci 24(2): E18. doi: 10.1176/appi.neuropsych.11060118. Habek M, Brinar M, Brinar VV, Poser CM. 2006. Psychiatric manifestations of multiple sclerosis and acute disseminated encephalomyelitis. Clin Neurol Neurosurg 108(3): 2904. Parrish JB, Yeh EA. 2012. Acute disseminated encephalomyelitis. Adv Exp Med Biol 724: 1–14. doi: 10.1007/978-1-4614-0653-2_1. Patel SP, Friedman RS. 1997. Neuropsychiatric features of acute disseminated encephalomyelitis: a review. J Neuropsychiatry Clin Neurosci 9(4): 534–40. Starkstein SE, Mayberg HS, Berthier ML, et al.. 1990. Mania after brain injury: neuroradiological and metabolic findings. Ann Neurol 27(6): 652–9.
NAHLA MAHGOUB*, OMOPARIOLA ADEGBOLA AND GEORGE S. ALEXOPOULOS Weill Medical College of Cornell University/New York Presbyterian Hospital, White Plains, NY, USA *E-mail: [email protected] Published online in Wiley Online Library (wileyonlinelibrary.com) DOI: 10.1002/gps.3978 Int J Geriatr Psychiatry 2013; 28: 1318
Acute demyelinating encephalomyelitis presenting with psychiatric symptoms Acute demyelinating encephalomyelitis presenting with psychiatric symptoms Acute demyelinating encephalomyelitis (ADEM) is a rare disease of the white matter, and sometimes the grey matter, of the central nervous system. Most cases have onset in childhood, but adult onset cases have been reported (Parrish and Yeh, 2012). ADEM usually, but not always, follows a viral, bacterial or parasitic infection. The typical initial presentation consists of confusion, cognitive impairment, and multifocal neurological deficits. Psychiatric symptoms can be part of the initial presentation even prior the development of neurological signs obscuring the ADEM diagnosis especially in psychiatric patients (Patel and Friedman, 1997). The psychiatric manifestations of acute demyelinating encephalitis have been described in case reports (Patel and Friedman, 1997). The initial presentation consists of mood lability, lethargy, disorientation, and confusion followed by delusions, hallucinations, agitation, disturbed sleep, personality changes, and disorganized behavior. Symptoms usually start acutely and may or may not be accompanied by neurologic findings. Psychiatric symptoms and cognitive impairment may persist after the resolution of neurologic signs in some cases (Patel and Friedman, 1997). We recently treated a 60-year-old woman with long history of bipolar disorder who developed ADEM with symptoms resembling her usual manic episodes but accompanied by delirium. She had responded poorly to lithium and risperidone. She had no focal neurological signs, but a brain MRI revealed a demyelinating process with large (≥1 cm) multifocal hyperintense lesions, most pronounced within the frontal and temporal lobes, right greater than left. Her delirium subsided after treatment with intravenous steroids. Diagnosing acute demyelinating encephalitis in psychiatric patients is challenging. First, it can present with only psychiatric symptoms. Habek et al. reported five patients who presented with psychosis, depression and anxiety, an unremarkable neurological examination, and an MRI with classical ADEM findings (Habek et al., 2006). Second, it can mimic a relapse of psychiatric illness. Cupina et al reported a case of a 31-year-old patient with bipolar disorder who was initially diagnosed as a manic relapse (Cupina and Short, 2012). This patient had some atypical features, including changing Copyright # 2013 John Wiley & Sons, Ltd.
presentation, poor response to antimanic agents, and high sensitivity to drug side effects. The location of neuroanatomic involvement may determine the presenting psychiatric symptoms. Habek et al. suggested that the mechanism of depression in his cases was related to cortical–subcortical disconnection compromising the frontolimbic networks (Habek et al., 2006). Consistent with literature on bipolar disorder, right frontotemporal involvement in our patient may have contributed to mania (Starkstein et al., 1990). Clinicians should be aware of the possibility of ADEM in patients with atypical psychiatric symptoms, symptoms of delirium, and atypical neurological findings, including er adults and with lifelong psychiatric disorders. These patients are often admitted to psychiatric services and are misdiagnosed as having a relapse of their psychiatric illness. Demyelinating lesions on brain MRI may assist the diagnosis. Early identification of ADEM and treatment with psychotropic agents along with steroids, intravenous immune globulin, or in some cases plasmapheresis may improve the prognosis. Conflict of interest None declared. References Cupina D, Short B. 2012. A case of acute disseminated encephalomyelitis disguised as mania in a woman with known bipolar disorder. J Neuropsychiatry Clin Neurosci 24(2): E18. doi: 10.1176/appi.neuropsych.11060118. Habek M, Brinar M, Brinar VV, Poser CM. 2006. Psychiatric manifestations of multiple sclerosis and acute disseminated encephalomyelitis. Clin Neurol Neurosurg 108(3): 2904. Parrish JB, Yeh EA. 2012. Acute disseminated encephalomyelitis. Adv Exp Med Biol 724: 1–14. doi: 10.1007/978-1-4614-0653-2_1. Patel SP, Friedman RS. 1997. Neuropsychiatric features of acute disseminated encephalomyelitis: a review. J Neuropsychiatry Clin Neurosci 9(4): 534–40. Starkstein SE, Mayberg HS, Berthier ML, et al.. 1990. Mania after brain injury: neuroradiological and metabolic findings. Ann Neurol 27(6): 652–9.
NAHLA MAHGOUB*, OMOPARIOLA ADEGBOLA AND GEORGE S. ALEXOPOULOS Weill Medical College of Cornell University/New York Presbyterian Hospital, White Plains, NY, USA *E-mail: [email protected] Published online in Wiley Online Library (wileyonlinelibrary.com) DOI: 10.1002/gps.3978 Int J Geriatr Psychiatry 2013; 28: 1318
