General Hospital Psychiatry 37 (2015) 274.e1–274.e2
Contents lists available at ScienceDirect
General Hospital Psychiatry journal homepage: http://www.ghpjournal.com
Case Report
Neuromyelitis optica presenting with psychiatric symptoms and catatonia: a case report☆,☆☆,★ Abdulkader Alam, M.D. a,⁎, Rachit Patel, M.D. b, Briana Locicero, B.A. c, Nicole Rivera, B.S. c a b c
Psychiatry and Internal Medicine, Stony Brook Medicine Emergency Psychiatry Program, 101 Nichols Rd., Stony Brook, NY 11794 United States Stamford Hospital, Affiliate of the Columbia University College of Physicians and Surgeons Stony Brook School of Medicine, 101 Nichols Rd, Stony Brook, NY 11794 United States
a r t i c l e
i n f o
Article history: Received 2 December 2014 Revised 19 February 2015 Accepted 19 February 2015 Keywords: Neuromyelitis optica Psychosis Catatonia Benzodiazepine
a b s t r a c t Neuromyelitis optica (NMO) is an aggressive disease characteristically affecting the spinal cord and optic nerves that has recently been differentiated from multiple sclerosis. We present a case of a 16-year-old Antiguan female previously diagnosed with NMO who presented with a 1-week history of confusion and agitation. She had symptoms of psychosis, including delusional thinking and auditory and visual hallucinations, and scored 11/23 on the Bush–Francis Catatonia Scale. This case demonstrates an NMO exacerbation that presented with psychotic symptoms and catatonia. © 2015 Elsevier Inc. All rights reserved.
1. Introduction Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder that characteristically affects the spinal cord and optic nerves, causing clinical manifestations of bilateral optic neuritis and longitudinally extensive transverse myelitis [1,2]. The clinical presentation plus the presence of NMO-immunoglobulin G — targeting central nervous system aquaporin-4 receptors — is diagnostic of NMO [3,4]. Catatonia is a behavioral syndrome that was first delineated in 1874 [5]. The syndrome can occur in patients with underlying psychiatric and general medical disorders [5,6]. It is marked by heterogeneous signs, which are observed or elicited, the most common being immobility, rigidity, mutism, posturing, excessive motor activity, stupor, negativism, staring and echolalia [5,6]. This is a case of a 16-year-old female with a history of NMO and no previous psychiatric history presenting with prominent psychosis and catatonia. 2. Case report The patient was a 16-year-old Antiguan female with a medical history significant for epilepsy diagnosed at age 10 and NMO diagnosed at age 16. Patient had no personal or familial psychiatric history. ☆ ☆☆ ★ ⁎
Financial support: none. Previous presentation: APA Annual Meeting in New York City, May 2014. Conflict of interest: none. Corresponding author. Tel.: +1 631 444 2919. E-mail addresses: [email protected] (A. Alam), [email protected] (R. Patel), [email protected] (B. Locicero), [email protected] (N. Rivera). http://dx.doi.org/10.1016/j.genhosppsych.2015.02.007 0163-8343/© 2015 Elsevier Inc. All rights reserved.
She was on carbamazepine 400 mg twice daily for her well-controlled seizures and had never been on any psychotropic medications. She was described by her family and pediatrician as a well-adjusted, respectful, slightly shy but friendly adolescent who performed well in school and was otherwise unremarkable behaviorally. No substance or alcohol use history was reported. Upon presentation to the emergency department, her parents reported a 1-week history of symptoms including confusion, incoherent speech, hallucinations, left-sided numbness and urinary incontinence. Examination revealed a thin, young female neatly attired in hospital pajamas and sitting upright. She was not in distress. She had a flat affect and poor eye contact, and it was difficult to establish rapport. She was alert, fully awake and oriented to self and place. Some cognitive impairments were noted [Montreal Cognitive Assessment (MoCA) score, 17/ 30]. She had minor hand rigidity; visual hallucinations; nihilistic delusions; a feeling her body was dead; and slowed, halting speech. Her vital signs were within normal limits. On the Bush–Francis Catatonia Scale, she had positive score in 11 of 23 domains consistent with a diagnosis of catatonia: excitement, immobility, mutism, staring, posturing, waxy flexibility, echolalia, verbigeration, withdrawal, perseveration and combativeness [6]. She also met the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, criteria for catatonia with 6 of 12 symptoms present. A diagnosis of psychosis not otherwise specified and catatonia was made, and she was admitted to the pediatric unit. Extensive testing was performed to rule out other infectious, oncologic and genetic etiologies, all of which were negative. Chest X-ray (CXR), electroencephalography, Purified Protein Derivative test (PPD) and computed tomography of the chest, abdomen and pelvis were all
274.e2
A. Alam et al. / General Hospital Psychiatry 37 (2015) 274.e1–274.e2
normal. Evoked potentials showed normal patterned visual evoked responses for each eye. New York State (NYS) encephalitis, West Nile and Arbovirus panels showed no evidence of infection. Cerebrospinal fluid (CSF) studies and basic labs were within normal limits, and paraneoplastic studies were negative. She had a low positive titer of anti-NMDA receptor antibody level at 1:20 (normal b 1:10). The presence of oligoclonal bands in the CSF and serum aquaporin-4 antibody level N 160 U/ml (normal b 1.6 U/ml) were consistent with a diagnosis of NMO. Magnetic resonance imaging (MRI) of the brain showed a large lesion within the right posterior frontal/anterior parietal/anterior temporal lobe likely to be an atypical demyelinating lesion associated with NMO (Fig. 1). During hospitalization, neurological illness was treated with azathioprine, steroids, intravenous immunoglobulin, plasmapheresis and rituximab. Catatonia was treated with lorazepam at 1.5 mg/day. After she received three doses, she was no longer posturing and was more responsive. However, the lorazepam was not increased as planned as she had marked psychomotor retardation and appeared more confused and disorganized in her thought process. Risperidone was then added at 0.25 mg PO BID to help address these symptoms and psychosis. The risperidone was increased to 2 mg/day as she slowly showed improvement in functioning, such as being able to get out of bed and use the bathroom independently, exhibiting less psychomotor retardation and disorganization, and more spontaneous speech. She was discharged after 5 weeks of hospitalization. On the final psychiatric evaluation, the patient felt that she was “partially alive” and was fully oriented with memory, language and perceptual functioning appearing intact. However, she continued to display features of mild impairment including difficulty with complex attention and visuospatial orientation (MoCA score, 25/30).
NMDA receptor encephalitis was considered owing to a positive serum titer of anti-NMDA, but the significance was unclear in light of her negative CSF paraneoplastic studies and a relatively lower serum titer. Catatonia is generally treated with benzodiazepines as the first line of treatment, and high doses are often required [7]. There is less clarity on the role played by antipsychotic medications in patients with catatonia and especially in treating patients presenting with both psychosis and catatonia [8]. While most patients have a robust response to lorazepam, some cannot tolerate higher doses or the effects are not sustained [8]. Titration to a higher dose of benzodiazepine in our patient was avoided because although her catatonia improved, it was felt that she became more psychotic. Her psychosis improved with the addition of antipsychotic medication; however, recovery was slow. This case illustrates a potential relationship between NMO exacerbation and the onset of psychiatric symptoms and catatonia. Management was difficult; small doses of lorazepam and risperidone appeared to have helped, and close consideration was given to dosing so that each aspect of the illness could be treated without adversely affecting the other. Further studies are needed regarding the association between psychiatric symptoms, catatonia and NMO as well as the treatment approach. 4. Conclusion Our case confirms one prior report that NMO can present with psychotic symptoms [1]. This is the first case that describes NMO presenting with acute psychosis and catatonia. Although the patient received treatment for catatonia and psychosis, improvement was protracted, suggesting that the treatment of catatonia in the setting of a neurological illness such as NMO can be challenging.
3. Discussion References NMO exacerbation was believed to be responsible for the patient’s psychiatric symptoms, including psychosis and catatonia. Other possible diagnoses were considered and ultimately felt less likely to be contributing to her presentation. Delirium was possible given the acuity of her symptoms but became less likely given that she was consistently psychotic with no fluctuation in the severity of her cognitive disturbance. Substance- or medication-induced psychosis appeared unlikely in the absence of any substance use history or recent medication change. With the sudden onset of her presentation and no history of psychiatric symptoms, a primary mood or psychotic illness was less likely. Further, she was on carbamazepine, which can be associated with neuroleptic malignant syndrome or serotonin syndrome, but these etiologies were unlikely given that there were no clinical, hemodynamic or laboratory findings to support them. Diagnosis of anti-
[1] Woolley J, Douglas VC, Cree BA. Neuromyelitis optica, psychiatric symptoms and primary polydipsia: a case report. Gen Hosp Psychiatry 2010;32(6):648.e5–8. [2] Graber DJ, Levy M, Kerr D, Wade WF. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation 2008;5(1):22–42. [3] Misu T, Fujihara K, Kakita A, Konno H, Nakamura M, Watanabe S, et al. Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. Brain 2007; 130(5):1224–34. [4] Saini H, Rifkin R, Gorelik M, Huang H, Ferguson Z, Jones MV, et al. Passively transferred human NMO-IgG exacerbates demyelination in mouse experimental autoimmune encephalomyelitis. BMC Neurol 2013;13:1–9. [5] Kahlbaum KL. Die katatonie oder das Spannungsirresein. Berlin: Verlag August Hirschwald; 1874. [6] Bush G, Fink M, Petrides G, Dowling F, Francis A. Catatonia I: rating scale and standardized examination. Acta Psychiatr Scand 1996;93(2):129–36. [7] Fink M, Taylor MA. Catatonia: a clinician’s guide to diagnosis and treatment. Cambridge, NY: Cambridge University Press; 2003. [8] England ML, Ongur D, Konopaske GT, Karmacharya R. Catatonia in psychotic patients: clinical features and treatment response. J Neuropsychiatry Clin Neurosci 2011;23(2):223–6.
Fig. 1. MRI of the brain with axial (left) and coronal (right) Fluid-Attenuated Inversion Recovery (FLAIR) sequences showing a large hyperintense lesion within the right posterior frontal/ anterior parietal lobe white matter which included the subcortical white matter and extended into the right external capsule into the right subinsular region.
Contents lists available at ScienceDirect
General Hospital Psychiatry journal homepage: http://www.ghpjournal.com
Case Report
Neuromyelitis optica presenting with psychiatric symptoms and catatonia: a case report☆,☆☆,★ Abdulkader Alam, M.D. a,⁎, Rachit Patel, M.D. b, Briana Locicero, B.A. c, Nicole Rivera, B.S. c a b c
Psychiatry and Internal Medicine, Stony Brook Medicine Emergency Psychiatry Program, 101 Nichols Rd., Stony Brook, NY 11794 United States Stamford Hospital, Affiliate of the Columbia University College of Physicians and Surgeons Stony Brook School of Medicine, 101 Nichols Rd, Stony Brook, NY 11794 United States
a r t i c l e
i n f o
Article history: Received 2 December 2014 Revised 19 February 2015 Accepted 19 February 2015 Keywords: Neuromyelitis optica Psychosis Catatonia Benzodiazepine
a b s t r a c t Neuromyelitis optica (NMO) is an aggressive disease characteristically affecting the spinal cord and optic nerves that has recently been differentiated from multiple sclerosis. We present a case of a 16-year-old Antiguan female previously diagnosed with NMO who presented with a 1-week history of confusion and agitation. She had symptoms of psychosis, including delusional thinking and auditory and visual hallucinations, and scored 11/23 on the Bush–Francis Catatonia Scale. This case demonstrates an NMO exacerbation that presented with psychotic symptoms and catatonia. © 2015 Elsevier Inc. All rights reserved.
1. Introduction Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder that characteristically affects the spinal cord and optic nerves, causing clinical manifestations of bilateral optic neuritis and longitudinally extensive transverse myelitis [1,2]. The clinical presentation plus the presence of NMO-immunoglobulin G — targeting central nervous system aquaporin-4 receptors — is diagnostic of NMO [3,4]. Catatonia is a behavioral syndrome that was first delineated in 1874 [5]. The syndrome can occur in patients with underlying psychiatric and general medical disorders [5,6]. It is marked by heterogeneous signs, which are observed or elicited, the most common being immobility, rigidity, mutism, posturing, excessive motor activity, stupor, negativism, staring and echolalia [5,6]. This is a case of a 16-year-old female with a history of NMO and no previous psychiatric history presenting with prominent psychosis and catatonia. 2. Case report The patient was a 16-year-old Antiguan female with a medical history significant for epilepsy diagnosed at age 10 and NMO diagnosed at age 16. Patient had no personal or familial psychiatric history. ☆ ☆☆ ★ ⁎
Financial support: none. Previous presentation: APA Annual Meeting in New York City, May 2014. Conflict of interest: none. Corresponding author. Tel.: +1 631 444 2919. E-mail addresses: [email protected] (A. Alam), [email protected] (R. Patel), [email protected] (B. Locicero), [email protected] (N. Rivera). http://dx.doi.org/10.1016/j.genhosppsych.2015.02.007 0163-8343/© 2015 Elsevier Inc. All rights reserved.
She was on carbamazepine 400 mg twice daily for her well-controlled seizures and had never been on any psychotropic medications. She was described by her family and pediatrician as a well-adjusted, respectful, slightly shy but friendly adolescent who performed well in school and was otherwise unremarkable behaviorally. No substance or alcohol use history was reported. Upon presentation to the emergency department, her parents reported a 1-week history of symptoms including confusion, incoherent speech, hallucinations, left-sided numbness and urinary incontinence. Examination revealed a thin, young female neatly attired in hospital pajamas and sitting upright. She was not in distress. She had a flat affect and poor eye contact, and it was difficult to establish rapport. She was alert, fully awake and oriented to self and place. Some cognitive impairments were noted [Montreal Cognitive Assessment (MoCA) score, 17/ 30]. She had minor hand rigidity; visual hallucinations; nihilistic delusions; a feeling her body was dead; and slowed, halting speech. Her vital signs were within normal limits. On the Bush–Francis Catatonia Scale, she had positive score in 11 of 23 domains consistent with a diagnosis of catatonia: excitement, immobility, mutism, staring, posturing, waxy flexibility, echolalia, verbigeration, withdrawal, perseveration and combativeness [6]. She also met the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, criteria for catatonia with 6 of 12 symptoms present. A diagnosis of psychosis not otherwise specified and catatonia was made, and she was admitted to the pediatric unit. Extensive testing was performed to rule out other infectious, oncologic and genetic etiologies, all of which were negative. Chest X-ray (CXR), electroencephalography, Purified Protein Derivative test (PPD) and computed tomography of the chest, abdomen and pelvis were all
274.e2
A. Alam et al. / General Hospital Psychiatry 37 (2015) 274.e1–274.e2
normal. Evoked potentials showed normal patterned visual evoked responses for each eye. New York State (NYS) encephalitis, West Nile and Arbovirus panels showed no evidence of infection. Cerebrospinal fluid (CSF) studies and basic labs were within normal limits, and paraneoplastic studies were negative. She had a low positive titer of anti-NMDA receptor antibody level at 1:20 (normal b 1:10). The presence of oligoclonal bands in the CSF and serum aquaporin-4 antibody level N 160 U/ml (normal b 1.6 U/ml) were consistent with a diagnosis of NMO. Magnetic resonance imaging (MRI) of the brain showed a large lesion within the right posterior frontal/anterior parietal/anterior temporal lobe likely to be an atypical demyelinating lesion associated with NMO (Fig. 1). During hospitalization, neurological illness was treated with azathioprine, steroids, intravenous immunoglobulin, plasmapheresis and rituximab. Catatonia was treated with lorazepam at 1.5 mg/day. After she received three doses, she was no longer posturing and was more responsive. However, the lorazepam was not increased as planned as she had marked psychomotor retardation and appeared more confused and disorganized in her thought process. Risperidone was then added at 0.25 mg PO BID to help address these symptoms and psychosis. The risperidone was increased to 2 mg/day as she slowly showed improvement in functioning, such as being able to get out of bed and use the bathroom independently, exhibiting less psychomotor retardation and disorganization, and more spontaneous speech. She was discharged after 5 weeks of hospitalization. On the final psychiatric evaluation, the patient felt that she was “partially alive” and was fully oriented with memory, language and perceptual functioning appearing intact. However, she continued to display features of mild impairment including difficulty with complex attention and visuospatial orientation (MoCA score, 25/30).
NMDA receptor encephalitis was considered owing to a positive serum titer of anti-NMDA, but the significance was unclear in light of her negative CSF paraneoplastic studies and a relatively lower serum titer. Catatonia is generally treated with benzodiazepines as the first line of treatment, and high doses are often required [7]. There is less clarity on the role played by antipsychotic medications in patients with catatonia and especially in treating patients presenting with both psychosis and catatonia [8]. While most patients have a robust response to lorazepam, some cannot tolerate higher doses or the effects are not sustained [8]. Titration to a higher dose of benzodiazepine in our patient was avoided because although her catatonia improved, it was felt that she became more psychotic. Her psychosis improved with the addition of antipsychotic medication; however, recovery was slow. This case illustrates a potential relationship between NMO exacerbation and the onset of psychiatric symptoms and catatonia. Management was difficult; small doses of lorazepam and risperidone appeared to have helped, and close consideration was given to dosing so that each aspect of the illness could be treated without adversely affecting the other. Further studies are needed regarding the association between psychiatric symptoms, catatonia and NMO as well as the treatment approach. 4. Conclusion Our case confirms one prior report that NMO can present with psychotic symptoms [1]. This is the first case that describes NMO presenting with acute psychosis and catatonia. Although the patient received treatment for catatonia and psychosis, improvement was protracted, suggesting that the treatment of catatonia in the setting of a neurological illness such as NMO can be challenging.
3. Discussion References NMO exacerbation was believed to be responsible for the patient’s psychiatric symptoms, including psychosis and catatonia. Other possible diagnoses were considered and ultimately felt less likely to be contributing to her presentation. Delirium was possible given the acuity of her symptoms but became less likely given that she was consistently psychotic with no fluctuation in the severity of her cognitive disturbance. Substance- or medication-induced psychosis appeared unlikely in the absence of any substance use history or recent medication change. With the sudden onset of her presentation and no history of psychiatric symptoms, a primary mood or psychotic illness was less likely. Further, she was on carbamazepine, which can be associated with neuroleptic malignant syndrome or serotonin syndrome, but these etiologies were unlikely given that there were no clinical, hemodynamic or laboratory findings to support them. Diagnosis of anti-
[1] Woolley J, Douglas VC, Cree BA. Neuromyelitis optica, psychiatric symptoms and primary polydipsia: a case report. Gen Hosp Psychiatry 2010;32(6):648.e5–8. [2] Graber DJ, Levy M, Kerr D, Wade WF. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation 2008;5(1):22–42. [3] Misu T, Fujihara K, Kakita A, Konno H, Nakamura M, Watanabe S, et al. Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis. Brain 2007; 130(5):1224–34. [4] Saini H, Rifkin R, Gorelik M, Huang H, Ferguson Z, Jones MV, et al. Passively transferred human NMO-IgG exacerbates demyelination in mouse experimental autoimmune encephalomyelitis. BMC Neurol 2013;13:1–9. [5] Kahlbaum KL. Die katatonie oder das Spannungsirresein. Berlin: Verlag August Hirschwald; 1874. [6] Bush G, Fink M, Petrides G, Dowling F, Francis A. Catatonia I: rating scale and standardized examination. Acta Psychiatr Scand 1996;93(2):129–36. [7] Fink M, Taylor MA. Catatonia: a clinician’s guide to diagnosis and treatment. Cambridge, NY: Cambridge University Press; 2003. [8] England ML, Ongur D, Konopaske GT, Karmacharya R. Catatonia in psychotic patients: clinical features and treatment response. J Neuropsychiatry Clin Neurosci 2011;23(2):223–6.
Fig. 1. MRI of the brain with axial (left) and coronal (right) Fluid-Attenuated Inversion Recovery (FLAIR) sequences showing a large hyperintense lesion within the right posterior frontal/ anterior parietal lobe white matter which included the subcortical white matter and extended into the right external capsule into the right subinsular region.
