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Arnold–Chiari I malformation association with generalized anxiety disorder: A case report
Progress in Neuro-Psychopharmacology & Biological Psychiatry 32 (2008) 1613–1614
Contents lists available at ScienceDirect
Progress in Neuro-Psychopharmacology & Biological Psychiatry j o u r n a l h o m e p a g e : w w w. e l s ev i e r. c o m / l o c a t e / p n p b p
Letter to the Editor (Case report)
Arnold–Chiari I malformation association with generalized anxiety disorder: A case report 1. Introduction Arnold–Chiari malformation is a disorder of embryologic development that is characterized by four types. Arnold–Chiari I malformation (ACM-I) consists of herniation of the cerebellar tonsils into the foramen magnum. Arnold–Chiari II malformation is almost exclusively associated with myelomeningocele and hydrocephalus. It consists of herniation of not only the tonsils, but also all of the contents of the posterior fossa into the foramen magnum. Arnold–Chiari III and IV malformations are very rare (Strayer, 2001; Nash et al., 2002). Generally, ACM-I patients are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years (Akin et al., 2003). The symptoms and signs include headaches, dizziness, visual or oculomotor symptoms, dysphagia, trunk or extremity dysesthesias, ataxia, and drop attacks (Mäkelä, 2006). To date, two cases have been reported of an anxiety disorder in association with this anomaly (Iwabuchi et al., 1985; Chisholm et al., 1993). 2. Case report Mr. K was a 29-year-old unmarried man admitted to our hospital. Upon examination, he was found to suffer from excessive anxiety, restlessness, difficulty concentrating, fatigue, irritability, impulsivity, muscle tension, and epigastric discomfort. He reported difficulty falling asleep, disturbed sleep, and not feeling rested in the morning. He was also worried about being unproductive at work and socially isolating himself. A diagnosis of generalized anxiety disorder was established according to The International Classification of Mental and Behavioural Disorders, Tenth Edition (World Health Organization, 1993). The first psychiatric symptoms, which arose at the age of 27, were characterized by excessive anxiety, restlessness, fatigue, insomnia, and epigastric discomfort. In the previous 2 years, he had not been hospitalized at any time. Although he was treated sequentially with various antidepressant and anxiolytic drugs during this period, he had only relative clinical improvement. He finally settled on 50 mg of sertraline and took it for 5 months, describing it as moderately helpful. When the patient was admitted to our hospital, he had been off of medication for about 20 days. There was no personal history of substance misuse or previous psychiatric disorders, and no family history of mental illness. In addition, the patient suffered from headache, diplopia, and vertigo. He had a consultation with the Department of Neurology. As proposed by the Department of Neurology, brain magnetic resonance imaging (MRI) was performed and revealed that the cerebellar tonsils 0278-5846/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.pnpbp.2008.05.018
herniated 1 cm into the foramen magnum (Fig. 1). An electroencephalograph (EEG) was inconclusive. After a consultation with the Department of Neurosurgery, a diagnosis of ACM-I was established, and the patient was recommended for outpatient follow-up after discharge. The severity of his anxiety symptoms was assessed by using the Hamilton Anxiety Rating Scale (HARS) (Hamilton, 1959). Initially, he scored a 21 on the HARS. He was treated with fluoxetine 20 mg/day and lorazepam 1 mg/day. Lorazepam treatment decreased to 0.5 mg/ day after 1 week and was later stopped. During the stay, there was a partial remission in excessive anxiety, epigastric discomfort, restlessness, difficulty concentrating, and fatigue and a complete remission of sleep disturbance and impulsivity. The patient was discharged after a 4-week period of hospitalization, with relative improvement on the HARS (the patient’s HARS total score decreased from 21 to 12). Also, we planned follow-up outpatient care with psychiatric and neurosurgical appointments. At outpatient follow-up 3 months after discharge, the neurosurgery department had planned a decompression surgery. However, the patient had not approved surgical intervention. They subsequently proposed an outpatient followup without surgery. His anxiety symptoms also continued despite the relative improvement. 3. Discussion This case report discusses the possibility of a causal relationship between ACM-I and a patient’s anxiety symptoms. To our knowledge, only two cases have been reported of an anxiety disorder in association with this anomaly. In the first case report, Chisholm et al. (1993) described a patient who suffered concurrently from panic disorder with agoraphobia and ACM. In another case report, Iwabuchi et al. (1985) described a woman who had a five-year history of insomnia with choked feelings, palpitations, clumsiness of hands, and anxiety. Also, there are some articles that suggested an etiologic relationship of ACM-I to other psychiatric and neurologic disorders. An ACM-I case has been reported that followed up by psychiatry as dysthymia for 3 years (Caykoylu et al., 2001). Grosso et al. (2001) suggested an association of ACM-I with epilepsy, speech delay, and mental retardation. Additionally, a very interesting clinical case of recurrent psychotic events caused by ACM-I in adults has been reported (Ilanković et al., 2006). The co-occurrence of the ACM-I malformation and the psychiatric symptoms in the reported case might be explained in various ways. First, the psychiatric symptoms might be caused by the ACM-I, which can lead to compression of the brainstem. Brainstem serotonergic systems in the dorsal and median raphe nucleus may
1614
Letter to the Editor (Case report)
for both conditions. The present report also, once again, shows the importance of ruling out neurological conditions in atypical psychiatric cases, even when the patients have a long history of disease and partial remission of the symptoms with psychotropic drugs. References
Fig. 1. MRI of patient shows cerebellar tonsil (white arrow) displaced 10 mm below foramen magnum (black arrow).
be part of a distributed neural system that regulates acute and chronic anxiety states (Spiga et al., 2006; Bouwknecht et al., 2007). On the other hand, the locus ceruleus (LC) in the brainstem is a major source of noradrenergic input to the cortex and an important regulator of anxiety (Reinscheid et al., 2005). The second possible explanation is that the malformation had caused some of the symptoms, which then triggered the psychiatric symptoms. Chisholm et al. (1993) found that their patient’s anxiety did not improve after decompression surgery and that the patient had a family history of anxiety disorder. The authors subsequently suggested that symptoms of ACM may have acted as a trigger to the patient’s susceptibility for an anxiety disorder. Although our patient had no family history of an anxiety disorder, it is possible that there are alternative factors that predisposed him to anxiety disorder. It is known that ACM may be a cause of breathing-related sleep disorders (Dauvilliers et al., 2007). Iwabuchi et al. (1985) proposed that their patient’s anxiety was due to the sleep apnea and sleep deprivation caused by ACM. This may be another explanation for our patient’s anxiety symptoms. However, it is difficult to suggest this causality since there was a complete remission of his sleep disturbance after psychotropic treatment of anxiety disorder. 4. Conclusion The reported case emphasizes that a psychiatric disease in patients with a neurological disorder can defer diagnosis and suitable treatment
Akin A, Canakci Z, Sen A, Tore HF. Chiari-I malformation in two fighter pilots. Aviat Space Environ Med 2003;74(7):775–8. Bouwknecht JA, Spiga F, Staub DR, Hale MW, Shekhar A, Lowry CA. Differential effects of exposure to low-light or high-light open-field on anxiety-related behaviors: relationship to c-Fos expression in serotonergic and non-serotonergic neurons in the dorsal raphe nucleus. Brain Res Bull 2007;72(1):32–43. Caykoylu A, Deniz O, Aygül R. A case of Arnold–Chiari I malformation admitted depressive symptoms (in Turkish). MJAU 2001;33:83–5. Chisholm BT, Velamoor R, Chandarana PC, Cochrane DK. Anxiety disorder in a case of Arnold–Chiari malformation. J Psychiatry Neurosci 1993;18(2):67–8. Dauvilliers Y, Stal V, Abril B, Coubes P, Bobin S, Touchon J, et al. Chiari malformation and sleep related breathing disorders. J Neurol Neurosurg Psychiatry 2007;78 (12):1344–8. Grosso S, Scattolini R, Paolo G, Di Bartolo RM, Morgese G, Balestri P. Association of Chiari I malformation, mental retardation, speech delay, and epilepsy: a specific disorder? Neurosurgery 2001;49(5):1099–103. Hamilton M. The assesment of anxiety states by rating. Br J Psychiatry 1959;32:50. Ilanković NN, Ilanković AN, Bojić V, Ilanković LM. Chiari I malformation in adults: epileptiform events and schizophrenia-like psychosis. Psychiatr Danub 2006;18(1-2):92–6. Iwabuchi K, Miyauchi T, Kyuuma Y, Hosaka H, Kunimi Y, Yagishita S. A sudden-death in a case of Arnold–Chiari malformation (type I) with sleep apnea. No To Shinkei 1985;37(6):575–81. Mäkelä JP. Arnold-Chiari malformation type I in military conscripts: symptoms and effects on service fitness. Mil Med 2006;171(2):174–6. Nash J, Cheng JS, Meyer GA, Remler BF. Chiari type I malformation: overview of diagnosis and treatment. WMJ 2002;101(8):35–40. Reinscheid RK, Xu YL, Civelli O. Neuropeptide S: a new player in the modulation of arousal and anxiety. Mol Interv 2005;5(1):42–6. Spiga F, Lightman SL, Shekhar A, Lowry CA. Injections of urocortin 1 into the basolateral amygdala induce anxiety-like behavior and c-Fos expression in brainstem serotonergic neurons. Neuroscience 2006;138(4):1265–76. Strayer, A, (2001). Chiari I malformation: clinical presentation and management. J Neurosci Nurs. 33(2): 90-6, 104. World Health Organization. The ICD-10 Classification of Mental and Behavioural Disorders: Diagnostic Criteria for Research. Geneva: WHO; 1993.
Ali Caykoylu Okan Ekinci⁎ Yakup Albayrak Murat Kuloglu Department of Psychiatry, Atatürk Education and Research Hospital, Ankara, Turkey ⁎Corresponding author. Ankara Ataturk Egitim ve Arastırma Hastanesi Psikiyatri Klinigi, 06520 Bilkent/Ankara, Turkey. Tel.: +90 312 2912525; fax: +90 312 2912705. E-mail address: [email protected] (O. Ekinci). Orhan Deniz Department of Neurology, Atatürk Education and Research Hospital, Ankara, Turkey 11 April 2008
Contents lists available at ScienceDirect
Progress in Neuro-Psychopharmacology & Biological Psychiatry j o u r n a l h o m e p a g e : w w w. e l s ev i e r. c o m / l o c a t e / p n p b p
Letter to the Editor (Case report)
Arnold–Chiari I malformation association with generalized anxiety disorder: A case report 1. Introduction Arnold–Chiari malformation is a disorder of embryologic development that is characterized by four types. Arnold–Chiari I malformation (ACM-I) consists of herniation of the cerebellar tonsils into the foramen magnum. Arnold–Chiari II malformation is almost exclusively associated with myelomeningocele and hydrocephalus. It consists of herniation of not only the tonsils, but also all of the contents of the posterior fossa into the foramen magnum. Arnold–Chiari III and IV malformations are very rare (Strayer, 2001; Nash et al., 2002). Generally, ACM-I patients are symptomatic in the earlier years of life. However, asymptomatic cases can eventually become symptomatic in later years (Akin et al., 2003). The symptoms and signs include headaches, dizziness, visual or oculomotor symptoms, dysphagia, trunk or extremity dysesthesias, ataxia, and drop attacks (Mäkelä, 2006). To date, two cases have been reported of an anxiety disorder in association with this anomaly (Iwabuchi et al., 1985; Chisholm et al., 1993). 2. Case report Mr. K was a 29-year-old unmarried man admitted to our hospital. Upon examination, he was found to suffer from excessive anxiety, restlessness, difficulty concentrating, fatigue, irritability, impulsivity, muscle tension, and epigastric discomfort. He reported difficulty falling asleep, disturbed sleep, and not feeling rested in the morning. He was also worried about being unproductive at work and socially isolating himself. A diagnosis of generalized anxiety disorder was established according to The International Classification of Mental and Behavioural Disorders, Tenth Edition (World Health Organization, 1993). The first psychiatric symptoms, which arose at the age of 27, were characterized by excessive anxiety, restlessness, fatigue, insomnia, and epigastric discomfort. In the previous 2 years, he had not been hospitalized at any time. Although he was treated sequentially with various antidepressant and anxiolytic drugs during this period, he had only relative clinical improvement. He finally settled on 50 mg of sertraline and took it for 5 months, describing it as moderately helpful. When the patient was admitted to our hospital, he had been off of medication for about 20 days. There was no personal history of substance misuse or previous psychiatric disorders, and no family history of mental illness. In addition, the patient suffered from headache, diplopia, and vertigo. He had a consultation with the Department of Neurology. As proposed by the Department of Neurology, brain magnetic resonance imaging (MRI) was performed and revealed that the cerebellar tonsils 0278-5846/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.pnpbp.2008.05.018
herniated 1 cm into the foramen magnum (Fig. 1). An electroencephalograph (EEG) was inconclusive. After a consultation with the Department of Neurosurgery, a diagnosis of ACM-I was established, and the patient was recommended for outpatient follow-up after discharge. The severity of his anxiety symptoms was assessed by using the Hamilton Anxiety Rating Scale (HARS) (Hamilton, 1959). Initially, he scored a 21 on the HARS. He was treated with fluoxetine 20 mg/day and lorazepam 1 mg/day. Lorazepam treatment decreased to 0.5 mg/ day after 1 week and was later stopped. During the stay, there was a partial remission in excessive anxiety, epigastric discomfort, restlessness, difficulty concentrating, and fatigue and a complete remission of sleep disturbance and impulsivity. The patient was discharged after a 4-week period of hospitalization, with relative improvement on the HARS (the patient’s HARS total score decreased from 21 to 12). Also, we planned follow-up outpatient care with psychiatric and neurosurgical appointments. At outpatient follow-up 3 months after discharge, the neurosurgery department had planned a decompression surgery. However, the patient had not approved surgical intervention. They subsequently proposed an outpatient followup without surgery. His anxiety symptoms also continued despite the relative improvement. 3. Discussion This case report discusses the possibility of a causal relationship between ACM-I and a patient’s anxiety symptoms. To our knowledge, only two cases have been reported of an anxiety disorder in association with this anomaly. In the first case report, Chisholm et al. (1993) described a patient who suffered concurrently from panic disorder with agoraphobia and ACM. In another case report, Iwabuchi et al. (1985) described a woman who had a five-year history of insomnia with choked feelings, palpitations, clumsiness of hands, and anxiety. Also, there are some articles that suggested an etiologic relationship of ACM-I to other psychiatric and neurologic disorders. An ACM-I case has been reported that followed up by psychiatry as dysthymia for 3 years (Caykoylu et al., 2001). Grosso et al. (2001) suggested an association of ACM-I with epilepsy, speech delay, and mental retardation. Additionally, a very interesting clinical case of recurrent psychotic events caused by ACM-I in adults has been reported (Ilanković et al., 2006). The co-occurrence of the ACM-I malformation and the psychiatric symptoms in the reported case might be explained in various ways. First, the psychiatric symptoms might be caused by the ACM-I, which can lead to compression of the brainstem. Brainstem serotonergic systems in the dorsal and median raphe nucleus may
1614
Letter to the Editor (Case report)
for both conditions. The present report also, once again, shows the importance of ruling out neurological conditions in atypical psychiatric cases, even when the patients have a long history of disease and partial remission of the symptoms with psychotropic drugs. References
Fig. 1. MRI of patient shows cerebellar tonsil (white arrow) displaced 10 mm below foramen magnum (black arrow).
be part of a distributed neural system that regulates acute and chronic anxiety states (Spiga et al., 2006; Bouwknecht et al., 2007). On the other hand, the locus ceruleus (LC) in the brainstem is a major source of noradrenergic input to the cortex and an important regulator of anxiety (Reinscheid et al., 2005). The second possible explanation is that the malformation had caused some of the symptoms, which then triggered the psychiatric symptoms. Chisholm et al. (1993) found that their patient’s anxiety did not improve after decompression surgery and that the patient had a family history of anxiety disorder. The authors subsequently suggested that symptoms of ACM may have acted as a trigger to the patient’s susceptibility for an anxiety disorder. Although our patient had no family history of an anxiety disorder, it is possible that there are alternative factors that predisposed him to anxiety disorder. It is known that ACM may be a cause of breathing-related sleep disorders (Dauvilliers et al., 2007). Iwabuchi et al. (1985) proposed that their patient’s anxiety was due to the sleep apnea and sleep deprivation caused by ACM. This may be another explanation for our patient’s anxiety symptoms. However, it is difficult to suggest this causality since there was a complete remission of his sleep disturbance after psychotropic treatment of anxiety disorder. 4. Conclusion The reported case emphasizes that a psychiatric disease in patients with a neurological disorder can defer diagnosis and suitable treatment
Akin A, Canakci Z, Sen A, Tore HF. Chiari-I malformation in two fighter pilots. Aviat Space Environ Med 2003;74(7):775–8. Bouwknecht JA, Spiga F, Staub DR, Hale MW, Shekhar A, Lowry CA. Differential effects of exposure to low-light or high-light open-field on anxiety-related behaviors: relationship to c-Fos expression in serotonergic and non-serotonergic neurons in the dorsal raphe nucleus. Brain Res Bull 2007;72(1):32–43. Caykoylu A, Deniz O, Aygül R. A case of Arnold–Chiari I malformation admitted depressive symptoms (in Turkish). MJAU 2001;33:83–5. Chisholm BT, Velamoor R, Chandarana PC, Cochrane DK. Anxiety disorder in a case of Arnold–Chiari malformation. J Psychiatry Neurosci 1993;18(2):67–8. Dauvilliers Y, Stal V, Abril B, Coubes P, Bobin S, Touchon J, et al. Chiari malformation and sleep related breathing disorders. J Neurol Neurosurg Psychiatry 2007;78 (12):1344–8. Grosso S, Scattolini R, Paolo G, Di Bartolo RM, Morgese G, Balestri P. Association of Chiari I malformation, mental retardation, speech delay, and epilepsy: a specific disorder? Neurosurgery 2001;49(5):1099–103. Hamilton M. The assesment of anxiety states by rating. Br J Psychiatry 1959;32:50. Ilanković NN, Ilanković AN, Bojić V, Ilanković LM. Chiari I malformation in adults: epileptiform events and schizophrenia-like psychosis. Psychiatr Danub 2006;18(1-2):92–6. Iwabuchi K, Miyauchi T, Kyuuma Y, Hosaka H, Kunimi Y, Yagishita S. A sudden-death in a case of Arnold–Chiari malformation (type I) with sleep apnea. No To Shinkei 1985;37(6):575–81. Mäkelä JP. Arnold-Chiari malformation type I in military conscripts: symptoms and effects on service fitness. Mil Med 2006;171(2):174–6. Nash J, Cheng JS, Meyer GA, Remler BF. Chiari type I malformation: overview of diagnosis and treatment. WMJ 2002;101(8):35–40. Reinscheid RK, Xu YL, Civelli O. Neuropeptide S: a new player in the modulation of arousal and anxiety. Mol Interv 2005;5(1):42–6. Spiga F, Lightman SL, Shekhar A, Lowry CA. Injections of urocortin 1 into the basolateral amygdala induce anxiety-like behavior and c-Fos expression in brainstem serotonergic neurons. Neuroscience 2006;138(4):1265–76. Strayer, A, (2001). Chiari I malformation: clinical presentation and management. J Neurosci Nurs. 33(2): 90-6, 104. World Health Organization. The ICD-10 Classification of Mental and Behavioural Disorders: Diagnostic Criteria for Research. Geneva: WHO; 1993.
Ali Caykoylu Okan Ekinci⁎ Yakup Albayrak Murat Kuloglu Department of Psychiatry, Atatürk Education and Research Hospital, Ankara, Turkey ⁎Corresponding author. Ankara Ataturk Egitim ve Arastırma Hastanesi Psikiyatri Klinigi, 06520 Bilkent/Ankara, Turkey. Tel.: +90 312 2912525; fax: +90 312 2912705. E-mail address: [email protected] (O. Ekinci). Orhan Deniz Department of Neurology, Atatürk Education and Research Hospital, Ankara, Turkey 11 April 2008