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Electroconvulsive Therapy for Pediatric Malignant Catatonia With Cerebellar Dysgenesis
Electroconvulsive Therapy for Pediatric Malignant Catatonia With Cerebellar Dysgenesis Lee E. Wachtel, MD*†, Thomas O. Crawford, MD‡, Dirk M. Dhossche, MD, PhD§, and Irving M. Reti, MBBS* Electroconvulsive therapy was successfully used to treat malignant catatonia in a 15-year-old male patient with congenital dysgenesis of the left hemisphere of the cerebellum and hypoplasia of the vermis and left pons due to a presumed cerebral vascular accident in utero. The patient experienced significant motor and communication delays with mild cognitive impairment, but was otherwise in good health until age 15 years, when he developed rigidity, posturing, stupor, unresponsiveness, repetitive self-injurious behaviors, and negativism, as well as autonomic abnormalities including profuse diaphoresis and flushing episodes, thus meeting criteria for malignant catatonia. After initial response to lorazepam, the patient required electroconvulsive therapy for resolution of malignant catatonia. The case supports the safe and efficacious usage of electroconvulsive therapy for catatonia in adolescents with cerebellar and other developmental disorders. The role of the cerebellum in catatonia is also reviewed. Ó 2010 by Elsevier Inc. All rights reserved. Wachtel LE, Crawford TO, Dhossche DM, Reti IM. Electroconvulsive therapy for pediatric malignant catatonia with cerebellar dysgenesis. Pediatr Neurol 2010;43:427-430.
Introduction Catatonia is a unique neurobiologic syndrome characterized by motor, vocal, and behavioral symptoms. The term was coined by Kahlbaum [1] in 1874. Most frequently observed in psychiatric patients with affective and psychotic illness, catatonia also occurs in diverse neurologic conditions of infectious, infarct-related, traumatic, neoplastic, autoimmune, or neurodegenerative etiology [2-5]. Catatonia responds to g-aminobutyric acid modulating interventions, including benzodiazepines in increasing dosages, or to electroconvulsive therapy (in addition to or instead of the drug) if the pharmacologic therapy is insufficient or if patient morbidity demands rapid symptom resolution. Efficacy rates of electroconvulsive therapy for catatonia exceed 80% in adults [2,6,7]. Reports also demonstrate electroconvulsive therapy efficacy and safety for catatonic symptoms in youth with primary affective, psychotic, and autism spectrum disorders [8-10]. Presented here is the case of a 15-year-old male adolescent with congenital cerebellar abnormalities who developed profound catatonic deterioration requiring electroconvulsive therapy for definitive symptom resolution. Case Report A 15-year-old boy of European origin had dysgenesis of the left hemisphere of the cerebellum and hypoplasia of the vermis and left pons with associated cranial nerve deficits; the etiology was a presumed stroke in utero, at an estimated gestational age of 16 weeks, during a diagnostic amniocentesis. Developmental delay of gross and fine motor coordination and of language was apparent early. He walked independently at 54 months, but never developed spoken language. He achieved independence in all simple activities of daily living, and gained functional ability for adaptive communication using a voice-output device. Cognitive functioning was estimated to be in the range of borderline to mild mental retardation. The boy was able to participate in many family, school, and community activities, including biking, swimming, and even yachting. At age 14 years, he began to demonstrate facial tics and compulsions including arranging and observing rituals that lasted for hours and interfered with all functional activities. He then developed unprovoked episodes of self-injurious and aggressive behaviors of head banging against walls, screaming, kicking, biting, throwing anything within reach, and breaking glass and furniture. He was started on risperidone 0.25 mg in the morning and 0.5 mg at night with behavioral reduction for 2 weeks, until school staff reported to the mother that the patient was ‘‘stuck’’ at the computer, reportedly having pushed the same button 86 times, and that he was unable to move his finger from the keypad, or his body from the chair, for more
From the *Department of Psychiatry and Behavioral Sciences and the ‡ Department of Pediatric Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland; †Kennedy Krieger Institute, Baltimore, Maryland; and §Department of Psychiatry, University of Mississippi Medical Center, Jackson, Mississippi.
Communications should be addressed to: Dr. Wachtel; Department of Psychiatry; Kennedy Krieger Institute; Johns Hopkins School of Medicine; 707 North Broadway St., Rm. 232; Baltimore, MD 21205. E-mail: [email protected] Received March 4, 2010; accepted June 8, 2010.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2010.06.009 0887-8994/$—see front matter
Wachtel et al: ECT for Catatonia With Cerebellar Dysgenesis 427
than 1 hour. Multiple further signs of catatonia then developed over the next several days, including rigidity, maintenance of various postures (Fig 1), grimacing, repetitive movements of his fingers, staring, unresponsiveness, cessation of all communication, negativism in the form of food refusal and urinary retention, and intermittent autonomic dysfunction characterized by profuse diaphoresis and flushing. When the patient was assessed in the emergency department, the catatonic symptoms were attributed to antipsychotic usage and risperidone was discontinued. Neurologic evaluation demonstrated baseline cranial nerve deficits and incoordination in so far as evaluation was possible; routine laboratory and electroencephalography studies were unremarkable. Repeat magnetic resonance imaging was unchanged from baseline. Lorazepam challenge test was not pursued. Catatonic symptoms abated for 3 weeks, then recurred spontaneously two times at approximately 2-3 week intervals, lasting for 6-7 days each time and encompassing the same range of motor, vocal, behavioral, and autonomic symptoms as originally observed. He was then readmitted for aggressive treatment with lorazepam, with rapid dosage increase up to 2 mg thrice daily, with excellent symptom resolution. Lithium carbonate was begun prophylactically, based on the periodic nature of the patient’s catatonia [11]. Lithium was gradually titrated upward to 450 mg twice daily, with a resultant serum level of 0.71 mEq/L. The patient remained free of all catatonic symptoms for 4 months, but then motor and behavioral symptoms recurred with similar posturing, rigidity, repetitive movements, grimacing, aggression, and self-injury. Lorazepam was increased to 4.5 mg thrice daily with only short-lived symptom reduction. Further increase in lithium dosage led to excessive ataxia with falling. Paroxetine was introduced to target recurring compulsive behaviors, and was increased to 40 mg daily with moderate benefit. Because of worsening motor symptoms, food refusal, weight loss, difficulty initiating urination, and frequent flushing and profound diaphoresis, a course of electroconvulsive therapy was pursued. Bilateral electroconvulsive therapy was administered with a Spectrum 5000Q unit (Mecta, Portland, OR), with seizure monitoring by bifrontal electroencephalography. Methohexital 1 mg/kg was administered for anesthesia and succinylcholine 1 mg/kg as the muscle relaxant. The patient received a total of six treatments, at a frequency of three per week. Average charge during the acute course was 155 mC and average seizure length was 98 seconds. Following the acute course of six treatments, electroconvulsive therapy tapering to one treatment session per week occurred over the next 2 weeks. The patient demonstrated a profound and rapid response to electroconvulsive therapy. Upon return to the pediatric unit after his first treatment, he spontaneously voided more than 1 L of urine, and began to eat everything on his lunch tray. He resumed ambulation, began to engage in books and video and board games, and resumed use of his voice-output box. Posturing, rigidity, repetitive movements, and behavioral dyscontrol similarly resolved. He reported that he felt much better, but that before electroconvulsive therapy, ‘‘my mind felt like mashed potatoes.’’ He also reported remembering that he could not move, even though he wanted to. He transitioned to maintenance electroconvulsive therapy after six treatments, and returned to home and school, as well as a wide range of physical activities including diving, swimming, hiking, and sailing. His teachers reported outstanding improvement in all academic subjects, and claimed that he ‘‘never learned like this before [electroconvulsive therapy].’’ The patient continued to require weekly maintenance electroconvulsive therapy for the next 9 months. Several attempts at reducing frequency to every 9 days resulted in rapid return of motor, behavioral, and autonomic symptoms of catatonia. Pharmacotherapeutic interventions were maximized. Lithium carbonate was maintained at 450 mg twice daily. Lorazepam was maintained at 4 mg thrice daily, because of intermittent catatonic symptoms even between weekly electroconvulsive therapy. Paroxetine was increased to 60 mg daily, and memantine was begun to target residual compulsive behaviors as well as catatonic symptoms [12]. Memantine was titrated upward to 15 mg twice daily, which allowed for successful reduction of electroconvulsive therapy schedule to every 14 days without the previously observed prompt catatonic deterioration. At the time of this report, the patient continued to receive electroconvulsive therapy every 14 days, with plans for further increase in memantine and an ongoing gradual reduction in electroconvulsive therapy frequency.
428 PEDIATRIC NEUROLOGY Vol. 43 No. 6
Figure 1. Rigid and sustained posturing: a motor symptom of catatonia that appeared at age 15 years in an adolescent boy with cerebellar dysgenesis.
He was able to resume schooling and family and community activities, and tolerated electroconvulsive therapy so well that he was frequently able to steer his parents’ yacht less than 24 hours after a treatment (Fig 2).
Discussion Malignant catatonia developed in this adolescent with congenital cerebellar dysgenesis, and electroconvulsive therapy was required for resolution. No complications from the therapy occurred. Concomitant pharmacotherapeutic interventions were necessary to reduce the maintenance electroconvulsive therapy schedule, with ongoing plans for further frequency reduction as possible. Diagnosis of Catatonia The patient had an unusual baseline deficit involving abnormal development of the brainstem and unilateral cerebellar hemisphere. The exact etiology cannot be known, but was presumed to be due to congenital infarct. The present case is novel for the association of catatonia with static congenital unilateral cerebellar dysgenesis. Nonetheless, the progression of catatonia in the patient and his subsequent treatment course followed a classic trajectory. He also began to demonstrate signs of malignant catatonia with autonomic dysfunction. Further morbidity was accruing with food refusal and associated weight loss, as well as urinary retention. First described in 1934 by Stauder [13], untreated malignant catatonia currently has a 10-20% fatality rate; however, it responds readily to electroconvulsive therapy [2,3,7]. The patient’s initial symptoms included tics. The comorbidity of tics and catatonia has been identified in a growing number of reports, some of which include additional self-injurious and aggressive behaviors, and with many cases demonstrating excellent symptom resolution of tics, catatonia, and behavioral disturbance with electroconvulsive therapy [14,15]. Tics have recently been proposed as an alternative sign for catatonia [14]. Self-injurious
bipolar affective disorder, even during the more stable times when interactive assessment was possible. Paroxetine provided moderate benefit for compulsive behaviors. Electroconvulsive therapy was chosen because of inadequate psychotropic response. The patient exhibited no acute adverse neurologic effects from electroconvulsive therapy, despite his multiple baseline brain abnormalities. This case adds to the growing number of reports of safe and efficacious usage of electroconvulsive therapy in adolescents with a variety of affective, psychotic, and catatonic pathologies. Finally, his maintenance electroconvulsive therapy course is in accord with the understanding of electroconvulsive therapy as a treatment rather than a cure. Indeed, current research in maintenance electroconvulsive therapy for major depression, including the multisite Consortium for Research in Electroconvulsive Therapy (CORE) studies [18], indicates relapse rates up to 50% even when maintenance electroconvulsive therapy is pursued, with recommendations for individualizing tailoring of treatment schedules based on early signs of relapse. Concomitant mono- or polypharmacotherapy is also encouraged [18]. Catatonia and the Cerebellum
Figure 2. Steering a yacht, 24 hours after electroconvulsive therapy.
behaviors such as those in the present case have also been reported in catatonia, with patients demonstrating profound response to electroconvulsive therapy in terms of both frank catatonia and dangerous self-injury [16,17]. Treatment of Catatonia The patient initially demonstrated catatonic deterioration while receiving risperidone. Antipsychotics are known to potentiate catatonic symptoms, with the term neuroleptic malignant syndrome proposed to describe malignant catatonia caused by an antipsychotic [2,3,5,7]. The patient’s catatonic symptoms improved acutely with risperidone withdrawal, but recurred within several days, emphasizing the waxing and waning, and often cyclical, nature of catatonia, a feature that has been consistently recognized [2,3,7]. The present patient responded acutely to lorazepam as a first-line treatment for catatonia, but required increasing dosages. He was then started on lithium, based on reported successful use in periodic and autistic catatonia [11,16]. It was also considered that he might have an underlying mood disturbance, given that catatonia is most commonly found in mood disturbance [2,3,7] and also because there was a positive family history of depression. The patient did not, however, exhibit any frank symptoms of depression or
Evidence for cerebellar involvement in catatonia is largely limited to imaging studies, given the small number of neuroanatomic studies in catatonia. A reduction in cerebellar volume, smaller cerebellar vermis, and fourth ventricle enlargement have all been reported in catatonic schizophrenia [19,20]. Imaging studies of baseline cerebellar injury have demonstrated concomitant hypoperfusion in parietal, temporal, and prefrontal cortices, as well as perfusion changes in the thalamus and basal ganglia, and all of these structures have been implicated in the pathophysiology of catatonia [2,3,7,20,21]. Catatonia has further been reported in one case each of spinocerebellar degeneration [22] and hereditary cerebellar ataxia [23], and in both cases excellent resolution was achieved with electroconvulsive therapy. Cerebellar involvement has been reported in some but not all cases of Prader-Willi syndrome associated with catatonia [24]. Cerebellar lesions have been further associated with other neuropsychiatric symptoms, many of which overlap with the motor, vocal, and behavioral symptoms of catatonia. Excision of posterior fossa tumors is specifically associated with the development of cerebellar mutism, and mutism is a classic catatonic symptom. Acquired and congenital cerebellar damage may also result in symptoms such as mutism, dysarthria and other linguistic disturbance, executive and visual-spatial dysfunction, distractibility and inattention, perseveration and compulsions, and difficulty in initiation of voluntary movement, as well as affective instability and inappropriate or aggressive behaviors. This presentation is termed cerebellar cognitive affective syndrome, and exemplifies the concept of diaschisis, wherein local insult affects distant yet connected structures (in this case, cerebellar damage affecting the cortex) [20,21].
Wachtel et al: ECT for Catatonia With Cerebellar Dysgenesis 429
Conclusion The present case adds to the growing number of reports of catatonia in pediatric developmental disorders, as well as the safe and efficacious usage of electroconvulsive therapy in its resolution. Thus, age and developmental level should not be seen as a barrier to electroconvulsive therapy in an acute clinical presentation of catatonia. Similar to typically developing patients, the present patient required maintenance electroconvulsive therapy to maintain therapeutic benefits. The effects of lorazepam, lithium, paroxetine, and memantine for acute and maintenance treatment were beneficial, but require further experience and study. Future research into the occurrence of catatonia in patients with cerebellar dysfunction, with and without autism, is warranted. References [1] Kahlbaum K. Catatonia, or tension insanity: a clinical form of psychic illness [Die Katatonie oder das Spannungsirresein. Eine klinische Form psychischer Krankheit] [In German]. Berlin: A. Hirschwald, 1874. [2] Fink M, Taylor MA. Catatonia: a history. The differential diagnosis of catatonia. Past treatments for catatonia. Management of catatonia today. In Catatonia: a clinician’s guide to diagnosis and treatment. Cambridge: Cambridge University Press, 2003:1-18; 71-113; 133-46; 147-176. [3] Caroff SN, Mann SC, Campbell EC, Sullivan KA. Epidemiology. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:15-31. [4] Carroll BT, Goforth HW. Medical catatonia. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:121-7. [5] Lopez-Canino A, Francis A. Drug-induced catatonia. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:129-40. [6] Rosebush PI, Mazurek MF. Pharmacotherapy. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:141-50. [7] Petrides G, Malur C, Fink M. Convulsive therapy. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:151-60.
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[8] Rey J, Walter G. Half a century of ECT use in young people. Am J Psychiatry 1997;154:595-602. [9] Fink M, Taylor MA, Ghaziuddin N. Catatonia in autism spectrum disorders: a medical treatment algorithm. In: Dhossche DM, Wing L, Ohta M, Neumarker K-J, editors. Catatonia in autism spectrum disorders. San Diego, CA: Academic Press, 2006:233-45. [10] Dhossche DM, Shah A, Wing L. Blueprints for the assessment, treatment, and future study of catatonia in autism spectrum disorders. In: Dhossche DM, Wing L, Ohta M, Neumarker K-J, editors. Catatonia in autism spectrum disorders. San Diego, CA: Academic Press, 2006: 268-84. [11] Pheterson AD, Estroff TW, Sweeney DR. Severe prolonged catatonia with associated flushing reaction responsive to lithium carbonate. J Am Acad Child Psychiatry 1985;24:235-7. [12] Munoz C, Yulan N, Achaval V, Appiani F, Carroll BT. Memantine in major depression with catatonic features. J Neuropsychiatry Clin Neurosci 2008;20:119-20. [13] Stauder KH. Malignant catatonia [Die to¨dliche Katatonie] [In German]. Arch Psychiatr Nervenkr 1934;102:614-34. [14] Dhossche DM, Reti IM, Shettar SM, Wachtel LE. Tics as signs of catatonia: electroconvulsive therapy response in two men. J ECT Feb. 8, 2010 (Epub ahead of print). [15] Karadenizli D, Dilbaz N, Bayam G. Gilles de la Tourette syndrome response to electroconvulsive therapy. J ECT 2005;21:246-8. [16] Wachtel LE, Griffin M. Reti IM Electroconvulsive therapy in a man with autism experiencing severe depression, catatonia and selfinjury. J ECT 2010;26:70-3. [17] Wachtel LE, Kahng S, Dhossche DM, Cascella N, Reti IM. ECT for catatonia in an autistic girl. Am J Psychiatry 2008;165:329-33. [18] Kellner CH, Knapp RG, Petrides G, et al. Continuation electroconvulsive therapy vs pharmacotherapy for relapse prevention in major depression: a multisite study from the Consortium for Research in Electroconvulsive Therapy (CORE). Arch Gen Psychiatry 2006;63: 1337-44. [19] Wilcox JA. Cerebellar atrophy and catatonia. Biol Psychiatry 1991;29:733-4. [20] Schmahmann JD, Weilburg JB, Sherman JC. The neuropsychiatry of the cerebellum: insights from the clinic. Cerebellum 2007;6:254-67. [21] Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain 1998;121:561-79. [22] Suzuki K, Itou K, Takano T, Harada N, Awata S, Matsuoka H. Catatonic stupor superimposed on hereditary spinocerebellar degeneration resolved with electroconvulsive therapy. Prog Neuropsychopharmacol Biol Psychiatry 2006;30:1179-81. [23] Folkerts HW, Stadtland C, Reker T. ECT for organic catatonia due to hereditary cerebellar ataxia. J ECT 1998;14:53-5. [24] Titomanlio L, De Brasi D, Romano A, Genesio R, Diano AA, Del Giudice E. Partial cerebellar hypoplasia in a patient with PraderWilli syndrome. Acta Paediatr 2006;95:861-3.
Introduction Catatonia is a unique neurobiologic syndrome characterized by motor, vocal, and behavioral symptoms. The term was coined by Kahlbaum [1] in 1874. Most frequently observed in psychiatric patients with affective and psychotic illness, catatonia also occurs in diverse neurologic conditions of infectious, infarct-related, traumatic, neoplastic, autoimmune, or neurodegenerative etiology [2-5]. Catatonia responds to g-aminobutyric acid modulating interventions, including benzodiazepines in increasing dosages, or to electroconvulsive therapy (in addition to or instead of the drug) if the pharmacologic therapy is insufficient or if patient morbidity demands rapid symptom resolution. Efficacy rates of electroconvulsive therapy for catatonia exceed 80% in adults [2,6,7]. Reports also demonstrate electroconvulsive therapy efficacy and safety for catatonic symptoms in youth with primary affective, psychotic, and autism spectrum disorders [8-10]. Presented here is the case of a 15-year-old male adolescent with congenital cerebellar abnormalities who developed profound catatonic deterioration requiring electroconvulsive therapy for definitive symptom resolution. Case Report A 15-year-old boy of European origin had dysgenesis of the left hemisphere of the cerebellum and hypoplasia of the vermis and left pons with associated cranial nerve deficits; the etiology was a presumed stroke in utero, at an estimated gestational age of 16 weeks, during a diagnostic amniocentesis. Developmental delay of gross and fine motor coordination and of language was apparent early. He walked independently at 54 months, but never developed spoken language. He achieved independence in all simple activities of daily living, and gained functional ability for adaptive communication using a voice-output device. Cognitive functioning was estimated to be in the range of borderline to mild mental retardation. The boy was able to participate in many family, school, and community activities, including biking, swimming, and even yachting. At age 14 years, he began to demonstrate facial tics and compulsions including arranging and observing rituals that lasted for hours and interfered with all functional activities. He then developed unprovoked episodes of self-injurious and aggressive behaviors of head banging against walls, screaming, kicking, biting, throwing anything within reach, and breaking glass and furniture. He was started on risperidone 0.25 mg in the morning and 0.5 mg at night with behavioral reduction for 2 weeks, until school staff reported to the mother that the patient was ‘‘stuck’’ at the computer, reportedly having pushed the same button 86 times, and that he was unable to move his finger from the keypad, or his body from the chair, for more
From the *Department of Psychiatry and Behavioral Sciences and the ‡ Department of Pediatric Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland; †Kennedy Krieger Institute, Baltimore, Maryland; and §Department of Psychiatry, University of Mississippi Medical Center, Jackson, Mississippi.
Communications should be addressed to: Dr. Wachtel; Department of Psychiatry; Kennedy Krieger Institute; Johns Hopkins School of Medicine; 707 North Broadway St., Rm. 232; Baltimore, MD 21205. E-mail: [email protected] Received March 4, 2010; accepted June 8, 2010.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2010.06.009 0887-8994/$—see front matter
Wachtel et al: ECT for Catatonia With Cerebellar Dysgenesis 427
than 1 hour. Multiple further signs of catatonia then developed over the next several days, including rigidity, maintenance of various postures (Fig 1), grimacing, repetitive movements of his fingers, staring, unresponsiveness, cessation of all communication, negativism in the form of food refusal and urinary retention, and intermittent autonomic dysfunction characterized by profuse diaphoresis and flushing. When the patient was assessed in the emergency department, the catatonic symptoms were attributed to antipsychotic usage and risperidone was discontinued. Neurologic evaluation demonstrated baseline cranial nerve deficits and incoordination in so far as evaluation was possible; routine laboratory and electroencephalography studies were unremarkable. Repeat magnetic resonance imaging was unchanged from baseline. Lorazepam challenge test was not pursued. Catatonic symptoms abated for 3 weeks, then recurred spontaneously two times at approximately 2-3 week intervals, lasting for 6-7 days each time and encompassing the same range of motor, vocal, behavioral, and autonomic symptoms as originally observed. He was then readmitted for aggressive treatment with lorazepam, with rapid dosage increase up to 2 mg thrice daily, with excellent symptom resolution. Lithium carbonate was begun prophylactically, based on the periodic nature of the patient’s catatonia [11]. Lithium was gradually titrated upward to 450 mg twice daily, with a resultant serum level of 0.71 mEq/L. The patient remained free of all catatonic symptoms for 4 months, but then motor and behavioral symptoms recurred with similar posturing, rigidity, repetitive movements, grimacing, aggression, and self-injury. Lorazepam was increased to 4.5 mg thrice daily with only short-lived symptom reduction. Further increase in lithium dosage led to excessive ataxia with falling. Paroxetine was introduced to target recurring compulsive behaviors, and was increased to 40 mg daily with moderate benefit. Because of worsening motor symptoms, food refusal, weight loss, difficulty initiating urination, and frequent flushing and profound diaphoresis, a course of electroconvulsive therapy was pursued. Bilateral electroconvulsive therapy was administered with a Spectrum 5000Q unit (Mecta, Portland, OR), with seizure monitoring by bifrontal electroencephalography. Methohexital 1 mg/kg was administered for anesthesia and succinylcholine 1 mg/kg as the muscle relaxant. The patient received a total of six treatments, at a frequency of three per week. Average charge during the acute course was 155 mC and average seizure length was 98 seconds. Following the acute course of six treatments, electroconvulsive therapy tapering to one treatment session per week occurred over the next 2 weeks. The patient demonstrated a profound and rapid response to electroconvulsive therapy. Upon return to the pediatric unit after his first treatment, he spontaneously voided more than 1 L of urine, and began to eat everything on his lunch tray. He resumed ambulation, began to engage in books and video and board games, and resumed use of his voice-output box. Posturing, rigidity, repetitive movements, and behavioral dyscontrol similarly resolved. He reported that he felt much better, but that before electroconvulsive therapy, ‘‘my mind felt like mashed potatoes.’’ He also reported remembering that he could not move, even though he wanted to. He transitioned to maintenance electroconvulsive therapy after six treatments, and returned to home and school, as well as a wide range of physical activities including diving, swimming, hiking, and sailing. His teachers reported outstanding improvement in all academic subjects, and claimed that he ‘‘never learned like this before [electroconvulsive therapy].’’ The patient continued to require weekly maintenance electroconvulsive therapy for the next 9 months. Several attempts at reducing frequency to every 9 days resulted in rapid return of motor, behavioral, and autonomic symptoms of catatonia. Pharmacotherapeutic interventions were maximized. Lithium carbonate was maintained at 450 mg twice daily. Lorazepam was maintained at 4 mg thrice daily, because of intermittent catatonic symptoms even between weekly electroconvulsive therapy. Paroxetine was increased to 60 mg daily, and memantine was begun to target residual compulsive behaviors as well as catatonic symptoms [12]. Memantine was titrated upward to 15 mg twice daily, which allowed for successful reduction of electroconvulsive therapy schedule to every 14 days without the previously observed prompt catatonic deterioration. At the time of this report, the patient continued to receive electroconvulsive therapy every 14 days, with plans for further increase in memantine and an ongoing gradual reduction in electroconvulsive therapy frequency.
428 PEDIATRIC NEUROLOGY Vol. 43 No. 6
Figure 1. Rigid and sustained posturing: a motor symptom of catatonia that appeared at age 15 years in an adolescent boy with cerebellar dysgenesis.
He was able to resume schooling and family and community activities, and tolerated electroconvulsive therapy so well that he was frequently able to steer his parents’ yacht less than 24 hours after a treatment (Fig 2).
Discussion Malignant catatonia developed in this adolescent with congenital cerebellar dysgenesis, and electroconvulsive therapy was required for resolution. No complications from the therapy occurred. Concomitant pharmacotherapeutic interventions were necessary to reduce the maintenance electroconvulsive therapy schedule, with ongoing plans for further frequency reduction as possible. Diagnosis of Catatonia The patient had an unusual baseline deficit involving abnormal development of the brainstem and unilateral cerebellar hemisphere. The exact etiology cannot be known, but was presumed to be due to congenital infarct. The present case is novel for the association of catatonia with static congenital unilateral cerebellar dysgenesis. Nonetheless, the progression of catatonia in the patient and his subsequent treatment course followed a classic trajectory. He also began to demonstrate signs of malignant catatonia with autonomic dysfunction. Further morbidity was accruing with food refusal and associated weight loss, as well as urinary retention. First described in 1934 by Stauder [13], untreated malignant catatonia currently has a 10-20% fatality rate; however, it responds readily to electroconvulsive therapy [2,3,7]. The patient’s initial symptoms included tics. The comorbidity of tics and catatonia has been identified in a growing number of reports, some of which include additional self-injurious and aggressive behaviors, and with many cases demonstrating excellent symptom resolution of tics, catatonia, and behavioral disturbance with electroconvulsive therapy [14,15]. Tics have recently been proposed as an alternative sign for catatonia [14]. Self-injurious
bipolar affective disorder, even during the more stable times when interactive assessment was possible. Paroxetine provided moderate benefit for compulsive behaviors. Electroconvulsive therapy was chosen because of inadequate psychotropic response. The patient exhibited no acute adverse neurologic effects from electroconvulsive therapy, despite his multiple baseline brain abnormalities. This case adds to the growing number of reports of safe and efficacious usage of electroconvulsive therapy in adolescents with a variety of affective, psychotic, and catatonic pathologies. Finally, his maintenance electroconvulsive therapy course is in accord with the understanding of electroconvulsive therapy as a treatment rather than a cure. Indeed, current research in maintenance electroconvulsive therapy for major depression, including the multisite Consortium for Research in Electroconvulsive Therapy (CORE) studies [18], indicates relapse rates up to 50% even when maintenance electroconvulsive therapy is pursued, with recommendations for individualizing tailoring of treatment schedules based on early signs of relapse. Concomitant mono- or polypharmacotherapy is also encouraged [18]. Catatonia and the Cerebellum
Figure 2. Steering a yacht, 24 hours after electroconvulsive therapy.
behaviors such as those in the present case have also been reported in catatonia, with patients demonstrating profound response to electroconvulsive therapy in terms of both frank catatonia and dangerous self-injury [16,17]. Treatment of Catatonia The patient initially demonstrated catatonic deterioration while receiving risperidone. Antipsychotics are known to potentiate catatonic symptoms, with the term neuroleptic malignant syndrome proposed to describe malignant catatonia caused by an antipsychotic [2,3,5,7]. The patient’s catatonic symptoms improved acutely with risperidone withdrawal, but recurred within several days, emphasizing the waxing and waning, and often cyclical, nature of catatonia, a feature that has been consistently recognized [2,3,7]. The present patient responded acutely to lorazepam as a first-line treatment for catatonia, but required increasing dosages. He was then started on lithium, based on reported successful use in periodic and autistic catatonia [11,16]. It was also considered that he might have an underlying mood disturbance, given that catatonia is most commonly found in mood disturbance [2,3,7] and also because there was a positive family history of depression. The patient did not, however, exhibit any frank symptoms of depression or
Evidence for cerebellar involvement in catatonia is largely limited to imaging studies, given the small number of neuroanatomic studies in catatonia. A reduction in cerebellar volume, smaller cerebellar vermis, and fourth ventricle enlargement have all been reported in catatonic schizophrenia [19,20]. Imaging studies of baseline cerebellar injury have demonstrated concomitant hypoperfusion in parietal, temporal, and prefrontal cortices, as well as perfusion changes in the thalamus and basal ganglia, and all of these structures have been implicated in the pathophysiology of catatonia [2,3,7,20,21]. Catatonia has further been reported in one case each of spinocerebellar degeneration [22] and hereditary cerebellar ataxia [23], and in both cases excellent resolution was achieved with electroconvulsive therapy. Cerebellar involvement has been reported in some but not all cases of Prader-Willi syndrome associated with catatonia [24]. Cerebellar lesions have been further associated with other neuropsychiatric symptoms, many of which overlap with the motor, vocal, and behavioral symptoms of catatonia. Excision of posterior fossa tumors is specifically associated with the development of cerebellar mutism, and mutism is a classic catatonic symptom. Acquired and congenital cerebellar damage may also result in symptoms such as mutism, dysarthria and other linguistic disturbance, executive and visual-spatial dysfunction, distractibility and inattention, perseveration and compulsions, and difficulty in initiation of voluntary movement, as well as affective instability and inappropriate or aggressive behaviors. This presentation is termed cerebellar cognitive affective syndrome, and exemplifies the concept of diaschisis, wherein local insult affects distant yet connected structures (in this case, cerebellar damage affecting the cortex) [20,21].
Wachtel et al: ECT for Catatonia With Cerebellar Dysgenesis 429
Conclusion The present case adds to the growing number of reports of catatonia in pediatric developmental disorders, as well as the safe and efficacious usage of electroconvulsive therapy in its resolution. Thus, age and developmental level should not be seen as a barrier to electroconvulsive therapy in an acute clinical presentation of catatonia. Similar to typically developing patients, the present patient required maintenance electroconvulsive therapy to maintain therapeutic benefits. The effects of lorazepam, lithium, paroxetine, and memantine for acute and maintenance treatment were beneficial, but require further experience and study. Future research into the occurrence of catatonia in patients with cerebellar dysfunction, with and without autism, is warranted. References [1] Kahlbaum K. Catatonia, or tension insanity: a clinical form of psychic illness [Die Katatonie oder das Spannungsirresein. Eine klinische Form psychischer Krankheit] [In German]. Berlin: A. Hirschwald, 1874. [2] Fink M, Taylor MA. Catatonia: a history. The differential diagnosis of catatonia. Past treatments for catatonia. Management of catatonia today. In Catatonia: a clinician’s guide to diagnosis and treatment. Cambridge: Cambridge University Press, 2003:1-18; 71-113; 133-46; 147-176. [3] Caroff SN, Mann SC, Campbell EC, Sullivan KA. Epidemiology. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:15-31. [4] Carroll BT, Goforth HW. Medical catatonia. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:121-7. [5] Lopez-Canino A, Francis A. Drug-induced catatonia. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:129-40. [6] Rosebush PI, Mazurek MF. Pharmacotherapy. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:141-50. [7] Petrides G, Malur C, Fink M. Convulsive therapy. In: Caroff SN, Mann SC, Francis A, Fricchione GL, editors. Catatonia: from psychopathology to neurobiology. Washington, DC: American Psychiatric Publishing, 2004:151-60.
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