Journal of ECT • Volume 31, Number 1, March 2015

Letters to the Editor

Ethan O. Bryson received royalties from Springer and New Horizon Press. Charles H. Kellner received grant support from National Institute of Mental Health, honoraria for writing electroconvulsive therapy (ECT) sections from UpToDate, royalties from Cambridge University Press, honoraria for teaching ECT course from North Shore Long Island Jewish Health System, and honoraria for ECT articles from Psychiatric Times. The remaining authors have no conflicts of interest or financial disclosures to report. REFERENCES 1. Patrick J, Lindstrom J. Autoimmune response to acetylcholine receptor. Science. 1973;180: 871–872. 2. Pande AC, Grunhaus LJ. ECT for depression in the presence of myasthenia gravis. Convuls Ther. 1990;6:172–175. 3. Calarge CA, Crowe RR. Electroconvulsive therapy in myasthenia gravis. Ann Clin Psychiatry. 2004;16:225–227. 4. Anzai T, Yokoyama J, Ohba S, et al. An important initial diagnosis of a patient with Graves' disease associated with myasthenia gravis, thyroid carcinoma, and thymoma. Head Neck Oncol. 2012;4:56. 5. Blichfeldt-Lauridsen L, Hansen BD. Anesthesia and myasthenia gravis. Acta Anaesthesiol Scand. 2012;56:17–22.

Use of Electroconvulsive Therapy in Schizophrenia With Klippel-Feil Syndrome To the Editor: linicians are cautious with electroconvulsive therapy (ECT) when the patient has significant nonpsychiatric comorbidity, or when the patient is receiving certain medications. What about abnormal variations in body structure? We present the uneventful use of ECT in a schizophrenia patient with Klippel-Feil (KF) syndrome.

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CASE REPORT S.T., an unmarried, 32-year-old man, was brought into treatment with an 8-year history of irritable mood, hearing voices, talking to himself, decreased self-care, and absence of meaningful engagement with his environment. He had been diagnosed with chronic schizophrenia and had been referred for ECT after failing to respond to trials with different antipsychotic drugs (details unavailable).

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S.T. was observed to have a very short neck with marked restriction of head movement: barring slight flexion, no lateral or rotatory movements were possible. The deformity was reportedly present since birth. His mother and brothers also had short necks. Radiological examination showed complex curvatural abnormality in the cervical spine with fusion of all cervical vertebrae. A diagnosis of KF syndrome was made. Electroconvulsive therapy was considered indicated because of the difficulties that the family faced in caring for a patient who was completely nonfunctional and disengaged from reality during his 8-year, medication-refractory illness. An othorpedic clearance was obtained for high-risk ECT. Written informed consent for ECT was obtained from the patient's legal guardian after explanation about treatment risks. S.T. received 12 bitemporal ECTs thrice weekly; his ongoing medications (clozapine, 25 mg/d; olanzapine, 10 mg/d) were continued unchanged. All treatments were modified using thiopentone sodium (250 mg) and succinylcholine (0.5 mg/kg). All treatments were delivered at 60 mC charge; motor seizure durations ranged from 21 to 50 seconds. He improved moderately with ECT—scores on the positive and negative syndrome scale dropped from 14, 37, 59, and 110 to 7, 26, 38, and 7, for positive symptoms, negative symptoms, general psychopathology, and total scale ratings, respectively; that is, a reduction of 35% on the total scale score. Clinically, hallucinatory behavior ceased, family interactions improved, and he assumed simple responsibilities in the family business.

DISCUSSION The KF syndrome is a heterogeneous group of conditions characterized by congenital fusion of at least 2 cervical vertebrae and consequent limitation of neck movements.1,2 The syndrome is rare, with prevalence estimated at about 1 in 40,000.3 The KF syndrome is problematic for at least 2 reasons in patients advised ECT: 1. When there is restriction of neck movement due to fusion of cervical vertebrae, there is a risk that a violent convulsion during ECT may damage the cervical spine; this, in turn, can damage the spinal cord.4 In retrospect, we realize that we should have administered a higher dose of succinylcholine, such as 1 mg/kg, for better muscle relaxation. Fortunately, there were no musculoskeletal or neurological complications.

2. Airway management can be difficult when neck movements are anatomically restricted.5 If the patient had required intubation to manage prolonged apnea after ECT, use of a flexible endoscope would have been necessary because of the impossibility of neck manipulation during intubation. Fortunately, such a need did not arise.

The patient received 12 ECTs uneventfully and showed modest clinical and functional response. This is probably the first report of the safe use of ECT in a patient with the KF syndrome. As a parting note, the clozapine that he was receiving may have lowered the seizure threshold, which is why he experienced adequate seizures despite the low electrical charge (60 mC) delivered at every ECT in the course. Delnaz Palsetia, DPM Sagar Karia, MD Nilesh Shah, DPM, MD Department of Psychiatry Lokmanya Tilak Municipal Medical College and General Hospital Sion, Mumbai, India

Chittaranjan Andrade, MD Department of Psychopharmacology National Institute of Mental Health and Neurosciences Bangalore, India [email protected]

The authors have no conflicts of interest or financial disclosures to report.

REFERENCES 1. Tracy MR, Dormans JP, Kusumi K. Klippel-Feil syndrome: clinical features and current understanding of etiology. Clin Orthop Relat Res. 2004;424:183–190. 2. Samartzis DD, Herman J, Lubicky JP, et al. Classification of congenitally fused cervical patterns in Klippel-Feil patients: epidemiology and role in the development of cervical spine-related symptoms. Spine (Phila Pa 1976). 2006;31:E798–E804. 3. Thomsen MN, Schneider U, Weber M, et al. Scoliosis and congenital anomalies associated with Klippel-Feil syndrome types I–III. Spine (Phila Pa 1976). 1997;22: 396–401. 4. Senoglu M, Ozbag D, Gumusalan Y. Two cases of Klippel-Feil syndrome. Int J Anat Variations. 2008;1:6–7. 5. Stallmer ML, Vanaharam V, Mashour GA. Congenital cervical spine fusion and airway management: a case series of Klippel-Feil syndrome. J Clin Anesth. 2008;20:447–451.

© 2015 Wolters Kluwer Health, Inc. All rights reserved.

Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.

Use of electroconvulsive therapy in schizophrenia with Klippel-Feil syndrome.

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