Electroconvulsive Therapy-Responsive Depression in a Patient with Progressive Supranuclear Palsy

We report the case of a 68-year-old woman with progressive supranuclear palsy whose depression was successfully treated with electroconvulsive therapy. She tolerated the treatments well and showed neither improvement nor decline in the neurologic symptoms of her illness.

INTRODUCTION

Electroconvulsive therapy (ECT) is the most rapid and effective method of treating major depression, and is safe and effective in treating depression in the context of many comorbid medical illnesses, including neurologic diseases resulting in movement disorders. Depression in Parkinson's disease ^(1,2), Huntington's disease ⁽³⁾, and tic disorders ⁽⁴⁾ have been successfully treated with ECT. Additionally, ECT has been used to successfully treat the symptoms of movement disorders in Parkinson's disease ^(5,6), Meige's syndrome ⁽⁷⁾, tardive dyskinesia ⁽⁸⁾, and tardive dystonia ^(9,16) in nondepressed patients.

Progressive supranuclear palsy (PSP) is a movement disorder with a Parkinsonian quality, marked by bradykinesia, supranuclear ocular palsy, subcortical dementia, and dystonia. No satisfactory treatment of PSP is currently available, although low-dose amitriptyline ⁽¹⁰⁾ has been reported to reduce some of the motor symptoms in severe cases. The combination of levodopa/carbidopa has also shown some mild efficacy in 33% of patients with PSP ⁽¹¹⁾.

ECT's effectiveness in the treatment of motor symptoms in PSP is unclear. One study of five patients ⁽¹²⁾ with PSP treated with ECT reported some improvement in the motor symptoms of three patients, but confusion limited the overall usefulness. ECT has also been reported to cause worsening of motor symptoms in a patient with PSP ⁽¹³⁾.

In this case report, we present a patient with PSP whose depressive disorder responded to ECT without significant confusion or exacerbation of PSP motor symptoms.

CASE REPORT

Ms. A. is a 68-year-old woman with a diagnosis of progressive supranuclear palsy who was referred to psychiatry at our facility for evaluation and treatment of depression characterized by emotional lability with frequent crying episodes, insomnia, anhedonia, decreased energy, and feelings of hopelessness. Her neurological symptoms began 3½ years prior to her presentation to our facility with the gradual onset of gait difficulty, and progressed to the development of rigidity in all extremities, axial extension, bradykinesia, and blepharospasm with upward gaze palsy. Family noted memory impairment, evidenced by leaving a gas stove turned on and unattended and being unable to manage finances and medications.

She developed progressively worsening behavioral difficulties. She slept poorly and called out loudly throughout the night. She would wander from the house, sometimes unclothed. Her appetite became quite diminished and she developed uncontrollable crying spells. Trials of carbidopa/levodopa provided no prolonged benefit in her neurologic symptoms; similarly, trials of risperidone, quetiapine, amitriptyline, and lorazepam brought no sustained reduction of insomnia, agitation, decreased appetite, or crying behaviors.

Two weeks prior to hospitalization at this facility, neurologic evaluation was consistent with PSP. The history and findings included insidious onset with progressive course, falls, and vertical gaze paresis. She scored 20/30 on a MiniMental Status Examination (MMSE). Physical examination was significant for upward gaze palsy, facial masking, increased axial tone with neck extension, and marked rigidity in all four extremities. She displayed an unsteady gait with short steps and bradykinesia. Reflexes were increased throughout. Laboratory studies including complete blood count, electrolytes, B12, folate, ALT (alanine aminotransferase), AST (aspartate aminotransferase), ceruloplasmin, protein electrophoresis, and sensitive thyroid-stimulating hormone (TSH) were all within normal limits. Magnetic resonance imaging of the head demonstrated decreased signal in the posterior putamen. The examining neurologist diagnosed her with PSP. Psychiatric consultation was obtained and it was recommended that she be hospitalized on a psychiatric unit for evaluation and treatment of a presumed depressive episode.

The patient was diagnosed with a major depressive disorder, meeting criteria according to DSM-IV. At the time of admission she endorsed feelings of hopelessness and depressed mood as well as anhedonia and decreased energy. Her appetite was notably reduced and she would cry frequently. She reported recurrent thoughts of taking an overdose of pills.

Her gait remained unsteady and she would frequently fall backwards. Prior to the initiation of ECT, a transverse sacral fracture was discovered. The patient did complain of pain and she was treated with analgesics, but the level of her agitation did not diminish.

Considering the severity and chronicity of her symptoms, along with the lag time necessary for antidepressants to be potentially effective in treating her depression, ECT was recommended. At the time ECT was initiated, the patient was receiving risperidone 0.5 mg b.i.d. and carbidopa/levodopa 25/100 immediate-release ½ tablet t.i.d. These were tapered and discontinued during the ECT course to lessen ECT-induced confusion. Thiopental anesthesia and glycopyrrolate were followed by succinyl choline for muscle relaxation. Seizures were induced by a Thymatron-DG device (Somatics, Inc., Lake Bluff, IL, U.S.A.) using a stimulus dose titration schedule beginning at 50.4 mC until a seizure threshold of 201.6 mC was attained. Subsequent treatment at 302.4 mC produced seizures of 62-109 s duration by electroencephalograph monitoring. Bilateral electrode placement was used throughout the ECT course.

The patient received three treatments a week for a total of seven treatments as an inpatient and showed great improvement in her presenting behavioral symptoms while hospitalized. After three treatments she was noted to be less tearful and showed a decrease in lability. At the time of discharge she was sleeping through the night, she was no longer anxious or agitated, her appetite was improved, she no longer expressed suicidal ideation, and she exhibited no behavioral disinhibition. Her MMSE score had decreased to 16/30 compared with the initial exam and was thought to have been induced by ECT. Despite this decline, her ability to meaningfully interact with family and her ability to assist nursing staff with her own activities of daily living improved and was maintained throughout the ECT course. The neurological examination, as detailed by the neurologist who had examined her prior to admission, was not appreciably changed relative to before starting ECT.

The patient was discharged to weekly maintenance ECT treatments and was placed in an assisted-living environment in her home community with family. She received five treatments on a weekly basis and her mood and behavior continued to remain stable. Additionally, serial MMSEs remained stable, ranging from 15-17 on subsequent evaluations.

The patient sustained a fall and was hospitalized in her home community, interrupting the outpatient series for 3 weeks. Approximately 2 weeks into this time period her insomnia, decreased appetite, and calling out behaviors began to return. The patient was rehospitalized at our facility and subsequently received a series of four bilateral treatments over a 10-day time span. In the interim, the patient's carbidopa/levodopa had been reinstituted by physicians in her home community. Given the reduced likelihood of ECT-induced confusion with maintenance ECT, it was continued during this phase of treatment. With the ECT series, she returned to the psychiatric and neurologic state that she had attained before the maintenance phase was interrupted.

The patient's psychiatric care was transferred to a facility closer to her home where maintenance ECT was planned, but the family declined further ECT. Citalopram was started and titrated to a dose of 40 mg q. day. At 4-month follow-up, the patient had experienced no significant return of the behavioral or affective disturbances that had precipitated the ECT.

DISCUSSION

While ECT had no positive or deleterious effects upon this patient's motor symptoms, as evidenced by the fact that there was no substantial change in her neurologic exam after the ECT course, her depression was rapidly and effectively treated with ECT. Despite her prominent neurologic illness, the ECT technique, anesthesia and neuromuscular blockade, and recovery room monitoring used in this patient's care did not need to be altered relative to those procedures used for ECT patients without PSP, with the exception that greater care was made in monitoring neurologic function during the procedure and in the recovery room.

Concomitant use of ECT and levodopa has been shown to increase the likelihood of confusion and dyskinetic movements in Parkinson's patients ⁽¹⁴⁾. This patient received the final four treatments while on levodopa without any evident adverse reaction, but, clearly, PSP patients receiving ECT while on levodopa need to be carefully monitored; if confusion or progression of motor symptoms occur, consideration should be given to decreasing or discontinuation of the levodopa. The patient experienced a 4-point reduction in the MMSE, which was not seen as debilitating, particularly since her ability to interact with family and engage in activities showed improvement. Previous research ⁽¹⁵⁾ has indicated that patients with basal ganglia lesions may have greater than usual cognitive impairment with ECT; thus, for PSP patients, consideration should be given to using unilateral electrode placement or twice-weekly treatment frequency.

Previous studies have shown a variable response of neurologic symptoms in PSP patients treated with ECT. This is likely due to a heterogeneity of factors within this disease population, including patient age, the stage of the illness at the time of treatment, the level of dementia, and comorbid illnesses. Clearly, clinicians treating depression in the context of PSP will need to consider these factors, and must be aware that ECT could potentially have a deleterious effect on motor and cognitive status during and after the course of ECT. However, based on our experience, we believe that for patients with PSP who have severe depressive symptoms requiring a rapid and effective treatment, ECT should be strongly considered.