Objective: Evaluation of therapeutic options for a patient of Progressive Supranuclear Palsy (PSP) presenting with incessant purposeless groaning.

Background: PSP is a neurodegenerative disorder characterized by early backward falls, rigidity, balance impairment, vertical supranuclear gaze palsy, dysarthria and frontal dementia. Purposeless
groaning is reported in PSP in later stages [1].

Method: A 65 year old retired plumber presented to the OPD with symptoms of gait difficulty with backward falls for 4 years, marked behavioral problems initially characterized by temper outbursts which later evolved into apathy for 3 years, dysarthria for 2 years, downward gaze abnormality for 2 years and had been non ambulatory and wheel-chair bound for past 1 year. MRI was consistent with diagnosis of PSP with magnetic resonance Parkinsonism index being 34 [Fig 1,2]. Patient had developed constant incessant purposeless groaning for past 6 months which was very distressing to his caretakers. He was constantly emitting a loud guttural groaning sound even while being examined. Patient had severe dysarthria but his wife could still partly understand his speech. Patient did not report any pain/discomfort to which the groaning could be attributed. He had been treated with Quetiapine, Resperidone and Olanzapine for this without any significant benefit.

Results: Pimozide was started at dose of 1mg and was subsequently increased to 4 mg over 3 weeks. After 4 weeks, patient’s attendants reported decrease in groaning by 70-80%. Patients truncal rigidity had increased minimally but since patient was non-ambulatory, it did not cause any apparent discomfort. The improvement was maintained at 6 and 12 weeks follow up at same dosage.

Conclusion: We report the first case of PSP with purposeless grunting-groaning or perseverative vocalization successfully treated with Pimozide. The neuro-anatomic localization of perseverative vocalization is believed to be frontal or a frontal-subcortical pathology. Pimozide is the preferred drug for involuntary vocalizations in Tourette’s syndrome [2].  Perseverative Involuntary vocalizations causes considerable distress to the patient, the caretakers and the attending health care workers. No clear guidelines exist on treatment. Pimozide is a promising drug despite the potential of extra-pyramidal side effects.

References: 1. Stamelou M, Rubio-Agusti I, Quinn N, Bhatia K. Characteristic constant groaning in late stage progressive supranuclear palsy: a case report. Parkinsonism Relat Disord 2011;17:575-576. 2. Sallee FR, Nesbitt L, Jackson C , et al. Relative efficacy of haloperidol and pimozide in children and adolescents with Tourette’s disorder. Am J Psychiatry 1997 Aug; 154 : 1057 –62

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