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 Table of Contents  
Year : 2014  |  Volume : 1  |  Issue : 2  |  Page : 110-111

Affective symptoms in progressive supranuclear palsy

Department of Psychiatry, Government Medical College, Thrissur, Kerala, India

Date of Web Publication3-Mar-2015

Correspondence Address:
Thrissur K S Shaji
Department of Psychiatry, Government Medical College, Thrissur - 680 596, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2348-9995.152433

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Progressive supranuclear palsy is an unusual neurodegenerative disorder, which superficially resembles Parkinson's disease, and the initial manifestations are characterized by depression, akinesia and mild cognitive impairment. The motor symptoms often appear later. Here we describe a case in whom affective symptoms predated the onset of other symptoms.

Keywords: Depressive disorders, progressive supranuclear palsy, Parkinsonian disorders

How to cite this article:
Shaji K S, Jyothi K S. Affective symptoms in progressive supranuclear palsy. J Geriatr Ment Health 2014;1:110-1

How to cite this URL:
Shaji K S, Jyothi K S. Affective symptoms in progressive supranuclear palsy. J Geriatr Ment Health [serial online] 2014 [cited 2020 Jul 14];1:110-1. Available from:

  Introduction Top

Progressive supranuclear palsy (PSP) is an unusual neurodegenerative disorder that superficially resembles Parkinson's disease (PD). It is characterized by gaze palsy, bulbar signs, Parkinsonian signs, and mental changes. While mental changes are a frequent finding, they have, with the exception of dementia, been poorly defined. Many studies had looked at cross-sectional prevalence of psychiatric symptoms. [1],[2],[3] We report the long-term course and management of psychiatric symptoms in a case of PSP.

  Case Report Top

A 51-year-old male presented with depressive symptoms of 2 month's duration. He had come on his own as he felt that something was wrong with his mind. He had mild akinesia and mild rigidity on neurological examination, but did not have tremor or cognitive impairment. The investigations including computerized tomography scan and thyroid function tests were normal. A diagnosis of major depressive disorder was made as per the Diagnostic and Statistical Manual of Mental Disorders-IV criteria. Early PD was considered as the probable cause for  Parkinsonism More Details. He was treated with tablet dothiepin (100 mg/day). Depressive symptoms remitted following this treatment, but the akinetic rigid syndrome persisted. In view of this, he was started on tablet trihexyphenydyl (2 mg in the morning and in the afternoon) and this lead to prompt symptomatic relief. He was seen by a neurologist who agreed with the diagnosis of PD.

Serial assessments of memory were done during this period. Initial Wechsler's memory scale score was found to be 73, which was on the lower side. This was attributed to his difficulty in constructive tasks and verbal productivity. Retesting was done again in the 2 nd year of the illness. There was improvement in the test scores following improvement in motor and depressive symptoms. Cognitive symptoms were judged to be mild and were not severe or disabling enough to meet criteria for dementia. He did not have any disability, which could be attributed to cognitive dysfunction.

Symptoms such as dysarthria, gait problems and subtle personality changes were noticed in the latter part of 2 nd year of the illness. Spastic dysarthria, gaze abnormalities, gait disturbances and history of frequent falls became prominent. At his point, a diagnosis of PSP was made. A magnetic resonance imaging study of brain done during the 6 th year of the illness showed midbrain atrophy consistent with the diagnosis of PSP. Affective disturbances were present throughout the course of the illness. Full syndromal depression was the initial feature, and it responded well to tricyclic antidepressants. Another remarkable feature was the gradual onset of apathy and the subsequent disappearance of depressive symptoms. Apathy continued as the predominant affective symptom in the latter part of the illness. Emotional lability and pathological laughter appeared later, along with other pseudobulbar symptoms.

We discontinued antidepressants in the 2 nd year, and the patient did not have a relapse of depression. However, the anticholinergic drug was continued for 3 years since the patient reported reduction in mobility and ease of speech while attempting to discontinue this. We did not notice any significant worsening of cognitive functions during the 3 years when he was on anticholinergics. As the illness progressed further, the frequency of falls increased and he had an accidental death, following a fall during the 7 th year of follow-up. According to relatives, he had worsening of cognition and speech months before his death.

  Discussion Top

It is not unusual to miss the diagnosis of PSP, especially during the earlier part of the illness. We made the diagnosis of PSP only when repeated falls were reported, and the eye signs were evident on clinical examination. Depression, akinesia and mild cognitive impairment were the early clinical features of this case. Depression is considered as the most frequent psychiatric symptom in PSP and the possibility of sub-cortical disease like PSP has to be suspected when patients present with depression and Parkinsonism. Depression can be an early presenting feature of PSP. [4],[5]

The longitudinal course of affective symptoms, in this case, is interesting. Full syndromal depression was the early feature. This lead help seeking, indicating the subjective distress due to symptoms and the preserved insight in the initial phase of the illness. The good response antidepressant drug indicate biological basis for the syndrome of depression. Tricyclic antidepressants and even elelctroconvulsive therapy had been used to relieve depressive symptoms in PSP. [6] We found apathy replacing the depression over a period. Development of inappropriate affective responses later on in the course of the illness happened along with the appearance of dysarthria and pseudo bulbar features. This can be referred to as pseudo bulbar affect (PBA) [7] which is characterized by uncontrolled crying or laughing which may be disproportionate or inappropriate to the social context. Thus, in PBA there is a disparity between the patient's emotional expression and his or her emotional and his or her emotional experience. This variability of affective symptoms during the course of PSP may depend on the progression of the illness and the involvement of more brain structures.

We found trihexyphenydyl, an anticholinergic drug useful in the control of akinesia and rigidity, without any adverse impact on cognitive functions. Presence of cholinergic deficits are postulated in PSP, but the usefulness of cholinesterase inhibitors is doubtful. [8] It is possible that there is no significant cholinergic deficit in PSP, at least in the early part of the illness and the use of anticholinergic drugs may be an option worth considering while managing motor symptoms of PSP.

Dementia with Lewy bodies (DLB) is yet another condition, which can present with early Parkinsonism and neuropsychiatric phenomenon like depression. Co-existence of spontaneous Parkinsonism, affective and cognitive symptoms, especially with an onset in the 5 th or 6 th decade of life, should lead to consideration of conditions like PSP and DLB. Differential diagnosis of neuropsychiatric syndromes are not easy and what could help is the knowledge of clinical neurology rather than use of investigations. [9]

  References Top

Bloise MC, Berardelli I, Roselli V, Pasquini M, Stirpe P, Colosimo C, et al. Psychiatric disturbances in patients with progressive supranuclear palsy: A case-control study. Parkinsonism Relat Disord 2014;20:965-8.  Back to cited text no. 1
Gerstenecker A, Duff K, Mast B, Litvan I, ENGENE-PSP Study Group. Behavioral abnormalities in progressive supranuclear palsy. Psychiatry Res 2013;210:1205-10.  Back to cited text no. 2
Schrag A, Sheikh S, Quinn NP, Lees AJ, Selai C, Mathias C, et al. A comparison of depression, anxiety, and health status in patients with progressive supranuclear palsy and multiple system atrophy. Mov Disord 2010;25:1077-81.  Back to cited text no. 3
Mahgoub N, Agapati R, Kalayam B. A case of an elderly woman with progressive supranuclear palsy associated with depression. J Neuropsychiatry Clin Neurosci 2014;26:E12-3.  Back to cited text no. 4
Kim WH, Lee YS, Jung SH, Choi HJ, Lee MJ, Kang MH, et al. Major depressive disorder preceding the onset of progressive supranuclear palsy. Psychiatry Investig 2009;6:112-4.  Back to cited text no. 5
Barclay CL, Duff J, Sandor P, Lang AE. Limited usefulness of electroconvulsive therapy in progressive supranuclear palsy. Neurology 1996;46:1284-6.  Back to cited text no. 6
Ahmed A, Simmons Z. Pseudobulbar affect: Prevalence and management. Ther Clin Risk Manag 2013;9:483-9.  Back to cited text no. 7
Liepelt I, Maetzler W, Blaicher HP, Gasser T, Berg D. Treatment of dementia in parkinsonian syndromes with cholinesterase inhibitors. Dement Geriatr Cogn Disord 2007;23:351-67.  Back to cited text no. 8
Nicholl DJ, Appleton JP. Clinical neurology: Why this still matters in the 21 st century. J Neurol Neurosurg Psychiatry 2015;86:229-233.  Back to cited text no. 9


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