Workshop Compiled

Abstract

Progressive supranuclear palsy (PSP) is a type of neurodegenerative extrapyramidal syndrome. The prevalence of PSP in studies is found to be 5.8-6.5 per 100,000 individuals with a slightly male preponderence. Onset is usually between 55 and 70 years of age and median survival is around 6 years after the onset of symptomatology.It is characterized by motor symptoms, such as postural instability, rigidity, akinesia, supranuclear gaze palsy, pseudobulbar palsy; levodopa-unresponsive parkinsonism, and behavioral and cognitive symptoms. Early PSP is also difficult to differentiate from PD; as it shares many features like abnormalities in gait, speech, and eye movements. Neuropsychiatric symptoms commonly observed in patients with PSP are dementia, apathy, depression, anxiety, disorders of sleep and disinhibition.A 60 year old man presented with impaired memory, disturbed sleep and restless behaviour for 2months in geriatric mental health outpatient department. He had a history of tremors on both the hands and stiffness of body which was gradually progressive for last 4 years. They consulted a neurologist 1.5 years back as his symptoms were severe enough to hamper in day to day functioning. He was diagnosed as a case of idiopathic Parkinson’s Disease and was prescribed Tab Levodopa 100mg and Carbidopa 25mg combination preparation thrice daily. On medication within 2months, family members reported decrease in his symptoms. For last 6months new symptoms like frequent falls during walking without any precipitant developed. Falls usually occurred within a few steps of walking mostly towards the left side and following which he sustained an injury over his left eye leading to lower lid ectropion. The activity of the patient became restricted and he started to remain confined to his bed for the fear of falls since last 3months. They consulted a physician again and he was prescribed Tab Trihexyphenydyl 2mg three times a day and dose of Tab Levodopa 100mg and Carbidopa 25mg combination increased to four times a day and Tab Levodopa 100mg and Carbidopa 25mg combination contolled relase preparation was added at bedtime. After taking those medications the frequency of falls increased, forgetfulness appeared, sleep got disturbed, at night he becomes restless and some confusion in behavior like pointing things, picking bed linen were noticed during evening hours.On examination the patient had prominent vertical wrinkling of forehead, wide opened eyes with reduced blinking, ‘procerus sign’ was present. His speech revealed hypophonia and reduced fluency. On the first two days his orientation was fluctuating; from the third day no abnormality was noticed in his mental state. Neurological examination revealed wide based festinating gait, intention tremor in upper limb, lead pipe rigidity in all four limbs bilaterally(right>left), axial rigidity and impaired balance and coordination; and supranuclear gaze palsy.During the hospital stay the anticholinergic drug (trihexyphenydyl 2mg) and Tab Levodopa 100mg and Carbidopa 25mg combination contolled relase preparation was withheld; Tab Melatonin 5mg was added along with other drugs were continued. Within 2days of hospitalization, his sleep improved, restlessness and confusion in behavior decreased, also his cognition was improved.Workshop Description:Approach to Parkinson plus syndromes presenting in Geriatric Psychiatric population.Case scenarios from the literature.Discussion on diagnostic and management issues.Methods/Issues:Progressive supranuclear palsy (PSP) is often difficult to discriminate clinically from idiopathic Parkinson’s disease (PD). Diagnosis in early stage is important for maximal benefit from disease modifying treatment for PSP. Delirium is defined as an acute organic brain syndrome which presents with decreased level of consciousness, varied psychomotor activity, wake-sleep rhythm disorders, cognitive impairment and attention deficits. High dose of Levodopa in Parkinson’s disease is reported to cause confusion and psychosis.Moreover dopaminergic drug in Progressive Supranuclear Palsy has response in only 20-40% cases.Anticholinergic drugs in ageing brain with comorbidities are known to cause confusion and delirium.Learning Objectives:Parkinsonism plus syndrome as a heterogeneous degenerative neurological disorders that differs from the classical idiopathic Parkinson’s disease in certain associated clinical features, poor response to levodopa, distinctive pathological characteristics and poor prognosis. Associated clinical features include symmetrical onset, infrequent or atypical tremor, prominent rigidity in axial musculature, bradykinesia, early postural instability, supranuclear gaze palsy, early autonomic failure, pyramidal affection, cerebellar involvement, alien limb phenomenon, apraxia and significant early cognitive dysfunction in some cases. Progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and dementia with Lewy body disease (DLB) are commoner disorders. Less frequent disorders are cortico-basal ganglionic degeneration (CBGD), frontotemporal dementia with chromosome 17 (FTDP-17), Pick’s disease, parkinsonian-dementia complex of Guam, Pallidonigral degeneration, Wilson’s disease and a rigid variant of Huntington’s disease.Conclusions/ Strategies:Patients with Parkinsonism-plus syndromes represent a relatively small portion of Parkinsonism patients.They have wide spectrum of disease phenotypes associated with these disorders and the often subtle clinical differences, establishing the correct diagnosis, especially at disease onset, can be difficult despite serial clinical observations and repeated neurological examination.