When PSP strikes

PSP, corticobasal degeneration (affecting the cortex, the brain’s outer layer and the basal ganglia, deep structures controlling movement) and multiple system atrophy (affecting the brainstem which connects the brain and spinal cord) are a group of uncommon neurodegenerative disorders. They are included in the Parkinson’s plus syndromes (also called Atypical Parkinsonism). All of these conditions are much less common than Parkinson’s disease and account for 10-15 per cent of Parkinsonism. No risk factors are known and most cases are not genetic, although some can be.

PSP begins in individuals in their late 60s to 70s. Pathologically, this shows up with shrinkage of the upper brainstem (which connects the brain to the spinal cord) and this can be seen in the MRI in an area called the midbrain.

Symptoms

The upper brainstem contains networks that are crucial to body balance and eye movement control. Thus, the earliest symptoms are recurrent, unexplained falls, often backward, and can occur in situations where balance is essential, e.g. climbing up or down stairs, getting out of a low seat, turning quickly or on uneven ground. PSP patients also seem unaware of their disability and show impulsivity, without the movement planning that is typical of older individuals.

Eye movement abnormalities also appear early and these patients have a fixed stare and may need to move the head to follow a visual target. Difficulty in looking down adds to the loss of postural righting in producing falls. Some features of Parkinsonism may also be seen such as body rigidity (often marked in the neck), slowness and difficulty in speaking and swallowing. Memory and cognitive difficulties also appear as the disease progresses.

Sleep may be disturbed and some patients may develop hallucinations.

Treatment

Standard treatment for Parkinson’s disease, i.e. levodopa, usually works for most patients for a few years and hence is always tried out. Physiotherapy also helps in mobility. The disease, however, is relentlessly progressive and most patients become substantially dependent in three to five years.

The focus then shifts to support for the family and symptom control (drooling, constipation, behavioural problems) for the patient, ideally through a palliative care team.

Once swallowing begins to get impaired, the major decision is whether to have feeding tube insertion. We prefer to have this discussion early, preferably with the patient. Some patients are clear about not having any artificial measures to prolong survival.

Advance care planning is the process by which these decisions are examined well in advance. A major component is identification of at least one but preferably three healthcare proxies (family members or caregivers) who are aware of the patient’s wishes, and can help make decisions in the best interests of the patient when he/she can no longer decide or communicate.

— The writer is consultant neurologist, PD Hinduja National Hospital, Mumbai

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