Third generation neurologist Alexander Pantelyat was moved to join the family profession partially because of his fascination with Parkinson’s disease, or PD. By just listening to patients and doing a physical exam — much of which can even be done over video — Pantelyat estimates that doctors can correctly diagnose PD up to 90 percent of the time depending on disease duration.

“Not that many diseases are diagnosed that way anymore,” he says.

But it’s the outlying minority with PD symptoms who don’t have this disorder who continue to fascinate Pantelyat. He currently directs the Johns Hopkins Atypical Parkinsonism Center, one of only a handful of clinics around the world dedicated to treating this rare population of patients.

Atypical Parkinsonian disorders — the clinical manifestations of which are referred to as atypical parkinsonism — encompass a group of four syndromes that have features in common with PD: progressive supranuclear palsy, corticobasal syndrome, multiple system atrophy and dementia with Lewy bodies. Patients who develop one of these conditions often share the tremors, stiffness and bradykinesia characteristic of “regular” PD. However, while PD usually progresses slowly over a period of many years, those with atypical parkinsonism have an accelerated timeline, with symptoms arising and progressing over months to a few years. These patients often have significant cognitive problems, which develop more slowly, if at all, in typical PD. Also, while Parkinson’s patients usually respond well to levodopa or dopamine agonists, these drugs don’t work — or become ineffective over time — in patients with atypical Parkinsonian disorders.

The clinic that Pantelyat directs sees patients with these atypical disorders once a month at Johns Hopkins Bayview Medical Center. Though there is no cure for these conditions and no truly effective symptomatic therapies to date, says Pantelyat, he and his colleagues offer a patient-centered, multidisciplinary model of care that helps patients extend their functionality and limit progression for as long as possible. At each visit, patients meet with him and nurse coordinator Becky Dunlop, along with experienced physical therapists, occupational therapists, and speech and swallow therapists.

The visits also give Pantelyat and his team a valuable chance to enroll willing patients in research studies that can help diagnose and differentiate the disorders, which currently can’t be definitively diagnosed until autopsy. His clinic is developing several different studies to better understand these conditions, including one using MRI to look for minute structures in the brain that are affected in these conditions and taking blood and other biofluid samples to search for biomarkers unique to these disorders. Treatment trials for some are also on the horizon.

Pantelyat’s goal is that, in time, better understanding and diagnostic techniques will lead to the first truly effective therapeutics.