This video guide to medical professionals for diagnosing progressive supranuclear palsy (PSP) is well worth watching. Note that multiple system atrophy (MSA) is mentioned (around 12:30) as well as corticobasal degeneration (CBD) (around 12:40) along with a description of alien limb syndrome.
This 18-minute diagnostic video, “A Physician’s Guide to PSP,” was announced a few months ago by CurePSP, the new name for the Society of PSP. You can order the video on a DVD but it’s easier to find it online here:
[Editor’s note: the above link was updated in 2012 when CurePSP moved video off its website on to YouTube.]I found the program understandable to a layperson.
Dr. John Steele, who was one of the MDs who defined the disease PSP, is the first speaker. There’s some interesting video of supranuclear gaze palsy. I guess so much time is spent on this because downward gaze palsy is a hallmark symptom. You probably don’t need a dictionary because the terms used (eg, apraxia, spastic) are described in video form.
Dr. David Williams is the second speaker. He is one of the leading stars of PSP research and practices in Melbourne, Australia. (He used to work with Dr. Andrew Lees in the UK.) He talks about brain structures affected by PSP and the neuropathology of PSP. (Don’t worry – this doesn’t last long!)
After Dr. Williams, we hear the voice of Dr. John Steele who says:
- “A population of 100,000 could contain 1-2 diagnosed PSP cases and 4-5 undiagnosed”
- “This is about 5% of the prevalence of Parkinson’s Disease”
- “The average survival in PSP is 6-8 years after symptom onset”
- “This is about 2 years less than for Parkinson’s Disease before the L-dopa era”
Dr. Lawrence Golbe is the third speaker. He indicates that the facial expression, gait, and speech of PSP are unusual. He says that the speech of PSPers (spastic and ataxic) is distinctive and occurs “in almost no other condition.” He says that one of the diagnostic criteria for PSP is falls occurring within the first year. (I’m going to have to look up again what other neurologists say about that. My dad did have falls this early but I didn’t think everyone did.) He always orders a brain MRI. He sometimes orders a SPECT.
The fourth speaker, Dr. Andrew Lees, notes that the arm swing is often preserved in PSPers. This is unusual and dissimilar from Parkinson’s Disease (PD). He talks about blepharospasm. He says that eye movements form an important part of the neurological exam. An eye exam is shown.
Dr. Lees notes that as part of taking a patient’s history, it’s important to ask about previous history of encephalitis. History of visual hallucinations, psychosis, and memory loss would indicate the patient may have Dementia with Lewy Bodies. As part of the physical exam, it’s important to exclude autonomic dysfunction. Symptoms of autonomic dysfunction are more commonly associated with PD and MSA. Dr. Lees says that CBD should be considered if there is marked asymmetry of symptoms (such as jerky, tremulous movements in one limb) or alien limb.
The video is also intended for families. It was produced by CurePSP and the PSP Society of Europe, hence the combination of US and UK researchers.
Here’s a description of the video from the CurePSP website:
“NEW! A Physician’s Guide to PSP – Diagnostic DVD – An important resource for neurologists, family physicians, and medical professionals created by the PSP Europe Association in the United Kingdom and CurePSP in the United States. This 18 minutes long video features commentary by top neurologists specializing in PSP, including Lawrence I. Golbe, MD, John C. Steele, MD, and Andrew Lees.”