Category Archives: PSP

MRI brainstem studies – 2 main PSP types and PD

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The two main types of PSP — Richardson’s syndrome and PSP-parkinsonism — are very different. I remember being in support group meetings early on, when the evidence of these two types wasn’t yet published, and wondering if the person sitting next to me was dealing with PSP at all! (“How can your parent not have cognitive impairment?”)

It’s great to have studies now that look in detail at the pathologic, radiologic, and clinical differences in these two main types. RS is easier to diagnose because it’s very different from Parkinson’s Disease or Alzheimer’s Disease; it is called “classic PSP.” PSP-P is much harder to diagnose accurately because it looks so much like Parkinson’s Disease or Multiple System Atrophy.

An Italian study was published this week that looks at MRI measurements of brainstem structures in RS (10 patients), PSP-P (10 patients), and Parkinson’s Disease (25 patients). Using certain measurements, MRIs could be used to differentiate RS from PD but the accuracy of differentiating PSP-P from PD was not as high. The authors say that the such MRI measurements “can, at least partially, contribute to the identification of patients with PSPP versus those with PD.”

The search continues for something to differentiate PSP-Parkinsonism from Parkinson’s Disease and MSA….

I’ve copied the abstract below.

Robin

Movement Disorders. 2010 Dec 15. [Epub ahead of print]

MRI measurements of brainstem structures in patients with Richardson’s syndrome, progressive supranuclear palsy-parkinsonism, and Parkinson’s disease.

Longoni G, Agosta F, Kostic VS, Stojkovic T, Pagani E, Stošic-Opincal T, Filippi M.
Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, Scientific Institute and University Ospedale San Raffaele, Milan, Italy.

Abstract
We investigated the diagnostic accuracy of brainstem MRI measurements in patients with different progressive supranuclear palsy (PSP) syndromes and Parkinson’s disease (PD).

Using 3D T1-weighted images, midbrain, and pons areas, as well as superior (SCP) and middle cerebellar peduncle (MCP) widths were measured in 10 patients with Richardson’s syndrome (PSP-RS), 10 patients with PSP-parkinsonism (PSP-P), 25 patients with PD, and 24 healthy controls.

The ratio between pons and midbrain areas (pons/midbrain), that between MCP and SCP widths (MCP/SCP), and the MR parkinsonism index ([pons/midbrain]*[MCP/SCP]) were calculated.

The pons/midbrain and the MR parkinsonism index allowed to differentiate PSP-RS from PD with high sensitivity (90%, 100%), specificity (96%, 92%), and accuracy (94%, 97%).

Only the pons/midbrain was found to distinguish PSP-P from PD, but with a lower diagnostic accuracy (sensitivity = 60%, specificity = 96%, accuracy = 86%).

Compared to PSP-RS, PSP-P experience a relatively less severe involvement of infratentorial brain.

The pons/midbrain looks as a promising measure in the differentiation of individual PSP-P from PD patients.

© 2010 Movement Disorder Society.

PubMed ID#: 21162106 (see pubmed.gov for this abstract only)

Can patient be expected to remain at home?

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I received these questions via email:

Based on what you understand about PSP, have most of the patients (whose caregivers want them to) been able to remain at home for the duration of the condition — i.e. is it possible for a PSP patient to remain in the home throughout the disease process — whether via home help, hospice and the like? I guess I’m trying to figure out for my mom — unless there is some crazy complication — it seems to me that she could remain in the home for the rest of her life — and any medical equipment that we may need down the line (oxygen, respirator?) could be equipment that we could provide in the home. (she has an excellent medical plan, so cost here is not likely an issue.)

Offhand, I can’t think of any equipment that couldn’t be provided in the home. Does this sound right to you?

(Of course, if she needed surgery, a transfusion, or chemo, or something like that, she would need to go into a medical facility….) But on the whole, from what i understand with PSP, she could, theoretically, remain in the home.

Do you hear the same?

 

The key in determining whether someone can stay at home or not is whether the patient is safe at home and whether the caregiver is safe in providing care. Sometimes it’s not possible to keep people at home. Common reasons for facility placement in PSP are:

* the patient weighs lots more than the caregiver, and the patient cannot assist with transfers;
* the patient requires 7×24 care (or monitoring), and the caregiver cannot provide this; (perhaps the patient is a fall-risk and must be monitored 7×24 for falls)
* the patient is incontinent, and the caregiver cannot deal with this situation; (perhaps because the patient wants to get up multiple times during the night)
* the patient is yelling and verbally abusive, and the caregiver cannot tolerate this;
* the patient has insomnia and often wakes up the caregiver; and
* the caregiver’s mental and physical health are compromised by providing daily care.

In our local support group, probably half of all PSP patients are in facilities receiving some level of care (assisted, skilled) or are at home receiving some level of care.

“Limited case” for recommending ChEIs in PSP

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This article reviews the use of ChEIs (Aricept, Exelon, and Razadyne) in PSP and other non-Alzheimer’s disorders. There is “only a limited case for recommending ChEIs in” PSP.
Robin

Expert Review of Neurotherapeutics. 2010 Nov;10(11):1699-705.

Cholinesterase inhibitors: beyond Alzheimer’s disease.

Larner AJ.
Walton Centre for Neurology and Neurosurgery, Liverpool, UK.

Abstract
Cholinesterase inhibitors (ChEIs) are widely licensed for the symptomatic treatment of Alzheimer’s disease, but their use has also been examined in a wide variety of neurological disorders besides Alzheimer’s disease, and this article reviews these uses.

The evidence currently available suggests that ChEIs may possibly have a role in the treatment of some patients with dementia with Lewy bodies and Parkinson’s disease dementia, but at this point in time there would seem to be only a limited case for recommending ChEIs in mild cognitive impairment, Down syndrome, progressive supranuclear palsy, pure vascular dementia, frontotemporal lobar degeneration, Huntington’s disease, multiple sclerosis, epilepsy, delirium, traumatic brain injury, sleep-related disorders or certain psychiatric disorders (e.g., schizophrenia and bipolar disorder).

Clinical practice with respect to non-Alzheimer’s disease indications for ChEIs may vary according to jurisdiction, specifically with regards to whether national guidelines effectively limit off-licence drug use.

PubMed ID#: 21046692

Excerpt from PSP/CBD Guide on Sinemet

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Several medications, all available only by prescription, can help PSP in some cases.

• Sinemet
This is the brand name for a combination of levodopa and carbidopa. Levodopa is the component that helps the disease symptoms. Carbidopa simply helps prevent the nausea that levodopa alone can cause. When levodopa came along in the late 1960’s, it was a revolutionary advance for Parkinson’s but, unfortunately, it is of only modest benefit in PSP. It can help the slowness, stiffness and balance problems of PSP to a degree, but usually not the mental, speech, visual, or swallowing difficulties. It usually loses its benefit after two or three years, but a few patients with PSP never fully lose their responsiveness to Sinemet.

Some patients with PSP require large dosages, up to 1,500 milligrams (mg.) of levodopa as Sinemet per day to see an improvement, so the dosage should be pushed to at least that level, under the close supervision of a physician, unless a benefit or intolerable side effects occur sooner. The most common side effects of Sinemet in patients with PSP are confusion, hallucinations and dizziness. These generally disappear after the drug is stopped. The most common side effect in patients with Parkinson’s disease, involuntary writhing movements “chorea” or dyskinesias) occur very rarely in PSP, even at high Sinemet dosages.

Patients with PSP should generally receive the standard Sinemet (or generic levodopa/carbidopa) preparation rather than the controlled-release (Sinemet CR or generic levodopa/carbidopa ER) form. The CR form is absorbed from the intestine into the blood slowly and can be useful for people with Parkinson’s disease who respond well to Sinemet but need to prolong the number of hours of benefit from each dose. In PSP, however, such response fluctuations almost never occur. Because Sinemet CR is sometimes absorbed very little or erratically, a poor CR response in a patient with PSP might be incorrectly blamed on the fact that the disease is usually unresponsive to the drug. Such a patient might actually respond to the standard form, which reaches the brain in a more predictable way.

A new formulation of levodopa-carbidopa is Parcopa, which dissolves under the tongue. For people with PSP who cannot swallow medication safely, this could be useful. Another approach for such patients is to crush a regular levodopa-carbidopa tablet into a food or beverage that is easily swallowed. Another new formulation of levodopa-carbidopa (called Stalevo) combines those two drugs with a third drug, entacapone, in the same tablet. The entacapone slows the rate at which dopamine is broken down. It is useful for patients with Parkinson’s whose levodopa-carbidopa works well but only for a few hours per dose. This situation rarely, if ever, occurs in PSP.