This August 8, 2000 document on the phases/stages of progressive supranuclear palsy (PSP) was written by two caregivers who were members of the now-defunct Johns Hopkins PSP Listserv. Many neurological diseases, such as Alzheimer’s and Parkinson’s, have “stages.” So this was an attempt by two PSP caregivers to have the same guide posts for the PSP community.
The guide is copied below. (I’ve fixed a few typos.)
[Editor’s note, 2013: This website is no longer active]
Caregiver’s Guide to the Phases/Stages of Progressive Supranuclear Palsy
Compiled by Patricia Lake of Texas and Mary Holeman of Indiana
August 8, 2000
In 1999 several members of the Johns Hopkins PSP Listserv decided to pool their collective wisdom to try to see if they couldn’t categorize the symptoms associated with the normal progression of progressive supranuclear palsy enabling the defining of phases or stages of the disease.
This is the result of these efforts. It was originally posted as a message on the PSPInformation mailing list. Visitors to the PSPInformation website often tried to find the information on the website and couldn’t – so, “by popular demand” we are also publishing it here. PSPInformation.com does not claim any credit nor does it assume any responsibility for the statements made in this article. It is solely the viewpoint of the writers.
This categorizing of the symptoms of progressive supranuclear palsy is not meant to be scientific. If you are caring for someone with PSP you should realize the disease follows its own “timetable”. The progress of the disease may be different in your case. The purpose of the article is to serve as a generalized caregiver’s guideline to what to expect as the disease progresses.
We must also note that progressive supranuclear palsy is often misdiagnosed because the diagnosis is based on symptoms. Many symptoms can apply to more than one of the many diseases and conditions that are very similar and determining their real association can be very difficult if not impossible. Most patients may also be on medication and very susceptible to possible medication caused side-effects. Thus, they may develop symptoms that may be thought to be from progressive supranuclear palsy but are not.
The Phases/Stages of PSP
Phase 1 – Deterioration of handwriting and difficulty writing. Speech problems, difficulty being understood by others, slurring, etc. Coordination problems leading to unexpected falls and stumbling; change in walking rhythms/patterns. Vision problems; some complain of ‘blurred’ vision; some try prisms, but examinations by eye Dr. generally will only find normal vision physically. Lethargy, apathy, no desire to do anything; changes in sleep patterns. Cognitive problems; decrease of sound judgement, decrease in modesty; increase in impatience and irritability.
Phase 2 – Problems sitting down or getting up; cannot lower self into chair gently; just ‘plops’ down. Increased difficulty walking; begins using a cane for balance; will progress to a walker; increased number of falls. Stooped posture because of vision problems; can’t see downward easily. Problems opening or closing eyes; some patients get ‘dry eye’ because their eyes do not close all the way. Difficulty dressing; cannot do buttons or zippers; hands and fingers do not work as they used to. Almost impossible to write anything legibly. Eating problems; coughing and choking; loss of eating etiquette; fills mouth too full; lots of spills; begins wearing a bib to save clothes. Bathroom problems; difficulty voiding/unable to get to bathroom in time; constipation or diarrhea; may need help with personal hygiene. Needs help bathing; may need hand rails/bathing bench, etc. A mobile shower head is a good idea, if possible. Weakness or neglect on one side of body; one side more dominant; ie: drags left or right foot, etc. (ShyDrager Syndrome) Subject to infections; urinary tract, respiratory tract (pneumonia) etc. Alien hand; sometimes holds on to things and cannot let go or takes the hand a long time to release. Difficulty concentrating; sometimes seems ‘out of it’.
Phase 3 – Some obsessive-compulsive behavior: ie: fingers “pill rolling”, hands smoothing out imaginary wrinkles on table, etc. Increased irritability; increased impatience. May become incontinent of urine and bowel. Increased speech problems; often very difficult to understand; cannot articulate proper speech sounds. Increased eating problems; more coughing/choking. Increased cognitive problems; cannot follow stories on TV; cannot read much, due to vision; will watch game shows, some news. In Theater movies are not recommended, as some suffer from ‘sensory overload’; loud sounds and many colors, lots of movement on screen make patient ‘nervous’. sleeps much of the day, and all night, too. Instances of ‘restless leg’ syndrome. Limbs and neck may become rigid; may loose ability to support self on legs. Increased falls; some falls may be close to geing described as ‘seizures’; complete loss of control of arms and legs, with resultant fall. After fall, will sleep for an hour or so; may not always know whether is injured or not; may not ‘feel’ the injury. Increased coughing and choking; drooling becomes common; often does not close mouth; Infections may be more frequent. Requires much more help in dressing and with all activities of daily living. Does not speak much, but does enjoy seeing friends and relatives, even though patient may not respond much to them. May have pain in arms or legs; non-specific pain for no apparent reason; application of ‘heat’ rubs may help; Tylenol may also help.
Phase 4 – Unintelligible speech/mumbling; cannot say words; may go days with out saying anything. constant drooling; coughing and choking may become so severe that eating normally is impossible; Dr. may recommend feeding tube, which requires a surgical procedure to install. May have trouble opening mouth, even for meds. Increased incontinence/constipation problems. Loosing interest in daily activities; sleeps most of the time; uncomfortable sitting for any length of time; prefers bed. Cannot support self on legs; ‘spaghetti legs’; body rigid, especially neck area. Little eye movement; cannot ‘look’ at something; slow to focus on things in view. delusions, hallucinations at times; may be disoriented and not know where they are. Pain, but cannot identify the area. Withdrawn, but remains aware of people; cannot move on own; needs extensive help for all activities of daily living.
Note: These phases or categories often overlap and are not the same for all patients. Some may have two or three phase 1 problems and one phase 3 problem. Some may never have all of the problems, but most will need extensive help to live out their lives and will need to be made as comfortable as possible for the duration of their illness. It is wise to gain from the patient, specific information as to feeding tubes, restorative therapies, etc. before the disease is well advanced; a living will is a good idea, so that loved ones will know how the patient wishes to be treated, should life threatening acute problems occur. Long term care will probably be needed and plans should be made before the patient becomes unable to participate in the planning.