atypical parkinsonian syndromes

Atypical parkinsonian syndromes (sometimes referred to as "parkinsonian plus syndromes") and other parkinsonian-like problems

A preliminary remark

Everything I have already written and will write in the previous chapters of the series “What we should know about Parkinson's” relates only and exclusively to the typical idiopathic Parkinson's syndrome. This should be 70 or 85% with us. The origin and development of the idiopathic Parkinson's syndrome has been well researched and should be very similar in all of us.
But this chapter is about the so-called

• Atypical Parkinson's syndromes (e.g. Lewy body dementia, MSA = multiple system atrophy, CBD = corticobasal degeneration, PSP = progressive supranuclear palsy)
• Parkinson's symptoms as side effects of medication
• Diseases triggered by metabolic disorders (eg Wilson's disease).
• by arteriosclerosis and many small strokes or
• repeated head injuries (Muhammad Ali!)

The diseases that are triggered are of a completely different nature. Although they also show some of the symptoms of Parkinson's disease, they develop in a completely different way and also start in a different place.
For example, olfactory disorders and depression are typical for Parkinson's syndrome, but not for the other diseases.
For those of you who have one of these rather rare forms, much of what I have written or will write here about the typical idiopathic Parkinson's syndrome does not apply.
Repeat: the typical Parkinson's syndrome
So far we have read and learned: The typical Parkinson's syndrome, also called idiopathic Parkinson's syndrome or Parkinson's disease, runs in stages and also shows the typical symptoms in this sequence:

• Stage I according to Braak:
o olfactory disorders (sometimes, not always)
o chronic digestive problems (sometimes, not always)

• Stage II according to Braak
o Depression of unknown cause
o Disorders of the sleep-wake cycle

▪ Sleep disorders (difficulty falling asleep / sleeping through the night
▪ REM sleep disorder = RDS = REM sleep disorder: typical "acting out" of the dream phases with movements and disturbances mainly of the bed partner
• Stage III according to Braak
o Hypokinesis / bradykinesia / akinesia: This is the core symptom of Parkinson's disease!
▪ Hypokinesis = limited range of motion (for example, incomplete flexion and/or extension in one joint)
▪ Bradykinesia = all movements become slower
▪ Akinesis = some movements are no longer made at all. Quite typical is the failure of movements that are not consciously performed, but which automatically follow in healthy people:
• Lack of movement and swinging of an arm when walking
• missing gestural movements when telling stories
• Lack of facial expressions: the facial expression becomes more rigid, for example no smile. This is sometimes misinterpreted by ignorant contemporaries as unfriendly, listless or evil.
oh rigor
▪ Persistent involuntary contraction of certain muscle groups. This causes:
▪ "waxy" resistance when arms, legs, fingers, hands are moved passively
▪ "Gear phenomenon" in passive movements
▪ very common: chronic “tension” in the neck, shoulder and back area: with the misdiagnosis of “shoulder-arm syndrome”, chronic back pain, etc., many are initially under permanent orthopedic treatment.
oh tremor
▪ The tremor = shaking is NOT the main symptom of Parkinson's disease. Only the laypersons and non-affected people think that. 15-20% of all Parkinson's patients have no tremors throughout the course of the disease.
▪ The typical Parkinson's tremor is
• relatively slow, "low frequency", 4 – 6 / second
• a "resting tremor" that decreases or stops with targeted movements (in contrast to the "intention tremor", which only occurs or increases with targeted movements - e.g. fingertip on nose tip)
o Psychiatric problems: depression or anxiety disorders
o Typical is the half-sided beginning and the half-sided emphasis of the symptoms (e.g. only or mainly left arm and left leg)
o It is only at this stage that the idiopathic parkinsonian syndrome is recognized. And only in hindsight are the symptoms of stages I and II interpreted as Parkinson's-related
• Stage IV to VI according to Braak
o We do not need to repeat that in this context.
So nothing new so far.

Red flags

It sounds strange: for the vast majority of us here in this group, the "normal" typical Parkinson's syndrome is supposedly secured. But it is not. Because if one of the following symptoms occurs, you have to question the original diagnosis and still suspect an atypical Parkinson's syndrome.
Of course, if you've had a so-called typical Parkinson's syndrome for eight years, it's unlikely that you'll have to change your diagnosis now. But it is not excluded.
These symptoms, which cast doubt on the original diagnosis of typical Parkinson's syndrome, are called "red flags". In other words: watch out!
Not one-sided asymmetrical, but primarily bilateral = symmetrical beginning

▪ ➔ should always give rise to a more precise diagnosis. The "normal" Parkinson's syndrome begins on one side and remains lateral throughout the course of the disease.
Abnormalities in the "completely normal" magnetic resonance imaging of the brain

▪ ➔ The "normal" Parkinson's syndrome is typically not visible in the "normal" MRI scan.

▪ ➔ Every Parkinson's “suspect” should have an MRI as a basic diagnostic to rule out other diseases.

• ➔ Brain tumors can be ruled out with certainty (but they don't produce the typical symptoms of Parkinson's syndrome either)

• ➔ Apoplex = Stroke usually causes other symptoms, but should be ruled out

▪ ➔ So-called atypical Parkinson's syndromes are sometimes noticed by a (very knowledgeable and specialized) neuro-radiologist in the magnetic resonance tomogram if the imaging technique is good.

▪ ➔ With a completely typical picture of a completely typical Parkinson's syndrome, special imaging is not necessary:
• Ultrasound of the substantia nigra

• So-called DAT scan: is noticeable in 90 (not 100)% of typical Parkinson's patients.

▪ ➔ However, these specialized imaging procedures are necessary at the very beginning or later if (by red flags) the diagnosis of the typical Parkinson's syndrome is called into question.

Insufficient or no response to Levo-Dopa
▪ ➔ In the past, a test with apomorphine or levo-dopa was used for the initial diagnosis of normal Parkinson's syndrome: in typical Parkinson's syndrome, the symptoms of akinesia - rigidity - tremor go away to a "usual dosage" or get significantly better.

▪ ➔ Today, this test is usually no longer carried out, but begins with the typical treatment. One should be suspicious if this typical treatment is not successful in clearing away most symptoms, or if one needs equally high dosages. ➔ atypical Parkinson's syndrome?

Taking psychiatric medication
▪ ➔ This is diagnostically simple: “What to swallow Do you otherwise use tablets?” Are suspicious

• Neuroleptics (30 or 40 different drugs, sometimes with 3 different trade names): these are drugs used to treat psychoses and other psychiatric disorders.
• some anti-nausea medicines (also called anti-emetics).

• symmetrical = symptoms on both sides
▪ ➔ This would also be therapeutically simple if one could omit the pills or replace them with others. But unfortunately that is usually not possible. Please never try alone!!!!!
Thinking disorders that appear early (already in the first year of illness) – pronounced forgetfulness, disorientation, dementia
▪ ➔ Parkinson's syndrome with dementia
▪ ➔ Lewy body dementia
▪ ➔ In contrast to Alzheimer's dementia, which is much more common, these disorders are characterized by Parkinson's syndromes and often greatly fluctuating brain performance (very good hours or days alternate with bad ones).

“Autonomous disorders” occurring early (already in the first year of illness): disturbed emptying of the bladder, urinary incontinence, erectile dysfunction in men
let think of

▪ ➔ So-called MSA = multisystem atrophy, for example MSA-P = the subtype with P = Parkinson's syndrome

Balance disorders occurring early (already in the first year of illness) with an unsteady, sometimes wide-legged gait
let think of

▪ ➔ So-called MSA = multisystem atrophy, for example MSA-C = the subtype with C = cerebellum = cerebellum symptoms
Tendency to fall early, especially when walking up stairs and double vision
lets think of

▪ ➔ PSP = "Steel-Richardson-Syndrome" = progressive supranuclear (gaze) paresis

Movement disorders of the hand that appear early and "alien limb" = feeling that the hand or arm does not belong to me
let think of

▪ ➔ CBD = Corticobasal (corticobasal) degeneration

combination of
• Gait disorder: legs apart, feet sticking together
• Bladder disorders with urinary incontinence
• Thought disorder to dementia
lets think of

▪ ➔ NPH = Normal pressure hydrocephalus = a special form of hydrocephalus (expansion of the fluid spaces in the brain) - but this is immediately noticeable in every computer tomogram or magnetic resonance imaging and can therefore be ruled out for most of us)
Atypical Parkinson's symptoms and non-neurological comorbidities or underlying diseases
▪ Liver disease / - cirrhosis plus eye changes
lets think of

▪ ➔ Morbus Wilson = copper storage disease

Even more information about the atypical ones Parkinson’s syndromes can be found in this section….

So, dear ones, that would be the first half of the chapter. In the second half I want to briefly characterize the individual atypical parkinsonian syndromes.