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 Myoclonus and LBD 
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Joined: Fri Aug 11, 2006 1:46 pm
Posts: 4811
Location: SF Bay Area (Northern CA)
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Post Myoclonus and LBD
I'm interested in all Atypical Parkinsonism disorders because they are often misdiagnosed for each other. I read an article tonight on myoclonus in PD and PD-related disorders, including LBD. Myoclonus = sudden, involuntary muscle jerks often caused by sudden muscle contractions.

I found fascinating what the article had to say about the similarity between LBD and CJD.

After reading this article, I wonder if the presence of myoclonus, the type of myoclonus, and the interval of tremor-like myoclonus can be useful in diagnosing which Atypical Parkinsonism disorder people have!?

In summary:

MSA: "Okuma et al. recently (48% of) MSA-p patients and (18% of) MSA-c patients. The condition was mainly observed in the distal part of the arm." "Rest tremor (a classic symptom of PD) is only present in its most common form in 10% of MSA cases."

CBD: Myoclonus "is observed in 50% of CBD cases. The condition is predominantly distal but can extend to the entire arm. More rarely, myoclonus is present at the distal extremities of the legs. Its association with distal dystonia, a parkinsonian syndrome, apraxia and asymmetrical sensory function is very evocative of CBD. The...myoclonus tends to repeat at an interval of 70­90 ms, and thus resembles tremor."

PSP: Myoclonus "is very rarely observed in PSP."

LBD: "Myoclonus is observed in between 15% and 20% of cases of Lewy body dementia. Rest myoclonus is more frequent than in PD. However, action myoclonus is observed more often than reflex myoclonus. The condition appears more frequently at night and in the very late stages of the disease, when it is often accompanied by generalized, convulsive attacks."

Here's the article's citation and abstract:

Myoclonus and extrapyramidal diseases

Neurophysiol Clin. 2006 September - December;36(5-6):319-325.
Available online 13 December 2006

L. Defebvre
Department of Neurology and Movement Disorders, Lille University Medical Centre, France

"Parkinsonism or dystonia are associated with myoclonus in several extrapyramidal diseases. Although the latter symptom is not always prominent, stimulus-sensitive, distal, or focal reflex myoclonus is frequently observed. This review will consider the clinical and electrophysiological features of myoclonus in Parkinson’s disease, multiple system atrophy, corticobasal degeneration, progressive supranuclear palsy, Huntington’s disease, dentatorubral-
pallidoluysian atrophy, Lewy body dementia, and myoclonus with dystonia. The evidence of a long-latency reflex response, the presence of giant somatosensory evoked potentials, and the demonstration of a back-averaged premyoclonus focal cortical EEG activity often lead to classify
myoclonus as a cortical phenomenon. However, a subcortical origin cannot always be ruled out."

PubMed ID: 17336776

Here are three definitions:

Extrapyramidal diseases are disorders of the basal ganglia, which is the part of the brain that controls movement. In such disorders there is a disruption in the nerves and muscles controlling movement and coordination.

"Distal refers to sites located away from a reference point. The hand is distal to the shoulder. The thumb is distal to the wrist. Usually, that reference point is the center or midline of the body." (MedlinePlus)

EMG = electromyographic. An electromyograph is "an instrument that converts the electrical activity associated with functioning skeletal muscle into a visual record or into sound and has been used to diagnose neuromuscular disorders and in biofeedback training." (MedlinePlus)

Here are some excerpts:

"Whenever confronted with a case of extrapyramidal disease,
the medical practitioner should systematically seek
to identify myoclonus, whose evidence should prompt suspicion
of an atypical parkinsonian syndrome, particularly multiple
system atrophy (MSA) or corticobasal degeneration

"Analysis of its electrophysiological features often leads
to classify myoclonus as a cortical phenomenon...
When rhythmic and of a low-amplitude, myoclonus can
sometimes be clinically misidentified as tremor, which
represents the main differential diagnosis. Electrophysiological
analysis then enables the practitioner to differentiate
both symptoms."

"In this review, we will successively present the clinical
and electrophysiological characteristics of myoclonus
observed in extrapyramidal diseases, where the condition
is combined with a parkinsonian syndrome or dystonia."

Parkinson's Disease
"In Parkinson’s disease (PD), myoclonus is usually induced by
L-DOPA; it is often observed during sleep, with less common
occurrence during “on” periods."

"Myoclonus is observed in between 15% and 20% of cases of
Lewy body dementia. Rest myoclonus is more frequent than
in PD. However, action myoclonus is observed more often
than reflex myoclonus. The condition appears more frequently
at night and in the very late stages of the disease,
when it is often accompanied by generalized, convulsive
attacks. An iatrogenic origin can probably be ruled out,
since myoclonus is observed during “off L-DOPA” periods.
The condition is not characterized by rhythmic EEG activity.
Back-averaging techniques confirmed a cortical origin, with
the absence of a LLRR and giant SEPs. Set against a
background of progressive dementia, myoclonus can suggest
a diagnosis of Creutzfeldt­Jakob disease, especially
when the topography of the condition is diffuse. Even
though spongiform lesions of the temporal lobe have been
observed in a few pathologically-proven LBD cases (two out
of the 30 patients in the series reported by Burckhardt et
al.), the condition’s slow progression and the absence of
ataxia and periodic EEG activities generally enables a diagnosis
of Creutzfeldt­Jakob disease to be ruled out. In the
event of diagnostic doubt, analysis of the cerebrospinal
fluid and of EEG and MRI (hypersignals in the basal ganglia)
should be performed systematically. In some occasions, a
definite diagnosis can only be made after anatomopathological

Sat Mar 10, 2007 4:03 am

Joined: Thu Aug 30, 2007 6:15 pm
Posts: 231
Location: Charlotte, NC
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I have been noticing that my LO has been doing alot more jerking lately. He used to get violent jerks but now they seem to be milder but alot more frequent. He has them both when awake and asleep. I have asked if he is in pain, but he really cannot tell me anything anymore. I do know that they used to actually snap his head and he said that did hurt. These are not rythmic. I am guessing that it is the progression of the disease as he is experiencing swallowing issues as well. Anyone else experiencing this?

Sun Aug 09, 2009 10:00 pm

Joined: Fri Aug 11, 2006 1:46 pm
Posts: 4811
Location: SF Bay Area (Northern CA)
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I doubt the presence or increase in frequency of myoclonic jerks has any correlation with disease progression. Dysphagia, yes. Myoclonus, no. Just my layperson opinion...

Sun Aug 09, 2009 11:36 pm

Joined: Fri Oct 16, 2009 10:37 pm
Posts: 6
Location: Arkansas
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Post Myoclonus

This particular symptom of LBD is extremely important to me, due to the fact that my Dad has suffered from this for some time. He went to many doctors, neurologists, and no one could ever give him or Mom any reasons for the muscle spasms.

There is no telling when my Dad first started having LBD because he has had memory problems, muscle spasms, tremors in his hands and a preoccupation with sex movies. I even found 2 old bottles of Viagra and Cialis. My Mom kept a lot of the this to herself and I wouldn't have found out about his hallucinations if she hadn't slipped and told me.

She said my Dad said, "Did you see that little girl just walk by?" She said,"no." it was probably just a shadow. She has a problem with denial and through the years has done this with any critical situation.

When I asked Dad if he sees people that no one else sees, he said, "Yeah, they stand next to me all the time." I asked him what they were doing, and he said, "just looking at me." It was at this time we went to the doctor and started this journey.

He has had night sweats for a while and I suppose my Dad never mentioned this to his doctor. We live in a rural area so the GP's around here don't know a lot about LBD. I plan to go with my Dad when he goes back to see his neurologist.

Thank you for this forum. It is so reassuring to be able to vent and discuss simular problems/solutions. Nanikj

Sat Oct 17, 2009 8:15 pm

Joined: Thu May 14, 2009 11:19 pm
Posts: 28
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Post My DH Definitely Had Myclonus
He has had major jerking, lightening-like jerks, focusing mostly on his neck but also including, at times, full body jerks. Risperdal seems to help reduce the jerks, but competes with Flomax (which is needed to help my DH urinate). My DH is on Seroquel. Even so, he still has involuntary jerks. The doctors didn't believe me when I said he was having the jerks at night during his sleep. Now they believe me. Now I know that the movement at night is a MAJOR sign of REM Sleep Disorder associated with LBD. It took FOREVER to get the LBD diagnosis, but at least we have it now.

May God hold you in the palm of His hand and shelter you in the light of His love.

Fri Oct 23, 2009 5:22 pm
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