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Thread: PLS Day at the Oxford MND Centre

  1. #1
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    PLS Day at the Oxford MND Centre

    The Oxford MND Centre is holding the first UK PLS Day on Friday 23rd October 2015 at the Oxford Spires Four Pillars Hotel, OX1 4PS

    A dedicated day for Primary Lateral Sclerosis patients, carers, and healthcare professionals. Expert talks on the history, clinical features, pathology, optimal management, and research into PLS, plus patient Q&A session.

    Wheelchair-accessible venue
    Easy parking
    Refreshments and lunch provided
    FREE for PLS patients (and one accompanying carer)
    All others £75
    Sponsored by the MND Association
    To register, please email hjsconference@btinternet.com

    Provisional programme

    0930-1000 Arrival and registration
    1000-1100 Is PLS a form of ALS or something different? Historical, clinical, histopathology
    1100-1130 Refreshments
    1130-1200 Research: MRI, MEG and genetics
    1200-1230 Patient Q&A on biology and research
    1230-1400 Lunch
    1400-1445 Guest lecture: Dr Mary-Kay Floeter, NIH, USA
    1445-1530 Refreshments
    1530-1615 Management of symptoms
    1615-1700 Patient Q&A on management

  2. #2
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    sounds very good hope they go round the country

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    Forum Member Barry52's Avatar
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    I have registered to attend. Please let me know if anyone else is going.
    Iím going to do this even if it kills me!

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    Barry:

    I am going. prof. Chris Shaw refined my diagnosis to 'predominantly PLS'. He sees PLS/ALS on a spectrum as opposed to distinct entities.

    I shall seek you out there and we can compare 'clinical notes' so to speak.

    Regards

    Charles

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    I hope to be there

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    Forum Member Barry52's Avatar
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    That's great Charles. Be good to put a face to the name.

    Barry
    Iím going to do this even if it kills me!

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    Forum Member Barry52's Avatar
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    And of course you Miranda. Posts crossed.

    Barry
    Iím going to do this even if it kills me!

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    Hi Guys,

    Here is something I prepared earlier.... (Valerie Singletonesk)



    NOTES ON PLS

    AN OVERVIEW

    Primary Lateral Scelorsis, PLS, is the name given to MND affecting the Upper Motor Neurones, UMNs.

    The UMNs nucleus reside in the motor cortex that locates towards the rear of the frontal lobe. This translates to more or less the top of the brain. Distinct regions of the brain control distinct muscle groups of the body. A diagram showing which areas of the motor cortex that control particular muscle group/appendages is given on the build-uk site. The nucleii of the UMNs reside in the brain's grey matter.

    The UMNs are controlled by the cerebral cortex to perform the high functions, for example, writing, walking, chewing. Interfacing the UMNs and cerebral cortex are neurones that control combinations of motor neurones that perform the complex tasks. A case was documented where a gentleman's arm would always move to open a door as the door came within range of the arm despite him not wanting to move through the door. This demonstrated how the motor neurone interface functions with control inputs from the eyes only initiating movement in this example. The cerebral control input was lost.

    There are over 600 muscle groups in the human body.

    The skull case may be removed and electrical stimulation of distinct parts of the motor cortex will illicit movement in the various muscle groups, for example causing a hand to grasp or a face to smile.

    The anatomy of motor neurones is well documented on wikipedia. The salient features are a nucleus with projections/tails from off the neucleus that connect to other neurones or direct to a muscle. The projections to the muscles or LMNs are up to 1 metre long and 1/10 the diameter of a human hair. The projections organise into groups (tracts) that are the white matter of the brain.

    Motor neurones do not have programmed regeneration cycle (apoptosis) within the body''s lifespan. Motor neurones take 15 years to fully mature.

    A type of glial (support) cell named oligodendrocyte supports the motor neurone by encasing the motor neurone projections in myelin. Myelin insulates the motor neurone projections both physically and electrically. Oligodendrocytes also support the mono-carboxylate transport 1 (MCT-1) system that nourish the neurone.

    Another type of glial cell is the astrocyte. These cells act as the resident immune system and patrol the motor neurones by touching the motor neurones. Chemical messages are sensed by the astrocyte to determine the health of the motor neurone. When the astrocytes senses the motor neurone is stressed, immune responses are generated including inflammation, anti-body and nerve growth responses.

    There are two methods of intra neurone communication, chemical and electrical. Different neurone cell types use different chemicals for signalling.

    The UMNs of the motor cortex project into the central region of the brain that is called the basal ganglia region. There are two distinct projections from the UMNs named the cortico-spinal tract and the cortico-bulbar tract. The cortico-spinal tract contains the UMNs' projections that control the limbs and trunk muscles. The cortico-bulbar tract contains the UMNs' projections that control the tongue, pallette, lips, throat, epyglotis (food flap), larynx muscles.

    The basal ganglia is the closed-loop control system of the human machine that collates feedback from the muscle groups, by means of the peripheral nervous system, to define the exact muscle movement demand from the UMNs of the motor cortex.

    There are Lower Motor Neurones, LMNs making the voluntary control decisions for muscles in the basal ganglia (mid brain) such as coughing, sneezing, breathing, pain reflexes, blinking, bowel and stomach movement, throat regurgitation, saliva production, yawning, hiccups.

    The UMNs and LMNs interact where they both have need to exert control over the same muscle group, for example, the eyelids. The LMNs control the blink, UMNs control sleep and shut-eye movements.

    UPPER MOTOR NEURON DISEASES

    Pure PLS

    This version of MND is rare, affecting only 2% of people with MND. A definative diagnosis of pure PLS takes a minimum of four years. The reason why it takes so long for a diagnosis of pure PLS is because it takes a minimum of four years for atypical symptoms NOT to appear.

    A mild dementia is typically associated with pure PLS.

    Riluzole not effective.


    Predominantly UMN ALS

    This version of MND is characterised by symptoms typical of PLS in the early stages of the disease, then symptoms of ALS as the disease progresses. It is commonly called PLS turning to ALS.

    Limb onset is typical. Slowness of movement, spasticity (stiffness) and some weakness of muscle strength will be noticed. Briskness of reflexes will become apparent. Walking gait will change as specific muscle groups weaken and often foot-drop is an early indicator of leg involvement. Over years muscle weakness will spread throughout much of the body, but what distinguishes this disease from pure PLS is that muscles will waste during the progression of the disease.
    Emotional lability will often develop in the early stages of the disease.The physical emotional responses will become more exaggerated, resulting in quickening to cry and laugh.
    Severe fatigue will develop as the disease progresses.
    Dementia is not a factor in this version of MND.
    Bulbar symptoms will develop. Speech will start to slur initially as muscle control slows. Eating and swallowing becomes a slow process and coughing when eating will become necessary to clear food that has penetrated and is interfering with the epiglottis. Coughing remains strong as the LMNs remain functional. The development of the bulbar symptoms typically takes years.
    The essential LMN functions remain unaffected. Breathing will continue to operate for many years and maybe decades.

    MRI scans reveal high signal change in the cortico-spinal tracts and cortico-bulbar tracts. As the disease progresses the degeneration of the neurone projections will travel down the spinal cord, impacting the LMNs. Lumbar Puncture results will offer oligoclonal bands suggesting demyelination, however not the classical bands associated with multiple sclerosis. Nerve conduction studies reveal slowing of signal conduction through the neurone projections. Anti-bodies will be present in the Lumbar Puncture sample during the early phase of the disease.

    It is reasonable to assume that the oligodendrocytes are the root cause of this version of MND. All the symptoms of demyelination of the UMNs are present backed up by clinical tests consistent with demyelination.

    The potential treatments could be:
    Stem cell treatment for remyeliination of the neurones (see Multiple Sclerosis websites)
    BrainStorm neurotrophic factor treatment.
    Neuralstem neurone replacement treatment.

    Riluzole not effective.

    References:
    Oligodendrocytes, Wikipedia
    Last edited by Graham; 4th July 2015 at 17:15.

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    Hi Graham:

    Were you diagnosed PLS? Are you going to plan on attending the day?

    harles

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    Hi Charles,

    I was first diagnosed with Primary Progressive Multiple Sclerosis, then Primary Lateral Sclerosis and finally Amyotrophic Lateral Sclerosis. However having researched my condition extensively, I have classified myself as Predominantly Upper Motor Neurone Disease.

    I now believe that my condition is genetic as my uncle had Primary Supernuclear Palsy and my father Parkinson's Disease. A wide variety of neurones on my father's side degenerate probably as a result of a faulty service mechanism.

    I expect that I won't be able to attend but in the age of Broadband it shouldn't be a major problem.

    Regards

    Graham

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