Old 03-14-2008, 07:25 AM #1 (permalink)
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By the mid-1990s there had been more or lessa consensus view that PLS could be recognizedclinically if the other conditions were excluded, that thesyndrome was more slowly progressive than ALS itself,that it was almost never familial or associated withdementia, and that onset was after age 40. Zhai et al[30] concluded that in most patients, symptoms startedwith spastic paraplegia, but a substantial minority beganwith bulbar or upper limb symptoms. Yet some patientsdeveloped lower motor neuron signs more than 25 yearsafter symptom-onset [31] and some authoritiesconcluded that PLS was merely another form of ALS(in parallel with the view that PMA is a form of ALS)[32-34]. There was another problem. The evidence forthe ”consensus” view of PLS was provided by only 22autopsies that had anteceded recognition of theimportance of Bunina and ubiquitinated inclusions. By the time of the Siddique meeting in June 2004,there had been 5 reports of postmortem examinationsthat included search for the inclusions [35]. All camefrom Japan and in all cases the major disability wascaused by upper motor neuron disorder. Two had PLSplus parkinsonism and two had PLS with dementia,all atypical. Only one of the six had no inclusions andsome had visible but not prominent fasciculations. Ifthe inclusions define ALS, then those patients hadResearch advances in ALSPathologyTau-positive inclusionsA. Pick disease: tau (+) argyrophilic neuronal inclusions. B. FTDP-17: tau (+) neuronal and astrocytic inclusions with tau mutations. C. Corticobasal degeneration: tau (+) astrocytic plaques,ballooned achromatic neurons. D. Argyrophilic grain disease: tau (+) inclusions react with silver stains. Tau-negative inclusions1. FTD-MND: tau (-), ubiquitin (+) inclusions in dentate,brainstem motor nuclei. 2. Dementia lacking distinctive histology. Clinical featuresA. Frontal, behavioral type: personality change, disinhibition, loss of empathy, eating disorders, ritual or stereotypic behavior, apathy.B. Primary progressive aphasia; fluent (temporal pathology,semantic dementia) or non-fluent (primarily frontal pathology,perisylvian). C. Either (A) or (B) with motor neuron disease. D. Overlap with corticobasal degeneration. Table 4. Clinicopathological classification of frontotemporal dementia fromHodges et al [19]
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Neurologia i Neurochirurgia Polska 2005; 39, 16Lewis P. RowlandALS, not PLS. Even the one case lacking inclusionscannot be taken as defining PLS because finding theseelusive structures depends on how many neuronsremain and how patient the pathologist can be inlooking for sparse structures. Nevertheless, 3 of the5 cases lived more than 7 years and prospective studiesare needed to determine whether UMN-dominantALS progresses more slowly than more typical ALS.(In ”UMN-dominant ALS”, corticospinal signs areevident and LMN signs are restricted to fasciculationin the tongue without major bulbar symptoms or smallmuscles of the hand without functional handicap). 4. Alsin and other genes affect definitions of ALS,PLS, and hereditary spastic paraplegiaIn 1990, Ben Hamida et al [36], in Tunisia,described three then-new forms of familial motorneuron disease in adolescents: juvenile ALS with bothupper and lower motor neuron signs; spastic paraplegiawith peroneal muscular atrophy; and a spastic syndromewith pseudobulbar symptoms. Some cases were familial,others sporadic. Then, Gascon et al [37] describedwhat they called ”childhood PLS with gaze paresis”;onset was in late infancy. The Tunisian investigatorssoon teamed with Pericak-Vance at Duke to map theautosomal recessive ALS type 2 to 2q33-q35 [38]. In2001, two teams of investigators [39,40] identified thegene as alsin, a quinine nucleotide exchange factor.Remarkably, the same mutations caused ALS2 (withcombined UMN and LMN signs) and also purelyUMN syndromes of PLS.
Primary Lateral Sclerosis (PLS) is defined by the presence of UMN without LMN signs. Throughout the clinical course some patients may never develop LMN signs. Often, however, LMN signs do eventually develop. If so the name is changed to UMN onset ALS. There is little doubt that the early appearance of prominent lower motor neuron (LMN) signs in a patient previously found to have a pure upper motor neuron (UMN) syndrome is a poor prognostic factor. The question still remains when, if ever, to draw a line between UMN-predominant ALS and PLS. Thus we would like to know whether analysis of the data acquired from periodic clinical and electrophysiological testing of patients who initially presented with an UMN syndrome (eventually diagnosed as having either PLS or ALS) allows an approximation of "time to LMN involvement"

i will try to find more
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Old 03-14-2008, 07:50 AM #2 (permalink)
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P99 THE CLINICAL MANIFESTATIONS OF PRIMARYLATERAL SCLEROSISGordon PH, Pinto M, Mitsumoto H, Kaufmann P, Rowland LPColumbia University, New York, USAE-mail address for correspondence: phg8@columbia.eduBackground: Primary lateral sclerosis (PLS), considered a form ofmotor neuron disease (MND), can be definitely diagnosed only byautopsy. Clinical diagnostic criteria have been proposed, but thereis no consensus except that there should be only upper motorneuron (UMN) signs on examination. Some patients, however,have minor denervation by EMG or subtle lower motor neuron(LMN) findings on examination, and some patients convert to ALSafter many years. Different clinical subtypes may have differentrates of progression, creating a spectrum between the two syn-dromes.Objective: To describe the clinical features of PLS, and to define‘UMN-dominant ALS’.Methods: We reviewed our database of MND patients and retro-spectively reviewed the records of 60 patients given the initial clin-ical diagnosis of possible PLS in the years 1984-2004. On thebasis of clinical features we defined clinical diagnostic subgroups.Results: Of the 60 patients, the diagnosis remained PLS in 34 fol-lowing initial evaluation. Excluded diagnoses included El EscorialALS, myelitis, multiple sclerosis, dystonia and stroke. Of the 34PLS patients, 17 were males and 17 were females, with average ageat symptom-onset of 56 years. Nineteen first noted symptoms inthe legs, four in the arms and 11 in the bulbar region. Fifteen hadsymmetric onset, and 15 had an ascending pattern of progression.Twelve patients eventually needed a cane, and four became non-ambulatory after an average of 66 months. Eighteen patients devel-oped dysphagia, but none had gastrostomy. Seventeen patientsdeveloped emotional lability, 14 had urinary dysfunction, and 2had cognitive deterioration. Eight patients had a family history ofanother neurodegenerative disease. Six of 34 had signal changes inthe corticospinal tract on MRI scan. Sixteen of 17 had abnormaltranscranial magnetic stimulation with prolonged central conduc-tion times, and 16 of 22 had abnormal magnetic resonance spec-troscopy with NAA/Cr ratio less than 2.5. The average diseaseduration at time of follow-up was 51 months. Thirty-three patientshad pure UMN disease at the initial evaluation and one hadminor LMN signs, defined as limited to 1–2 muscles in oneregion. Seven patients, all with predominantly UMN symptoms,developed minor LMN signs over an average follow-up of sixyears, and one died after three years.Conclusion: These data confirm that clinically diagnosed PLS hasa more benign course than ALS. A minority of cases progress to‘UMN-dominant ALS’, which may represent a spectrum of diseasebetween classical ALS and PLS. Large-scale, prospective studies ofincident cases are needed to determine disease frequency and prog-nosis based on clinical subtype, including clinically diagnosed PLS,PLS with minor denervation by EMG, and UMN-dominant ALS.Further definition of clinical subtypes may facilitate study ofetiologies and prognosis, and lead to eventual treatment trials.
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Old 03-14-2008, 08:40 AM #3 (permalink)
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Personally Olly i look for the Conclusions part of these studies as because the rest of this crap is BS . You can make a statement but if your talking in a foreign Language nobody can say what your saying is false or if you talk in circles your not really saying anything .So when all the Jabber is done what is the Conclusionary Statement . Geo (ps ) i know of a Doctor who gets paid to go and make these speeches
and people actually applaud ,and you ask them why they applauded they say "well eveyone else is "
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Old 03-14-2008, 08:58 AM #4 (permalink)
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LOL geo i was trying to find data / stastistic studies for hopingforacure.
alot of it is over the head lol, i did erase alot out but if you erase all of the over the head stuff your left with one sentence and no proof of final statement.
sorry i'll try to find some simple reading lol
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Old 03-14-2008, 03:37 PM #5 (permalink)
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Caroline,
Thank you so much for the info, I feel much smarter now, and believe it or not, I read it enough to understand it all. I come up with PLS is about 50/50 to turn to ALS, talk about a crap shoot. Thank you for all your research, I think a cure to pls will cure or slow down als...
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Old 03-16-2008, 01:48 PM #6 (permalink)
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One major characteristic used to identify a lower motor neurone lesion is flaccid paralysis. This is in contrast to a upper motor neurone lesion, which often presents with spastic paralysis.

The axons of lower motor neurons are a type of motor fibers. Lower motor neurons are classified based on the type of muscle fiber they innervate:

Alpha motor neurons (α-MNs) innervate extrafusal muscle fibers, the most numerous type of muscle fiber and the one most involved in muscle contraction.
Paralysis is one of the most pronounced effects of damage to α-MNs. Because α-MNs provide the only voluntary innervation to extrafusal muscle fibers, losing α-MNs effectively severs the connection between the brainstem and spinal cord and the muscles they innervate. Without this connection, voluntary and involuntary (reflex) muscle control is impossible. Voluntary muscle control is lost because α-MNs relay voluntary signals from upper motor neurons to muscle fibers. Loss of involuntary control results from interruption of reflex circuits such as the tonic stretch reflex. A consequence of reflex interruption is that muscle tone is reduced, resulting in flaccid paresis. Another consequence is the depression of deep tendon reflexes, causing hyporeflexia.

i found this it is very worrying, what do you think
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Old 03-16-2008, 08:09 PM #7 (permalink)
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Hi Caroline,
I am so grateful for all of your research. I am concerned if you are having some of the flacidity issues, lmn.. Maybe you are just so spastic that it seems the opposite. I am thinking about your appt. on Thursday, and will be waiting to hear as soon as you return. I am so so hoping for good news, you have been in my thoughts.. Hoping
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Old 03-17-2008, 05:33 AM #8 (permalink)
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i did find that an acute umn lesion can cause flacidity but it would be a whole limb and it would return to a spastic state, mine is certain muscles and left lower leg but the flacidity is getting worse. my appt is at leeds university hospital, it's the best university teaching hospital in the country so i'm hopefull for more definate answers.
when i get some news i will make sure your the first to know. i really appreciate your support and concern it means alot to me, i hope i can do the same for you, just let me know if you need any help or support with anything.
i understand your problems with the jaw, the bulbar symptoms have" knocked me for six", which is what usually happens if your not prepared that is why a definate diagnosed is important then at least you know what to exspect.
i hope you are ok and not feeling too bad
take care
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Old 03-18-2008, 08:08 AM #9 (permalink)
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They speak of Lesions . Well if the MRI is clean NO LESIONS Hmmmmm . then they look at you like your from outer space . All my clinical workup was normal for the most part ,other than High Cholesterol ,Gerd ,Hyatial Hernia ,DJD of the Neck ,Psoriasis ,Sebhoreic Dermatitis. They said we are not sure what you have for sure but you'll problably not die from it . And Yes that will be $40,000 Compliments of Mayo Clinic . I still get a Newsletter from them though . Geo
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Old 03-18-2008, 12:00 PM #10 (permalink)
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Caroline and George,
You guys are something else. I am with you all the way. This thing is so crazy.
Caroline I will be thinking of you all day on Thursday, I am so glad you are going to a good hospital, that in itself is hard. Like Geo said they treat us like we are MARTIANS.
Just wish we could all live in one area together, maybe then we could find a cure, and of course it would be the only place in the world that would really understand MND and diagnosed. and living with them. This is a tough tough road we have to travel. Hang in guys, we all know what the others are going through.. Your Friend Hoping
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Old 03-18-2008, 01:12 PM #11 (permalink)
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as you both have pseudo bulbar problems i wanted to ask you if you have a feeling in your throat like you are being strangled. it feels like someone has got hold of my adams apple and is throttling it. i've noticed this before but not thought anything of it. but now with the bulbar symptoms i wondered if it is related. probably throat spasms? i did'nt know if i should mention it on thursday along with the other bulbar problems.
i have to say the clonus/spasms in my face and jaw have calmed down a little, just typical that is when you've got an appointment, then after it it gets bad again, "sods law" lol. my son said on thursday morning chew some gum for an hour to get them going,it's a good idea but a bit torturess!.
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Old 03-18-2008, 07:12 PM #12 (permalink)
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The closeness of the throat is part of having this disease. Like i said its like a pipe that is closing in ,when the muscles tighten they make the throat feel like its closing in . I think drinking some water helps. just a little . but try pinching your nose and blowing with your mouth closed ,i think this inflates the throat just a little or pushes it out . Chewing gum makes more saliva which in turn has to be swallowed . Research says even if you think about eating ,your body makes saliva . I was told we swallow every 2 minutes even when we sleep . I was also told get this , Parkinsons Patients do not have tremor when they sleep . Go figure . I dont know of any PLS sleep issues No body has ever written about not sleeping good . My self i only wake once about 3 -4 then go right back to sleep . If you have a chewing issue like chomping down on your cheek like i did , take some food and park it between the teeth and the cheek which puffs out the cheek so the teeth dont get it . If swallowing is an issue try this think about swallowing on your good side . Mine is my left side if im having a swallowing issue when im swallowing like liquids i try to move them to my left side of my mouth then it seems to work better .Or i use My TENS unit for an hour or so on my throat . Geo
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Old 03-18-2008, 10:36 PM #13 (permalink)
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Yep guys, the throat spasms and spasicity can do a real number on the ever closing throat, and the clonus of the jaw well that is just the icing on the cake.
I like George's ideas for us, and will try them. Darn biting of the cheek is a fun one also, I bite my cheek so much it now has a long scar. And the biting of my lips, and the out of line jaw, oh what a fun fun time..
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Old 03-19-2008, 05:43 AM #14 (permalink)
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thankyou both of you for your replys, sometimes you get a symptom and you think is that related? am i being paranoid?.
geo, they are good tips i will definatly try them.
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Old 03-19-2008, 07:40 AM #15 (permalink)
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In the beginning i even think i made a permanant scar in my mouth ,which made a callus ,But my lip gets it too .These seem to happen when i get cocky and think im having a good day i start moving and chewing faster, then it hits ,i'll chomp down on my lip or cheeks ,so ive learned to slow down . Emotions play a huge role here as ive found ,if i get upset my diaphram wont open fast enough to get speech out and i run out of air ,so ive learned to stay calm and move slow . I think whatever we have affects the Emotions as well as the Motor Cortex . I cant watch Mooooshy Movies or i'll get teary eyed .Never happened before .They did a Psyhc Evaluation at Mayo and i came out fine . So im not a Psyhco Case . Geo
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