Cerebral Palsy maybe/maybe not

Cerebral Palsy - a different way to view it - with a long venting preface:

There is no need to apologize for a new way to think about cerebral palsy simply because this whole topic is a shambles born in assumption that became dogma by default. And yet, the underpinnings of what will be presented here were pretty much spelled out by Claude Shannon at

Bell Labs in the 1930s and built upon by Weaver & Nyquist who pulled Shannon's concept into a variety of practical applications. Such as: telephone ( including present cellphones) radio text messaging video … and just about any formulation of the transfer of knowing or INTENTION from one place or person to another. Hither to thither has rules and limits. Can we bypass Shannon's concept and still transfer thought: YES!! We can. On a tree, carve Alex loves Lisa inside a Valentine heart -. Cut the tree down and drag it behind a tractor to where you want the world to know 1) that you, Alex, 2)love Lisa … AND that 3) you are, in doing this, an absolute moron. In this example we have a person identified (Alex) who has an affinity for another person (Lisa) and an action. The first two we call SIGNAL [what to relay]. Beyond the ‘how’ everything else is irrelevant and called noise. Noise? The how, dragging, may well have scraped away some or all of the original carving. Is it somebody named lex loves Li? Or sh oves Li? Or wanted, as in Ovid's Metamorphosis, to imply that Lisa had turned into a tree? We can measure our confusion by polling how many interpretations are possible from what arrived at the destination. Metadata is that the sender is a moron having not considered the weaknesses

of the chosen communication choice and cost relative to clarity. But morons abound. The arrogant ones object if you say a simple thing and then repeat yourself, reiterate, rephrase in different terms. Or, the listener requests, “In other words..??? What?” Language has so many terms & phrases that can change meaning in CONTEXT. Repetition of thought rephrased & spiced with cliche are NOT faults but rather redundancies that help FILTER out possible misinterpretations of what is intended. The filtering is in the listener's head. The system allows redundancy to assist the listener to make interpretive corrections on the fly. So, along come pretend scientists who frown on any redundancy in scientific writing and make “scientific” articles the most unreadable format in creation. Although CLEAR speech is normally 30% redundant, scientific (sounding) speech is barren of this fundamental of clarity - of communication and of knowing – or at the least suspecting. Error is very hard to spot. Bad filtering. Who controls the language wins the argument. How do you win with a faulty argument? By planting land mines in your opponents language. Make every clear concept into a not to be used word or phrase or equivalence a phrase to an existing excommunication. Confine observation description to approved parcels of literature approved description terms and measures. Result? Current literature lags about ten years behind active practitioners, or worse. It is no wonder that the really big contributions to

understanding reality have come from persons outside of the established fields. Requiring only specific formats of idea conveyance is an overt attack on lucky revelation. Gamesmanship posed as science. How do you publish observations that cannot be clearly described nor measured in the current accepted format of nomenclature ? How many articles get printed in a journal? How thick is a journal? What if a definitive

study, conducted over an extensive period with many clarifying alterations generates more than 400 pages of summary data? Who publishes that ? Where does that get presented? What if it only makes sense in light of something else, something seemingly unrelated and so taken to be unimportant? This book is NOT about creating new dogma. It is simply an attempt to grab you by the head from behind and turn your direction of gaze away from published jargon [bedeviling sources] toward actual visually overt things that defy presentation to those reactionary guardians of what is be allowed to be read and only in allowed terms and approved measures. Peers or censors? We will start disassembling what you currently KNOW [can quote articles] and strip you naked of surety and do that from the ground up. This does NOT dismiss good science. It just requires the ability to reformulate what “results” actually means. Often the answer is simply a formulation of a better question to ask.

Science is nice, but it can be like the oracle of Delphi and confuse us if we are too skewed in how we interpret what we are actually seeing and not color it with personal biases that color the language we use. Accepted classifications bias even seeing and certainly telling. Ground up. Start with no particular biases. We are NOT here as mathematicians, nor scientists. We are engineers. We are not looking for fact deluge, but insights to fixing FUNCTION. Start with inventory. Look at actions that do work. What is common to what works? Is what works conditional on any CONTEXT?

OK, take this on faith (for now) and you will be ahead in spotting misleading information. Whenever you want to find out something about something [NO EXCEPTIONS], you must first decide what is a reasonable scope of expectation.

This is the RULE OF REASONABLE EXPECTATION !

Before you study anything, or get ready to hear any study, have a clear idea of what could be possible, even at the edge of broad experience. Why? Because that dictates what is required of the tool used to measure whatever happens. You won't measure the temperature on Jupiter by licking your finger and pointing at Jupiter. Trekking in Antarctica? Bring a candy thermometer? Measure varus of the hind foot? Is a lateral x-ray of the foot able to even approximate a clinically meaningful deviation? No, it isn’t. And yet those 'results' were reported. How tight a measurement is needed to change the way shoes are made or reinforced. If the x-ray sensitivity can barely detect a change of 30 degrees, is THAT enough to make clinical declarations? Is it even ethical to radiate 1000 children with x-rays looking for that totally insensitive answer? That happened. Almost every organized human endeavor that measures things or studies things uses this rule. Unfortunately, it is given a different name in nearly every context or field of application. We already hinted at where this is going. There is SIGNAL (within the scope of what we want). There is NOISE, that which makes signal hard to see or detect or which bedevils our detection tools.

Here’s an example where common sense & reasonable expectation & assumption prevents flawed conclusions.

A heavy smooth steel ball is rolled down a nicely made smooth incline. A camera is used to video the ball’s acceleration and travel. Data (x & y timed locations of ball center), frame by frame, are plotted. A jagged graph emerges. Jagged? A JAGGED graph? From that ? Some unusual gravity thing going on? Forget the speed. What could make that ball so inertially insignificant so as to do anything in a non-smooth, jagged, way? What kind of ball is this???? Or maybe the camera was not secured properly and was jiggling. Look below. This is a computer generated knee angle curve of a normal person walking on a gymnasium floor with a HAND HELD camera recording the walk. White line. Do we suspect the camera person of having a tremor?

Actually, computed perfect data, was passed through a program to mimic possible recorder vibration. The results of this are quite visually obvious . But our inertial facts allow us a better computation of what happened, a far

more probable graph of reality seen as the red curve. So, fudged smooth data is closer to reality than the actual data? Yes, in this case. The beginning assumption had not included camera wobble as contributing to what was measured. That is the big error. Rule of reasonable expectation quickly focuses on “Did anybody secure the camera mount?” So what did Shannon, Weaver, & Nyquist teach us? How fast to sample with our best tool, and how good does the tool have to be to not wash away truth. How do we see truth in a bath of noise? This is basic engineering. There is another trap. We have a sequential [array] of measurements of something which we can measure with accuracy & precision [not the same] – say – knee angle at points in time. A nice horizontal time line and a vertical scope to show the amount of angle of the measured thing (hip- knee-ankle). Whoa. Did we come here to bathe in numbers devoid of relevance? Do you have a firm correlation of degree of 'knee angle' with what the leg is accomplishing? Are all angle deviations of angle scope equivalent in effect? What if there were no knee, but instead a telescoping rod from hip to ankle that was made (magic?) to deliver the ankle/foot position exactly as it is was When using the knee? We would measure its LENGTH. Also we would measure verticality relative to the ground, and its distal horizontal 'vector' (momentary speed of the foot in the direction toward the goal), and how much the shortening telescoping of the Hip-Ankle rod passing through verticality allowed clearance at mid swing (all that was needed, or just some, so look elsewhere for more). Measurements vs measurements. AHA! Report that!! What? The angle seen along the time line!

Actual example:

How much change in length of the invisible (but actually the functional aspect) line between the hip to ankle? Isn't that just a 1:1 correlation? No. The knee angular changes graphed are “periodic” as it is a rotation effect and not a point to point measurement. Also the functional implication depends on the angle which that linear but invisible Hip-Ankle line makes to the GROUND. Ahhhh. The ground . We have all heard of 'the ground'. We walk on THAT. So, the invisible (and nearly always unmentioned nor measured) hip to ankle line is also a spatial vector which becomes a functional measurement when tracked relative to the ground . It measures what the knee accomplishes .

But it is also a scalar , in that the size of the person (3 y/o to 19 y/o ?) matters as to whether the measurement is USEFUL relative to the task at hand... step over a 3” tall rock. Measure the vertical HA line in statures and utility or insufficiency screams at us. FUNCTION!

This is why we are not pretend scientists (wearing butcher's frocks) excited by statistical relevance devoid of functional utility. We are engineers. We want relevance. We want relevance in CONTEXT. We want contexts that assist function or thwart dysfunction.

If you are a journal editor and right now have blood issuing from your ears... that's a good thing. We can measure length in inches & in centimeters. But also in STATURES ! (1 stature = standing height). That allows comparing things as they grow – real change? Or, just the same thing – but bigger. Bottom up. Let's go. Hip Ankle Relative Position HARP line

What is the bottom? Shannon's pamphlet on the limitations of communication (log base 2) which must allow for the bandwidths of the information transfer mechanisms. Two hands clapping. Send & receive. One hand can't be wearing a thick mitten (noise). It is the birth of signal to noise consideration in design. It turns out this is OLD . VERY OLD. Very very very – well maybe back to some sponges evolving. Any biologic mechanism that detects or allows reaction to the environment has this built in . ABSOLUTELY. Frogs? Yup. Birds? For sure.

How about... ...those microscopic thingies, living in pond water, with those tentacle looking fingers? Them too. Think Darwin. It wastes energy to have vague functional communication at any biologic scale. ENERGY !!! Tight communication pushes the mechanisms of communication ( & control) right to the quantum energy mechanical limits. It is THAT tight. Human walking is insanely energy efficient. Maintaining energy conservation is a goal of many key functions. Waste not, want not.

The way to useful answers is asking the right questions.

Bottom up. Start in the dirt.

Go read ‘Mud’. We will wait for you, here.Let’s start our discussion of cerebral palsy with one really important recognition. Now this varies from place to place, but, about 30% (easily) of patients seen in ‘CP’ clinics – do not have cerebral palsy as is defined by the books and medical articles and diagnostic codes.

Oh, and this:

About 30% (varies a lot) of patients who are treated in cerebral palsy clinics do not have cerebral palsy.

We have to stay planted in reality. This is a very practical thing. You just can’t have a separate place to go for every diagnosis. Patients in cp clinics have cp or wink wink cp . When ‘other’ or ‘unknown’ is used as a diagnosis, it is hell on earth and massively costly for getting obvious needs approved and paid for. Even though diagnostically different, they often require many of the same needs and interventions.

Cerebral Palsy is what, then?

In the womb, during or after being born, something happened that is no longer ongoing. The brain was affected and depending on what areas or functions – we might see something amiss – or not. Babies don’t do much. A missing arithmetic skill won’t get noticed. Brain cells don’t all do things we can see. Much of the brain cell mass is SUPPORTIVE. The supportive cells mechanically help position and support neurons, true. But, they even more importantly make many of the substances that neurons need to be and act as neurons and to sustain their own structure. Neuron damage may be a failure of neurons – over time – from the missing supportive cells. A very obvious example is when, to big applause, a kid drags somebody out of a garage that has a car running. That somebody is ‘ saved ’. But then, over a year to even two years, we see odd tremors that increase in scope and amplitude until it is full blown uncontrollable movement. The carbon monoxide made oxygen unavailable by taking up all the O2 receptors in the red cell hemoglobin. The most oxygen needy cells regardless of spatial location or tracts are damaged. Those are the ones making stuff, not just synaptic-ally holding hands with other neurons.

Brains use tremendous amounts of energy. Yet, the synapses, where all that magic happens, are way too crowded with recycling loops of complexity and such that there isn’t enough room for mitochondria. So, they don’t rely on local mitochondria (where O2 is used to make energy and lots of stuff). The glial cells have

space. They – in a sense – feed the neurons as energy rich stuff is made upstream and then passed down the axons to the synapses super charged and ready to go when triggered.

It is very possible that the amount of energy demanding packets for certain tasks is more than can be generated in real time. So, there has to be both surplus reservoirs to hold reserves and inactive periods to catch up – yawn… zzzzzzzzz

General Terms You Hear in Cerebral Palsy Clinics

Monoplegia Paraplegia Triplegia Quadriplegia - words that pretend that the speaker can count to four - in Greek. These terrible words just won't go away and are typically sought by insurers as if they actually had medical meaning. They don't. Hemiplegia - one side of the body involved. The opposite side of the brain probably lost flow to the middle cerebral artery by any of several means. Plugged by a clot? Hemorrhage can also do this.

Typical of hemiplegia from damage to the brain region served by the middle cerebral artery, or one of its branches, has more dense involvement of the arm than of the leg as the hand and arm representation on the brain surface is in an area with less cross flow from other vessels. Sensory and motor are in this arterial flow zone so sensory loss in the arm can be severe. From knee down the brain region is in the area between the hemispheres where anterior and posterior arteries have some contribution. Thus hands worse than feet.

Children with four limbs involved with the uppers more than the lowers in a pattern of elbow & wrist flexion and leg extension are often called bilateral hemiplegia (rather than quadriplegia) to emphasize this pattern of kind of involvement rather than just a limb count. In effect, both middle cerebral arteries were compromised or bilateral hemorrhages occurred. Sudden low blood flow might involve the middle cerebral territory most as there is least cross flow from other vessels (at full term, this is not so earlier in development). It would seem that the brain surface, anticipating birth shearing of the skull, drops cross connecting vessels that were feeding the brain surface. Quadriplegia - in the context of this list this term is OK, but not best - It implies a very generalized event, such as anoxia better called TOTAL BODY as the head and neck are involved (and not excluded just because they are not limbs). Besides, this term is already spoken for. Spinal cord injury may use this term (correctly), but seldom is that so with CP. Total body CP?

There can be good blood flow with poor oxygenation such as is caused by placental disconnection. The child's heart pumps but the blood gets no oxygen from mother. CO2 also builds, as does acidosis. Damage is to those brain areas that are most sensitive to oxygen deficit and to acidosis is a

double whammy. The base of brain, populated with many ‘nuclei’ [cells of a feather flocking together] is particularly hurt by oxygen lack and CO2 poisoning. These high metabolism structures are also the most eleptogenic when damaged .. seizure prone.

Cerebellum and other areas are also involved. Generally, involvement manifests very severely in the legs with a different pattern of arm paralysis than seen in adult stroke. Again, a better term is Total Body, as children seldom have head and neck spared.

Diplegia - Spastic Diplegia is nearly 1/3 of actual cases of CP and most commonly related to prematurity. <===Ventricles in BLUE Internal capsule in RED to/from spinal cord The brain shows a change called peri-ventricular leukomalacia (PVL). This translates as clear whitish soft spots (necrosis) scattered near the ventricles. Why there? In preemies, the brain surface just on the shore line of the fluid filled ventricles has least blood vessel access as no vessels bridge the ventricles. A low-flow pressure event will manifest here.

The PVL worst area is near where tracts representing the lower legs are passing by. The tracts from the body form a boomerang shape in cross section horizontally. That shape carries certain nerve fibers away from low flow danger.

As the flow drop worsens, the damage spread will bring in wider representation of the body and even a bit from the hands. Nestled in the hollow of the boomerang, however, are other structures which are associative. As hands get more involved (the scope of injury larger) the chances that these associative areas will also suffer, increases. For that reason, in this group there is an association between more hand involvement and mild learning difficulties. The more severe PVL cases can have considerable hand and arm involvement, perhaps only on one side. Again a flow issue so it can vary with local vascularity unlike O2 debt which does not respect spatial boundaries.

PVL four limb involvement is unlike that seen in total body involvement as the kind OF neurological injury is different and where it resides is different.

PVL is a consequence of insufficient flow pressure AND prematurity. Why? Well, the fetal brain surface is covered with many many arteries & veins not seen in the full term baby. The basic brain is being built. If blood pressure drops & flow of blood slows then the brain surface is kind of protected by that abundance of vessels and can survive low pressure while other places suffer. Why low flow? Born too soon. The heart has a big hole in it for placenta based flow as does the aorta (ductus arteriosus). These will get a mechanism to quickly narrow & close up when birth starts, but not now, not yet.

The one wide arcing area that is at highest risk is adjacent the ventricles (= blue in right illustration).

That is shore front property. There is no blood flow crossing that ocean. Here all the vessels are kind of dead ends. Low pressure leaves this tissue wanting. Spotty bits undergo necrosis (croak) and at autopsy look like white (leuko) soft spots (malacia) in the peri ventricular tissues, hence PVL. In horizontal cross section (exempli gratia in MRI) this zone looks like a boomerang with the apex pointing to the mid-line.

This apex has the least flow and so determines the most frequent consequences. The foot- leg-hip tracts radiate away from this center. Trunk to hands are further from this arcing zone center. When you see hand involvement, the spread isn’t just longer along the boomerang region. The boomerang damage itself is thicker, especially spreading out at the apex. This then approaches hand areas and ‘ganglia’ which bring in some dystonic qualities – especially in the hands – less so in the legs. The cortex is still spared unless those extra vessels rupture (hemorrhage).

Now it gets really interesting. Go take a vitamin. You’ll need it. We must grapple with information theory & control. With this we need to consider that there are two functional ‘do it’ pathways, pyramidal and extra-pyramidal. The pyramidal pathway is the one you thought you already knew. The brain to muscle informational path (actually in cats, it isn’t). In most mammals and especially humans it is the telegraph line, hither to thither, with two transmission relays en route. So like pony express usually three horses to get the mail from here to there.

BRAIN nerve=======> New nerve=====> Last nerve===> muscle.

But is this 100% true? It is my theory, more a question, [based on behavior and on the edge uncertainties of histology sections wherein not all nerve fibers stain, but most do – just loose packing?] are there less repeated bits included? BRAIN nerve=======> New nerve=====> Last nerve===> muscle. BRAIN nerve=====================> Last nerve===> muscle. BRAIN nerve=======> New nerve=====> Last nerve===> muscle. BRAIN nerve=================================> muscle. BRAIN nerve=======> New nerve=====> Last nerve===> muscle.

This might not make neat-freaks happy, but it is good engineering in that a few faster arrivals let the target ease into its chore, not BANG! We do know that the extra-pyramidal

signals which are ALSO sent are sent a wee bit ahead. They adjust the receptors. If the result does not match the expectation, the spindles send ‘correction’ signals directly (to spinal cord built-in patterns- to muscle) so as to correct the difference..

This is the underpinning of EXPECTATION as a major determinant of muscular activity. The brain isn’t sending massive environmental and structural information. It sends only CHANGE so much from what is current. It has an expectation as to what that change will do, but it takes too long to send back a “How’szat boss?” message. Instead, the extra-pyramidal mechanism resets the receptors (spindles) to a state of neutrality with the way things will be - IF - it it goes as planned. If good, then no news means it is as expected. What next? To do this or that has a very wide range of subtlety possible, even unique stuff bound for the circus or instrument mastery, horse riding, cliff climbing, river wading and so on. Anything goes. But expectation needs PRACTICE.

Building a library of experience expectation is what childhood and play is all about. It takes time to get skill and make the difficult familiar. Actual physical stuff has to be swapped or added to what is there. But cats? Here’s our back door which is their front door. You can send no signal to the muscles at all but rather just send the intended result to

the spindles which then do it all. They, via a very rich built-in INHERITED cat library, execute it PERFECTLY. Fast and precise but only what was put In their spinal cords via DNA…built in. You can’t teach old dogs new tricks? Hmm? OK. You can’t teach any cats any tricks. “CAT-like” We kind of knew this already, behavior wise. This is the underpinning. These patterned movements are many but aimed squarely at hunting and survival and FAST. People do not have this incredible library of total body automatic moves. But we do have some. Our collection is used to supply obviously needed postural changes to support ongoing intended actions. An arm extended backward as balance for the forward reaching hand? Leg shift? Both?

But when the main pyramidal do-it path is not doing it?

Thus in many cases of neurological impairment we see things being done in a very patterned way – cat-like? – not so well without a rich array of complex choices. Extra pyramidal is now taking over. Endless therapy to teach spinal what by brain would otherwise be easily taught. So, now we return to spasticity wherein the spindles unset are way too sensitive (“high gain”) to acceleration. With unexpected acceleration the unset spindles blast out signal floods. If you tap a muscle or even give it a passive quick stretch, not only does a muscle react, but other body parts jump or twist. “Cross reflexes” are movements elicited mechanically or by stimulation from afar. They cross our mental boundaries, down to up or this side of the body to the other side… whatever. A troubled right hip might be driven by a left irritable source, so beware of sidedness, it can fool you.

But now consider, we have seen that there is a second way to do things, via spindles and patterned response. Though crude it is, at least, available.

This leaves us now to consider spasticity in parallel (spastic diplegia for example). Intact direct control paths with spindle hyper-response layered on.

Here we see immediacy of effort, hemmed in by dysfunctional reaction (contracture which is BOTH tissue based and that from abnormal confounding reflex). When in postures that relieve tension, they move well. Crawling can look quite normal. W-sitting can allow arms to relax. The pyramidal system which orchestrates, draws on inertia, inner ear balance, toe sensation, general position sense, and more. All is still there, but hitting walls. It is a string quartet playing beneath machine gun fire.

These kids have the ‘moves’. You can sense their skill of movement choices or attempts. They seek mobility .

But, spasticity in series (mediated by spindle controlled movement) is thick, slower to handle the unexpected, and maddeningly doing its own thing that isn’t quite the best choice. It is movement direct from a movement plan, not adapting with any speed using sensory factors. They seek stability . They need to do a conscious thing, then confirm that what they wanted to do was actually done. In a walker they bend forward to create an inherently large base (as a pyramid). But, they also LOOK at their feet. Some can not ‘walk’ if you hold a file folder under their chin (not touching (such that it blocks their vision of their feet). They will have heavy foot landings that make confirmatory sound or drag firmly to make the step. They are not ‘walking’. They are ‘stepping’. Each step is a self contained event. They need islands of stability to ambulate.

This is the basis of our two main ambulatory treatment groups.

In group 1, the dynamic group, both surgery and bracing seek making and keeping the transition points fluid and not destructive of momentum and energy. A walk has dd/dt as a straight line. In group 2, stability seeking, both surgery and bracing are aimed at allowing stable stance (even some comforting back knee if not slamming), but not so stable as to require a complex release to take the next step. Some trial & error with shims in the shoe heels is common to find how much stability is too much. We don’t want a collapsing leg.

But there is a sub group, wherein the motor control is actually OK when tested seated performing moves as requested by the examiner. But devoid of sensory control, they just freeze up. Canes, something to touch (couches, walls, chairs on wheels, lawn mower toys) often results in magical

appearance of what was thought to be absent motor control.

Sensors that generate signals to make round the chest belt vibrations can be used to increase knowing. Making AFOs flexible & thin and designed to increase skin sensation as they deform can unfreeze these kids. Thick rigid AFOs remove more sensory cues and make them worse. Sensory types tend to improve over time if you can keep them going. They are rewiring new neural pathways the way the blind do wonders with sound.

The spasticity in spastic diplegia tends to be the purest, if free of the mid brain kinds of tone abnormalities. The range of motion loss is worse with rapid testing than with slow movement testing. Circuits most involved are those that interact with accelerations – SPINDLES.

We will beat this topic into oblivion in another book on our book shelf..

Intermission So much of this is difficult to accept all at once and especially when it is so seemingly off mainstream thinking. Depends on whose stream you are wading in. <== This is a recent publication (MIT Press) that is NOT about CP. It is about biologic information handling with a slight leaning toward visual systems, but overall quite general. It details energy needs of circuits. Guess what? All this stuff we were assuming to be the reality is, in fact, solid. Rock solid.

If you are in any field of biology, read this book. It is quite readable.

The sections on mitochondria and their role and limitations in complex synaptic structures is worth the whole book.

Go to the bathroom ...

OK! Back to work!

Triplegia – It is hard to know what this term means. Most of the time it is spastic diplegia + hemiplegia. That is, periventricular leukomalacia (diplegia) plus a stroke – more likely focal hemorrhage or intra-ventricular hemorrhage. If you are using this term thinking it is actually something, then most of your ideas for what to do will go up in smoke. In one side we have mostly diplegia type findings but some extension stiffness stirred in. Dystonia - Certain mid brain structures when faulted will leave a condition not too unlike Parkinson's disease, as they often involve some of the same anatomy. Thickness of movement and zigzag patterns of successive joints occurs. Stiffness across joints feels uniform and unrelated to speed of testing. It is very apt to change quality and form when sensation picks up noxious change (loud noise, earache, tooth ache, bladder infection, finger injury etc.). Passively move one body part (example: head) and other parts alter their Posture typically into familiar named forms such as ‘fencing’.

Symmetric and asymmetric tonic neck reflexes are well known. But, even experienced practitioners will leave out of the list the nastiest of all – wind swept legs (one side adducts as the other abducts). Both legs headed in same direction. It is a huge destroyer of hips. It is amplified in scope and magnitude of force by sensory input. What is felt as pain in us may not be felt as pain but instead manifests as dystonic posturing. Over time distended colon causes hip dislocation. It does so often - ‘Poop hip’.

One patient was strangling herself with her right arm locked down tight behind her neck. It was massively forceful beyond anybody’s ability to dislodge it. The plan was have an orthopedist break the arm (in a nice way?) to allow intubation. She is quite purple. Injecting local anesthetic into her crooked left middle finger (which she fractured on a plunging communication board hit), ended the medical emergency as the right arm simply on its own came to her right side, relaxed. Another dystonia feature – maddening – is that because the origin is from high mid-brain, it isn’t experienced as an imposed abnormality. It is felt to be choice. ‘Dystonic patients will often seek abnormal postures against treatments that thwart them. They kind of live in curved space. Rigidity - is another kind of stiffness wherein, again, the stiffness of one joint is related to the posture of the next joint. Thus an ankle very rigidly pointed downward at an extreme and feeling like stone, suddenly points upward when the knee is flexed. Likewise the hip responds to knee position or the reverse. We see thrust or withdraw patterns to these associations which stem from the mid-brain primitive circuits intact without higher level inhibition. But these are not zigzag and if correction pressure is manually applied and held, the rigidity will suddenly just melt away. Athetosis - is an oscillating rhythmic movement of the limbs often associated with extremes of twisting facial movements, though not always. A very specific area in the brain (basal ganglia) does this when injured. It can be injured by high bilirubin (jaundice) levels. Today that is rare, although we have seen it increase in the wake of drive by birthing, as the detection skills at home may be wanting.

It was a complication of parental Rh Factor incompatibility before the newer preventative measures were made standard procedure. It is reliably prevented. So, beware! Today, athetoid cerebral palsy probably occurs in the full term infant who undergoes a brief but profound anoxic event, implicating a brain region of high oxygen need. Athetosis is seldom manifest before two years after birth. This makes sense as a similar disorder is seen in carbon monoxide poisoning. Interestingly, the movement disorder of CO poisoning shows up many months after everybody has celebrated the good fortune of surviving. Babies with athetoid cerebral palsy often do not show the disorder until one to two years. They may be described as a bit floppy at birth, however. The thinking is that the damage may be to supportive cells that are woven like lace through the brain. Absent these cells, the unattended neuron elements then wither, late. Beware that most of what is written about athetoid as being this or that is just blindly repeated from old texts that were written before the bilirubin mess was fixed. Today’s athetosis isn’t what it was with yellow babies. Today we are far more likely to see bits of athetoid quality mixed with rigidity and spasticity. Whereas undoing athetoid movement is really not available (short of brain surgery), spasticity that aggravates athetoid reaction can be dealt with to reduce athetoid extremes.

Slowing neuron signal trains like this /\/\/\/\ to this __/\______/\______ helps.

Ethanol perineural injections that bugger myelin is used effectively to put speed bumps on those nerve paths which are eliciting aggravated athetoid activity.

Neurotransmitter abnormalities or medication problems

Neurons must divide to increase their number then sprout long branching filaments which must migrate among billions of other neurons to form associative connections. The chemistry used by a neuron has a pedigree. So the chemistry of one path (green) is not the same as nearby paths traveling the same general winding way

(blue & red & black in the illustration).

Substances that affect the minds of adults do so, usually, by neurochemistry.

Alcohol affects neurochemistry. Anything that affects neurochemistry might also affect the step-wise processes of brain formation : cell division, migration, connection and also myelination (a special coating to make signal conduction speed faster). Maternal shared alcohol ruins baby brains. Cocaine does it in most subtle but scary ways (impulsive children blind of consequences). Low thyroid function, when uncorrected, guarantees profoundly low IQ. When you list the really bad causes of developmental brain conditions we find many can be prevented. Genetics is finding really subtle abnormalities that impair brain function.

Terminology & Reality:

It is difficult to discuss this subject without first having yet another snit about customary medical terminology. The official categorization of neurological injury which is deeply ingrained into medical "codes" (computer classifications for insurers) is based on the ability to count to four. If a person with neurological injury has only one limb impaired that is monoplegia; two limbs impaired, diplegia; three limbs is triplegia. Four is quadriplegia. Four limbs with body and head is total body. But what USEFUL information does that convey? This classification is worse than useless. It is annoying. It tends to only characterize these further by what the person can not do. Like a ‘guido’ on the corner whistling at girls and rating them from 1 to 10 the cp rating is less crude but only rates to 5! It is rating what patients can’t do without major consideration as to why. Self rewarding outcomes are nudged as these set the limits of outcome targets (future levels of support that will be needed). Aside from the fact that the limbs are not the site of injury but just taking orders as sent, the terminology homogenizes many causes. Imagine if we only had the word tree for that collection of upright plants. So much for ash, elm, oak, maple, catalpa, evergreen, spruce, balsam, birch, tulip, aspen, fir, cypress, juniper, larch, tamarack, pine, cedar, beech, chestnut, eucalyptus, hickory, walnut, sycamore, ailanthus, magnolia, olive, fig, ficus, plane, palm, willow, locust, sequoia, redwood, poplar, acacia, cottonwood, beech, box elder, apple, crabapple, redbud, mulberry, cherry, peach, plum, pear, prune, banyan, baobab, bamboo, abba, calabra, betel, mahogany, ebony, bo, ironwood, dogwood, ginko, bottle, or bonsai, burned, worm ridden, moss covered or whatever. Mono, di, tri, quad. That's it? But it is worse than insufficient. It is misleading, very misleading.

SPASTIC DIPLEGIA

Spastic diplegia is about 30% or more of actual cerebral palsy cases. The descriptions imply things that just are not true.

Let’s dissect this. Getting description right requires getting perception of what you are seeing right. Spastic diplegia may be the greatest optical illusion on Earth! Had everybody fooled – including me. So, diplegia ("two limbs weak") has come to mean that which results from tiny speckled white infarcts (or small dead spots) scattered just outside the periphery of the ventricles of the brain ( whether two limbs are involved or not ). In microscopy lingo that description becomes periventricular leukomalacia (PVL). Peri= around, ventricular=relative to ventricles (fluid cisterns in the brain), leuko = white or clear colored, malacia= oooy gooey or mush, or pathologically "soft". The premature brain has a network of extra blood vessels that deliver blood to the brain surface or cortex. This is protective of the cortex when flow pressure drops for any reason. The least served area for low flow is adjacent the ventricles as that is a fluid region devoid of solid structures. Adjacent the ventricles (periventricular) is bay side property. When the winds of trouble blow in the preemie, the bay side property gets hit most. When PVL speckled damage is confined to a small region, manifestation of malfunction may well be in the legs only. Perhaps, just the ankles. Maybe just a trace of ankle reflex sensitivity not even noticed clinically at all. Typical PVL can be one sided in which case the diplegia is really only one leg ( hemi-diplegia ).

As the scope of these small PVL injuries scatters further out in a larger radius into the periventricular brain suburbs, hands and arms may also be involved

somewhat. Even so, we still don't call it quadriplegia.

The peculiar pattern and quality of muscular usage is what is important. PVL or "diplegia" conveys that. So diplegia - forget that di means two - may well have four limbs involved. The dominant manifestation is legs AND in a certain way. There is a unique quality to the way diplegia function is impaired. Lately, even parents are referring to their children as having PVL rather than diplegia. Good. That tosses out the old misleading nomenclature. I like that. PVL is a very common neurological complication in preemies. One third of all CP is prematurity related and thus have the PVL type of neurological based involvement. The classic look of spastic diplegia (PVL) is inward rotation crouch. The typical functional limitation is via speed related recruitment of unasked for muscular activation (speed related recruitment of additional muscle activity is called spasticity). Anything else which is called spasticity is called that WRONGLY!!!! It is an important distinction. Kids with high levels of spasticity may have their control mechanisms totally intact, but, overloaded by extraneous stuff. Sensory mechanisms are usually working. Remember that prematurity is, itself, a complication of something else. There can be incompetent cervix or twin / triplet issues. Prematurity may be brought on by genetic problems within the child's genome causing late spontaneous abortion. The earlier the prematurity, the more likely an underlying embryological or fetal cause is also present.

Nit picking? No. Don't get blinded by statistics. Statistics do not cause. They report.

You can simply report that last year 0.001% of the population got run down by trucks (I made that number up) - and/or - you can advise that folks don't stand in highways. Percent relatedness of CP to prematurity is a batting average. The idea is to avoid being a statistic. Look to causation. Maybe the first truck just brushed you, but a bigger wider one is bearing down? Turns out that of the 0.001% who are hit by trucks, 80% are run over by six more following cars. Ouch. That's the point. When brushed by a truck, don't stand there giving the trucker the finger. Move! Fortunately, outside the ventricles the brain has a superhighway of up and down going motor and sensory pathways made mostly of cell projections and not the cell bodies themselves. Conduits. The motor lanes that involve the legs are most central. As you go from the feet upward, neurological input paths layer on top and thus are further from center. Geometry figures in diplegia. The range of severity in diplegia correlates with the concentric layering on of motor pathways as you travel up the spinal cord and to the brain past the ventricles. Closest to the ventricles are the projections from the foot and ankle. Furthest away are those from the neck. So, just foot involvement means a small radius of damage. Hand or neck involvement implies a larger radius of damage which may well overlap other kinds of structures. Other kinds? Closest are the basal ganglia. Damage can generate inappropriate rigidness and/or dystonic signals (described below). Because the cortex is spared in spastic diplegia (PVL), intention and knowing of movement is intact. What was actually damaged were tracts that inhibit (tune) many of the spindles which, by default, sets them high. The connections For normalcy of intent & action are there. I am guessing that some of those more direct neurons (not 3 to a run but 2 or 1) get damaged with the neurons

heading to the spindles. That would cause a small % of muscle fibers to be denervated. Denervated muscle involution replaces muscle units with scar. That could account for the long bands of non-growing fiber that seems to gravitate to the high speed muscle areas. A theory or a mnemonic, it fits. So a PVL kid makes a move and his own undampened hyper spindles send out confounding reflex signals as the needed expectation setting was never received. This is spasticity in parallel robbing normal mechanisms of inertia & Energy. The pendulums sought by walking are dampened by an envelope of pointless reaction from many locations. We have the same need as with the elastics experiments – to find a new wheel to travel on - the 6 th wheel, we will call it. Read the Range v Resist book on our shelf – a must read. Here is a snippet. This is a young girl.

That girl is crouched with medially rotated legs, and walking on-toe with short steps. She is wearing sensors and some stuff of our making that does not block any of her joints, at all. Her final data with stick figures was seen by several gait labs & pundits. Her abnormalities were enumerated as were the joint angle curves well outside the normal standard deviation envelopes everybody was using (& still do). A list of open bone surgeries at the hips and shins and muscle surgeries were recommended. Though many variations, all sought to correct her documented deviations. Doing ‘shark bite’ was included (top to bottom big surgeries).

The reality is that the stuff we had on her was designed to dampen (defeat) the leg pendulums. Rob her energy. That’s it. Period. Oh, she was an international Irish Step Dancing championship level competitor. Not only was she not abnormal in a negative functional sense, whatever she did had to have been a solution to what we were doing to her .

These ‘abnormalities’ were solutions not disease and yet there was a universal ‘expert’ professional agreement to undo all of her well documented movement ‘abnormalities’ with multiple extensive surgeries. We did operate that evening by removing the rig. It took her two days to “get her legs back”. Although exhausted, she was our only test subject to finish our sequence of 9 levels of severity of inertia dampening (with 3 controls).

Oh, what about those publications that asserted that CP muscle must be metabolically abnormal based mostly on what was similar to all our test subjects? We have a place for them; the janitor empties it regularly.

Two joggers and a marathoner could not get halfway through these tests. EMG was continuously on with little rest intervals if any. Here we see a jogger at level 5 only able to crawl around the room. Just getting across the camera view was impossible for him. Despite his crippling energy state, he did crawl well.

Cocontraction. Swing phase despite being in a crouch, barely had any knee angle change. Toe walking was universal and early in onset in all our subjects. In later detailed review we did see our 6 th wheel at play. These are not just normal people, they are athletes whose expectation in things physical is excellence. Whatever they do we must assume is the best choice of what is available to them.

The disability was exhaustion. Other studies that only restricted various joint ranges of motion had no equal to this.

We were looking at a solution that all of our subjects immediately found. It Was some kind of built-in plan B. Plan B – for what?

Advancing in the absence of momentum . We all do this when we ascend steep stairs. Our eccentric walking – an evolutionary masterpiece – drops back to concentric walking. Eccentric energy conservation swaps to concentric stepping which is not involved in passing momentum between alternating pendulums. Walk in deep mud? Concentric.

Oh my! Are those mud prints of imposed CONCENTRIC walking? That would screw up our idea as to how the prehistoric people walked.

Back to our PVL people. So, rather than saying PVL is only able to eke out medial rotation crouch, we now say they use alternate (concentric) stair climbing gait to compensate for the missing (robbed) momentum. Robbed? By what? Spasticity in parallel. Get it? We used elastics to emulate spasticity in parallel. Once you see it, it gets so obvious. Angles become secondary. The body sheds angles for something that doesn’t need changing angles (muscle work). The goal is to make the transitions and swing phase unhampered.

When we experimentally restricted joint range sequentially (here knee joint), the normal subjects consistently found the well timed (relative to other leg) pendulum that allowed side to side energy transfer. Unfettered legs would alter posture to have symmetrical correspondence with the range of the restricted side.

Even with 60 degree ‘contracture’ the walking was robust. All (12) teen age subjects easily did the tasks. Range loss alone is not badly disabling. It can make you look funny, but it won’t keep you from doing things. Perhaps a bit sweaty. A tiny bit of this was published in CORR.

“Quadriplegia” & Ataxia: Quadriplegia, nooooo, TOTAL BODY cerebral palsy TBCP, on the other hand, is what is seen after diffuse anoxic brain injury (no O2). The pattern of brain involvement reflects damage wherever the brain's metabolic rate most demands oxygen and also where tissues are most easily damaged by concomitant CO2 build up (which makes acid). Include the heart in that, a reason to get at least one screening EKG in these kids. The really important brain stuff, the basic richly cellular deeper brain base regions are highly metabolic and thus sensitive to oxygen deprivation and CO2 excess & acidity. This is true in adults as well as children. Carbon monoxide poisoning, for example, plugs up hemoglobin such that oxygen can't get delivered by the red blood cells. Those basal brain cells which use oxygen most get damaged first. These are NOT only neurons, but also include cell types which support the brain structure and which nurture neurons. Interestingly, the effects of damage to these cells may not be apparent initially. Damage to supportive cells may take quite a while to manifest as secondarily affected neurons become more disabled over time by the lack of nurture from dead supportive cells. HEADLINE: "Hero Girl Scout Pulls Unconscious Man From Running Car In Garage. Man is fine." but slowly, over months or even years, time reveals the initially unseen damage. BACK PAGE: "Man Exposed to Fumes, Claims Lost WALKING. Insurer Cries Fake! (citing newsreels of him intact.) Back to TBCP. The typical basal ganglia insult will manifest over about two years. Children with ataxia, or athetosis often don't get diagnosed for that length of time. It doesn't mean that the symptoms were missed. It may well mean that the neuron manifestations evolved over time. Below the ventricles, spreading large blotches or lakes of injury may be found positioned along key brain centers called ganglia. Ganglia are centers