Amyotrophic Lateral Sclerosis (ALS) & the corticospinal tract

corticospinal_tract

Amyotrophic Lateral Sclerosis (ALS) is a degenerative disease of the motor neurons in the brain and spinal cord. It progressively affects all the muscles in the body but there is no known cause and no treatment. Only about 5-10% of cases are inherited while the rest are sporadic.

The neurons ALS affects are primarily the upper motor neurons. These are the ones that originate in the brain and travel down the spinal cord. These neurons then synapse with the lower motor neurons in the ventral horn, and it is the lower motor neurons that go directly to the muscles.

In ALS there are both upper motor neuron and lower motor neuron symptoms. As the neurons die, a constellation of symptoms including numbness, weakness and paralysis emerge. Eventually the paralysis progresses leading to inability to speak, swallow and breath. There is no cure for ALS and treatments only help with the symptoms, they do not slow the progression of the disease.

So you may have seen a lot of ice bucket challenges over the last few weeks but please support this cause as it is a horrible disease that up until now had almost no recognition or support. So please donate to The ALS Association (alas.org).

And in case you get tired or jaded seeing your social media full of these videos, watch this one of my father doing it. He’s not an emotional guy, but he has lost more than his fair share of friends to this disease.

donate to help fund ALS research and support from Ali & Mike on Vimeo.

PHACE Syndrome (hemangiomas)

PHACE_syndromeThere are no shortage of congenital syndromes that are acronyms arranged into some sort of vaguely pronounceable word. There will be lots of doodles about these, but we’ll start off with a more uncommon one – PHACE Syndrome.

PHACE Syndrome is a collection of findings that go along with large infantile hemangiomas. They’re the more worrisome (but less obviously disfiguring) things you need to look for when you see a baby with a large hemangioma on the face or multiple hemangiomas.

  • Posterior fossa brain malformations
  • Hemangiomas
  • Arterial anomalies
  • Cardiac anomalies and coarctation of the aorta
  • Eye abnormalities
  • Sternal cleft

The most common symptom of PHACE is cerebrovascular abnormalities, followed by cardiac anomalies (coarctation, aortic arch anomalies, VSDs). If you suspect PHACE, do clinical exam of the skin and eyes and MRI of the head, neck and chest.

Other cool facts

  • PHACE occurs in full-term normal birth weight infants (other hemangiomas tend to occur in preterm infants)
  • Quite common, more girls than boys (8:1)
  • Don’t confuse it with Strurge-Weber (port wine stain, associated with the facial dermatomes)
    • Port wine stains don’t proliferate and then regress like an infantile hemangioma

Brachial Plexus Part 1 – anatomical relations

brachial_plexus

The brachial plexus is the bane of many med students’ existence during any sort of neuro block. So many nerves, so many connections, so many seemingly arbitrary names of different sections. It’s just a woven mess of misery. (especially when they start getting into the “where is the lesion” questions)

Thus I’ve decided to have a couple posts about the brachial plexus, hopefully demystifying it to some extent. This first doodle is about the brachial plexus and its anatomical relationship to some of the structures that show why anatomists who named the parts weren’t as crazy as they seem.

Important structures to remember because they explain why parts are named the way they are:

  1. Vertebrae
  2. Anterior and posterior scalene muscles
  3. Subclavian artery
  4. The arm (in its anatomical position)

Vertebrae: There are 7 cervical vertebrae and 12 thoracic vertebrae. To make things confusing the cervical spinal nerves exit ABOVE their named vertebrae (except for C8) while the thoracic, lumbar and sacral exit BELOW. This messes up the whole numbering system because there are SEVEN cervical vertebrae but there are EIGHT cervical spinal nerve roots. The brachial plexus generally includes the nerve roots C5-T1*
* I say generally because there’s are anatomical variations such as a “prefixed” plexus that goes from C4-C8 and a “postfixed” plexus that goes from C6-T2

Scalene Muscles: The brachial plexus is nestled between the scalenes in the neck. At this point the plexus is oriented up and down and therefore the trunks are superior (closest to your noggin), middle, and inferior.

Subclavian Artery/Anatomical Position: The artery is in front of the plexus at the level of the trunks and then the plexus starts to wrap around it (or at least seems to because we don’t keep our arms straight out to our sides in “anatomical position” at all times). The cords are named for their relationship to the artery. One is lateral (again, if the arm was held out to the side), one is posterior and one is medial (think closest to armpit).

 

Subdivisions of the Brachial Plexus

The parts are: Roots/Trunks/Divisions/Cords/Branches or, as I remember them being a classy east coast Canadian: Real/Truckers/Drink/Cold/Beer

Then you might think, “But how do I remember which of the terminal branches comes off where?” For that I think of the two “M” branches being on the M: Musculocutaneous, Median and (M)Ulnar and that the whole thing together can just be said as “MARMU” Pick the mnemonics you want, the brachial plexus is rife with them. I personally just like the sound of the word marmu.

Complex Regional Pain Syndrome

crps

Hypo/Hyperalgesia:Decreased/increased sensitivity to a usually-painful stimulus (e.g., pinprick).
Hypo/Hyperesthesia: Decreased/increased sensation to a usually-innocuous stimulus (e.g., light touch).
Allodynia: Sensation of pain from a usually-innocuous stimulus (e.g., light touch).

Complex Regional Pain Syndrome (CRPS) refers to a chronic neuropathic pain condition with a broad and varied range of  clinical presentations. CRPS patients experience severe pain out of proportion to their original injury, and this may start at the time of injury or weeks later. The pain is described as deep-seated and burning/aching/shooting. Sesnory changes are common, including hypo/hyperesthesia, hypo/hyperalgesia, and allodynia. For instance, many patients describe not being able to tolerate the sensation of bedsheets on their painful limb.

In the affected area, there is often marked edema, temperature asymmetry (usually cooler), and sweating changes (usually increased). Loss of hair and nail growth is common, and disuse of the limb can result in weakness, muscle atrophy, and contractures.

The diagnosis is made clinically, using the Budapest Criteria. Some pain physicians use a nuclear medicine test, three-phase bone scintigraphy, for CRPS diagnosis but this test is becoming less popular, since it has a low positive predictive value.

Budapest Criteria

  1. Pain, ongoing and disproportionate to any inciting event
  2. Symptoms: at least one symptom in three of the four categories:
    • Sensory: reports of hyperesthesia and/or allodynia
    • Vasomotor: reports of temperature asymmetry and/or skin color changes and/or skin color asymmetr
    • Sudomotor/edema: reports of edema and/or sweating changes and/or sweating asymmetry
    • Motor/trophic: reports of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)
  3. Physical Signs: at least one sign at time of evaluation in two or more categories:
    • Sensory: evidence of hyperalgesia (to pinprick) and/or allodynia (to light touch and/or deep somatic pressure and/or 
joint movement)
    • Vasomotor: evidence of temperature asymmetry and/or skin color changes and/or asymmetry
    • Sudomotor/edema: evidence of edema and/or sweating changes and/or sweating asymmetry
    • Motor/trophic: evidence of decreased range of motion and/or motor dysfunction (weakness, tremor, dystonia) and/or trophic changes (hair, nail, skin)
  4. No other diagnosis better explains the signs and symptoms

CRPS is classified as Type I when there is no apparent history of nerve damage, and Type II when associated with definite peripheral nerve injury. CRPS most commonly occurs following fractures and immobilization, but can happen even with little to no trauma.The pathophysiology is thought to involve autonomic dysfunction and inflammation, but much is still unknown.

CRPS affects females about 2-4 times more often than males, and onset is usually in middle age (though there are rare pediatric cases reported). It is a progressive disease that can result in spread of pain, sensory disturbances, and physical changes to other limbs.

Treatment for CRPS may involve physiotherapy, complementary medicine (e.g., acupuncture, qi gong) psychological therapies, and a variety of pharmacologic (e.g., NSAIDs, anticonvulsants, antidepressants, opioids, ketamine, bisphosphonates) and interventional procedures (nerve blocks, sympathectomy, neurostimulators). As with all things CRPS, there isn’t great evidence for any particular intervention.

  • Harden RN, Bruehl S, Perez RSGM, Birklein F, Marinus J, Maihofner C, Lubenow T, Buvanendran A, Mackey S, Graciosa J, Mogilevski M, Ramsden C, Chont M, Vatine J-J. Validation of proposed diagnostic criteria (the “Budapest Criteria”) for Complex Regional Pain Syndrome. Pain; 150:268.
  • Hord E-D. Complex regional pain syndrome. In: Massachusetts General Hospital Handbook of Pain Management (Eds: Ballantyne JC, Fields HL). Lippincott Williams & Wilkins.
  • Moon JY, Park SY, Kim YC, Lee SC, Nahm FS, Kim H, Oh SW. 2012. Analysis of  patterns of three-phase bone scintigraphy for patients with complex regional pain syndrome diagnosed using the proposed research criteria (the ‘Budapest Criteria’). British Journal of Anesthesia; 108:655.
  • O’Connell NE, Wand BM, McAuley J, Marston L, Moseley GL. Interventions for treating pain and disability in adults with complex regional pain syndrome – an overview of systematic reviews. Cochrane Database of Systematic Reviews; 4:CD009416.
  • Schwartzman RJ, Erwin KL, Alexander GM. 2009. The natural history of complex regional pain syndrome. Clinical Journal of Pain; 25:273.
  • Smith H, Popp AJ. The patient with chronic pain syndromes. In: A Guide to the Primary Care of Neurological Disorders (Eds: Popp AJ, Deshaies EM). Thieme.
  • Tran DQH, Duong S, Bertini P, Finlayson RJ. Treatment of complex regional pain syndrome: a review of the evidence. Canadian Journal of Anesthesiology; 57:149.

Types of sutures (and when to use them)

sutures

There are many types of sutures and they differ by size, material and needle. I made this handy chart to help remember how long each type of material lasts in the body and what it’s commonly used for:

50% Strength Gone Reactivity Use
Ethibond
(coated polyethylene)
indef n/an/a + Tendon
Mersilene
(uncoated polyethylene)
indef n/a + Tendon
Nylon 20%/y n/a + Skin
Silk 1 year >2y ++++ Vessel ligation, drains
Prolene
(polypropylene)
indef n/a Skin
Steel indef n/a Tendon, sternum
Fast Gut 6d 20d ++++ Skin
Plain Gut 7d 70d ++++ Skin
Chromic Gut 28d 90d ++++ Oral mucosa
Monocryl
(Poliglecaprone 25)
7d 110d +++ Skin, subcuticular
PDS
(Polydioxanone)
21d 100d ++ Internal organs, fascia
Vicryl
(Polyglactin 910)
21d 90d ++ Skin, soft tissue

Key:
* Monofilament
* Braided

Vertebral Disc Prolapse (slipped disc)

A prolapsed (slipped) disc is when the squishy innards of the disc (nucleus pulposus) bulge out past the stiffer wall of the disc (annulus fibrosis). The problem is that sometimes when this happens, the bulge can impinge the spinal cord or the spinal nerve root. This could result in an anterior cord syndrome (remember this doodle) or it could just knock out the nerve root, resulting in a specific radiculopathy (check out this doodle for where to check for numbness and weakness).

The tricky thing to remember is that though, for example, the L3 root exits at L3, if the L3,4 disc herniates, it doesn’t hit the L3 root but the L4.

Slipped L3,4 disc = L4 nerve injury

The disc hits the nerve after it has branched off the spinal cord, but before it has exited the  vertebral canal.

Intracranial Hemorrhages

For the most part, bleeding in the brain (intracranial hemorrhage) is a pretty bad thing. Though like most things in medicine, there are varying degrees of badness, all with different mechanisms that help us sort of why we really wouldn’t want something to happen.

Intracranial hemorrhages are categorized into 5 subtypes, and are given obvious sounding names depending on where the bleed is in the brain and in relation to the layers of the meninges.

  1. Epidural (above the dura, right under the skull)
  2. Subdural (below the dura, above the arachnoid)
  3. Subarachnoid (below the arachnoid, above the brain)
  4. Intraventricular (in the ventricles)
  5. Intraparenchymal (in the meat* of brain)

* The brain is not meaty, “parenchyma” means the functional part of the organ

The poor pia mater did not get any hemorrhage named after it, but if you want you can think of intraparenchymal as “subpial” just so it doesn’t feel left out.

Telling them apart

The most confusing thing, and thing that likes to get asked the most on exams, is the difference between epidural and subdural hematomas.

Epidural Subdural Subarachnoid
Above the dura Below the dura Below the arachnoid
Respects suture lines Doesn’t respect suture lines No respect for anything
High force trauma Low force trauma Aneurysm rupture or high force trauma
Arterial blood (commonly the middle meningeal artery) Venous (from venous plexus) Arterial from the circle of Willis
Lentiform (lens-shaped) or biconcave on CT Cresent (banana-shaped) on CT Lining surface, going into fissures and sulci and sella (death-star)
Acute presentation May be insidious (worsening headache over days) Acute presentation (thunderclap headache)

The reason intraventricular and intraparenchymal aren’t included in the table as they each have a bunch of causes, but for both of them trauma is a potential cause as well as hypertension and stroke. It’s good to remember that premature infants are at a much higher risk of intraventricular hemorrhages.

Blood on CTs

  • New blood: bright white
  • 1-2 weeks: isodense
  • Old blood (2-3 weeks): dark grey

Innervation of the lower leg

The lower leg (and especially the foot) have a pretty fancy pattern of skin innervation by the terminal branches. For example, the skin of the foot is innervated by 7 separate nerves:

  1. Superficial peroneal nerve
  2. Deep peroneal nerve
  3. Sural nerve
  4. Saphenous nerve
  5. Calcaneal branch of the tibial nerve
  6. Medial branch of plantar nerve
  7. Lateral branch of plantar nerve

Also good to keep in mind that the anterior compartment is innervated by the deep peroneal nerve, the lateral compartment by the superficial peroneal nerve and the posterior compartment by the tibial nerve.

Glasgow Coma Scale (GCS)

BUY THIS AS A STUDY CARD

The Glasgow Coma Scale is a scoring system used to evaluate someone’s level of consciousness. It is scored out of 15 with 15 being totally awake and alert and 3 being totally not.

The important thing to remember is that the lowest score possible is 3.

Absolutely anything can score a 3, however if you are a living, breathing human being, hopefully you are scoring well up into the 10s.

Generally the GSC is applied in trauma situations and can be used as part of the decision making process of such thing things like should this patient be intubated?

  • ≥13 correlates with mild brain injury (or being ok)
  • 9-12 correlates with moderate injury
  • ≤8 represents severe brain injury – you should probably consider intubating them as they most likely cannot protect their airway
Thanks to Mike for the guest doodle!

DANGER ZONE!!! (the cavernous sinus)

The danger zone on the face is a little triangle from the corners of the mouth up to the bridge of the nose. The reason it has such an epic name is because due to its venous drainage (from the facial veins and pterygoid plexus) there’s the possibility of infection traveling from that area into the cavernous sinus.

The cavernous sinuses (there’s one on each side) is an area posterior to the maxillary sinuses and lateral to the pituitary. It receives blood from the superior and inferior ophthalmic veins, superficial cortical veins and the basilar plexus. The blood then drains into the petrosal sinuses (you guessed it, there’s a superior and inferior one of those too) and then those drain into the internal jugular vein.

The thing about the cavernous sinuses a whole lot of important stuff passes through it.

  • CN III (occulomotor)
  • CN IV (trochlear)
  • CN V1 (ophthalmic branch of trigeminal)
  • CN V2 (maxillary branch of trigeminal)
  • CN VI (abducens)
  • Internal carotid (and the sympathetic fibres on the carotid)

This means that if you are so unfortunate as to have infection tract back into it, there can be some nasty consequences like meningitis and cavernous sinus thrombosis which will generally present as problems involving those nerves.

The abducens and carotid are more medial and thought to be more bathed in the warm loving venous drainage meaning these are generally the first to show signs of a problem a-brewin’.

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