After a bit of a break, we are back! This week we bring you an interesting case combining our medical and neurologic knowledge, Duriel’s lifestory is explained and Noah gets to read about his favorite peripheral nerve. We hope that everyone in LA takes a break from neurology for more neurology!
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Case of the Week : That time when intern year really paid off…
A young man presented to the emergency department with progressive pulmonary symptoms, abdominal pain, diarrhea and painful paresthesias of his hands and feet. His symptoms date back almost a year ago when he developed sinus congestion and wheezing and was started on azithromycin for a presumed sinus infection, without clinical relief. This was followed by a second course of persistent pulmonary symptoms treated with prednisone with improvement. He was doing well until a few months later when he developed abdominal pain and weight loss and presented again to the hospital. Work up at this time revealed elevated peripheral eosinophils (57%) in addition to acalculous cholecystitis. He was treated with albendazole and zosyn, but continued to worsen. During this time he began having painful paresthesias, first starting in his feet bilaterally moving up to the ankles over a few days and ultimately into his hands bilaterally, mostly in the ulnar nerve distribution. At the time of consult, he had developed weakness in his right hand as well.
Sensory examination revealed abnormalities in the bilateral ulnar and tibial nerve distributions. Weakness was noted to the right > left interosseous muscles of the hand, wrist flexion and right plantarflexion. Laboratory work up revealed a significantly elevated ESR and CRP, marked eosinophilia and a positive p-ANCA (MPO), solidifying the diagnosis of EGPA (eosinophilic granulomatosis with polyangiitis, or Churg-Strauss). Neurologically, his multiple neuropathies was consistent with mononeuritis multiplex, by clinical examination and EMG. Patient was started on high dose steroids in addition to rituximab. A major learning point from this case for both neurology and rheumatology is that the presence of mononeuritis multiplex on diagnosis of EGPA predicts the need for additional immunosuppressant agents beside steroids in order to control the disease. Neurologists can be very helpful in this manner to help rheumatology with appropriate therapeutic decisions. In this patient’s case, therapy was initiated even prior to EMG as our clinical suspicion and examination heavily supported MM.
Baby Duriel Finds His Way Through Life: A Story of Milestones
To preface this discussion, every child is different. Although some kids may be ahead in terms of milestones and some others can be behind, both kids could be developmentally normal despite the variation in reaching milestones. Different resources have varying ranges on when certain milestones can/should be reached which also speaks to the fact that meeting milestones is extremely variable. If you are concerned that a child may be delayed, one trick while on the neurology floor is to ask the family if they have other kids. They will often know when their older child reached “x” milestone, so this can be helpful to compare. The CHOP JAR guide has a great section with a chart about milestones for your viewing pleasure (with many more details) as well. Let’s begin with a story.
Let’s take a look at a normal child, who we will call Duriel. Duriel has lovely parents who have documented his young developing journey. When they first brought Duriel home he was helpless, always crying, pooping and napping, but then at one month of age he started on his well way, finally being able to control his head somewhat, but just barely. As you walked to his side at about 90 degrees, he would follow you, but then ignore you completely. At two months of age he could follow you even more, to a whole 180 degrees! He was so excited about the development that he started to coo and smile. Nothing much happened for the next few months until he was about 6 months old when things started to get interesting. Now that his head could stay up unsupported, his body followed suit and was officially sitting, grabbing toys and rolling over. He started to get a little clingy though and would crawl around everywhere you went, babbling away. At this point, being a parent seemed okay, easy enough. After all, they couldn’t really go anywhere too far. Unfortunately things took a drastic turn, when at 9 months, Duriel started to pull himself up to stand and would spill his sippy cup everywhere now that he could actually drink from it. He would say mama and dada incessantly, but they thought he was still cute and therefore threw him a one year birthday party with all their friends a few months later. As the birthday cake was being passed around, Duriel got himself up and started to take a walk across the room, passing John, the neighborhood boy who was already 18 months and wasn’t walking (John is going to see the pediatrician next week), on the way to his presents. His presents were so numerous it took Duriel three months to open them, so finally at age 15 months he was able to point to the coloring book he received, use the crayon to color (albeit no where near refrigerator status art) and throw the softball Uncle Bob got him into the bookshelf, promptly spilling his mother’s coffee. The 4-6 words he could say at his current age did not include “sorry, mom.” A few months later, at a year and a half, Duriel began to run (sort of, not quite his current 5 minute mile pace) and build blocks with his friends. Duriel’s parents decided it was time to have another child, so documented his development only at year intervals after this. At age two, he was pulling the laundry out of the upstairs hampers (Duriel learned to climb stairs…), forming short sentences with his 50 word vocabulary and learning to use an actual toilet. Unfortunately toilet training lasted him until the age of three, but as a reward for his success his parents bought him a tricycle he could finally ride and a new closet of clothes he could dress himself with. When Duriel got to age five, he was so excited to start kindergarten that he skipped all the way there, naming all the colors and numbers he saw along the way. Congratulations Duriel, you have made it to school, and now your parents finally have the break they deserve.
The Price is Rite Review : A Blinding Headache
A 35 year old male presents to his primary care doctor with a headache and an odd constellation of symptoms. His italian grandmother’s lasagna doesn’t have the same pizzaz he remembers when eating it as child. In addition, he has trouble reading his twitter feeds when covering his right eye. His examination was notable for central vision loss of the right eye and papilledema on the left. An example of his MRI scan is demonstrated below.
This is a classic example of an entity known as Foster Kennedy syndrome. Classically, this is caused from meningiomas or any other mass occupying lesion in the frontal lobes (especially in the occipital groove, highlighted in purple on the adjacent figure). Patients classically present with optic nerve compression and central vision loss of the ipsilateral eye. In contrast, the contralateral eye typically demonstrates papilledema as a result of increased intracranial pressure. To the horror of italian grandmother’s everywhere, the patient’s often have anosmia. Other associated symptoms include nausea/vomiting, and emotional lability or memory complaints.
Anatomic Ambushes : Because anatomy is our strength
We at the cortical ribbon think it’s only Rite to give a proper shout-out to the true MVP of our recent exam, the anterior interosseus nerve. This nimble nerve hailing from Oliver Sack’s Venice Beach days is an exclusive motor nerve with no sensory branches. Associated with the median nerves, it innervates the three deep forearm flexors, the flexor digitorum profundus (finger flexors!), Flexor pollicis longus (thumb flexor), and the pronator quadratus (pronate wrist, i.e. hand down). When compressed, the classic associated deficits it the inability to make an OK sign with the thumb and forefinger.
This is your Brain on Poetry – Weekly Neurology Haiku
Three more months to go
Two intern years come and gone
Please, q4 no more