Baffling Neurology Pathology Strikes Again…

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…forever seeking the unsuspecting emergency medicine intern.

A previously healthy teenage male presented to the emergency department via emergency medical services with the complaint of weakness, sensory changes, and increasing difficulty of breathing.  Family reported the patient went outside to mow the grass approximately 10 hours prior to presentation.  After two passes in the yard with a push mower, the patient had sudden onset occipital headache, fatigue, parasthesias described as tingling in both lower extremities, and generalized weakness.  Patient reported these symptoms to parents and laid down to rest.  After an hour long nap, patient was encouraged to take a shower to see if symptoms improved.  During shower, patient became weaker, had one episode of vomiting and was no longer able to stand on legs.  The patient’s father reports patient was able to make small movements but unable to ambulate or push against resistance.  Emergency medical services were contacted at this point, however, after assessment, the symptoms were deemed related to anxiety and patient was not transported.  Over the course of the day, the patient’s weakness progressed to the complete inability to move legs, followed by inability to move arms, with continued paresthesias in all extremities. After patient demonstrated worsening respiratory distress, EMS was called again.  Parents denied history of asthma, prior wheeze, fevers, recent illness or trauma, recent travel or drug abuse.  Family reports patient went camping one week prior but denies tick exposure.

On exam, the patient had significant respiratory distress with poor air movement and was only able to answer questions with one word responses secondary to respiratory distress.  Neurological exam revealed 0/5 strength in all extremities, areflexic biceps and brachioradialis reflexes, areflexic patellar and Achilles reflexes, and downward going Babinski bilaterally.  Sensation to light touch was intact but diminished in all extremities.  There were no rashes or lesions on skin exam.

Initial differential included but was not limited to:

  • Organophosphate toxicity – although patient self-decontaminated earlier in the afternoon and did not have diarrhea, salivation, or lacrimation
  • Guillain Barre – although acute time course without report of recent URI or GI syndromes
  • Tick paralysis – although no known exposure or lesions identified on skin or scalp
  • Transverse Myletitis – although no personal history of recent illness, no family history of multiple sclerosis or other autoimmune disease
  • Spinal Cord Infarct – although patient and family deny trauma, recent surgery, or history of coagulation disorder
  • Conversion Disorder – although no anxiety or other psychiatric history

As concern for bronchospasm, the patient was given IM epinephrine and started on a hour long albuterol while history was being obtained.  Bedside ultrasound demonstrated grossly normal myocardial contractility without effusion, normal lung sliding, and a collapsible IVC.  Chest x-ray was unrevealing. After lack of improvement with initial intervention, second dose of epinephrine and fluid bolus begun.  Intial VBG demonstrated hypercapnea and patient was started on BiPAP.  Within minutes, patient showed improvement in respiratory status and appeared more comfortable.  Initial CMP, CBC, CRP, and ESR were unrevealing.   Lumbar puncture was performed after head CT revealed only an arachnoid cyst.  CSF studies demonstrated mildly elevated segs, mildly elevated glucose, negative gram stain and normal protein.  Foley catheter was placed after patient complained of bladder fullness (confirmed by bladder scan) with inability to void.  Neurology and Neurosurgery were consulted from the emergency department, who requested urgent MRI on admission.  Patient was admitted to PICU with ED diagnoses of acute flaccid paralysis and acute neuromuscular respiratory failure.   After admission, MRI was obtained and demonstrated (drum roll please) ischemia vs. infarct from approximately C2-T5 with predominance in the anterior horns.

The literature review of non-traumatic spinal cord infarction is as rare as the pathology itself in the pediatric population and predominately consists of case studies.  Causes typically include but are not limited to hypotension, vascular injury, thrombus, embolus or compression.  Considering many of these etiologies are already scarce in the pediatric population, finding cause proves to be a difficult task.   Pain in neck, back or legs, weakness, tingling, and numbness are commonly described symptoms in pediatric case studies and were present in the patient that presented to us.   Prognosis studies have been performed on primarily adult patients; one such study demonstrated >40% recovery in ambulation amongst the 37 patients that left the hospital wheelchair bound through aggressive physical therapy and rehabilitation programs.[1]   Poor prognostic factors include female sex, advanced age, severity of symptoms, and lack of improvement within 24 hours after infarction.[2]   It appears as though outcomes are primarily dependent on access to physical therapy and supportive care including psychological treatment.   Workup and treatment recommendations if suspicious of atraumatic spinal cord infarct include obtaining an MRI for definitive diagnosis, followed by autoimmune, hypercoagulable, and infectious evaluations. Treatment in the emergency department is mostly supportive and includes addressing any contributory conditions and the management of respiratory concerns.[3]

On follow up, I found that the patient required intubation after exhibiting respiratory decompensation after the MRI was obtained.  Initial treatment with high-dose steroids and IVIG were discontinued when infectious and immunologic work-ups were unrevealing.  The hospital course was complicated by neurogenic bowel and bladder, neuropathic pain, anxiety, intermittent autonomic instability with hypotension, pneumonia and a urinary tract infection.  The patient required tracheostomy and percutaneous gastric tube placement during his hospital stay.  The patient continued to exhibit flaccid paralysis of all four extremities and ventilator dependence on discharge to acute rehabilitation.

Per my own investigation, I was relieved to find that Frazier has both success managing these cases and many unique resources that may positively impact this patient’s course (if you are curious here is some video evidence https://www.youtube.com/watch?v=8o_w174rI3s).  Needless to say, my differential for motor weakness, particularly in pediatrics, will forever be haunted by this case.

 

[1] Robertson CE, Brown RD Jr, Wijdicks EF, et al. Recovery after spinal cord infarcts: long-term outcome in 115 patients.  Neurology. 2012; 78: 114–121.

[2]Cheshire WP, Santos CC, Massey EW, Howard JF Jr .  Spinal cord infarction: etiology and outcome.  Neurology. 1996;47(2):321.

[3] Spencer, Sandra P. MD; Brock, Timothy D. MD; Matthews, Rebecca R. MD; Stevens, Wendy K. MD.  Three Unique Presentations of Atraumatic Spinal Cord Infarction in the Pediatric Emergency Department Pediatric Emergency Care. 30(5):354-357, May 2014.

8 thoughts on “Baffling Neurology Pathology Strikes Again…”

  1. You’re able to take your training to the next stage and that additional little kick is
    all you want. J.R.S. has performed industry-sponsored analysis on creatine and
    different nutraceuticals over the previous 25 years.
    Further, J.R.S. has additionally received financial
    help for presenting on the science of assorted nutraceuticals, except creatine, at industry-sponsored scientific conferences.
    Bear In Mind, what’s necessary is finding what works best for you and aligns together with your values.
    It’s a high pick for athletes who can’t risk banned substances and for anybody who cares concerning the security and efficacy of what they’re placing in their bodies.
    The European Food Security Authority (EFSA)
    recommends that DHT levels should not exceed three mg/kg.
    Energy could be very easily and rapidly mobilized from creatine and used
    to replenish the main vitality source which human cells
    use for his or her functioning (adenosine triphosphate – ATP).
    While Kre-Alkalyn does have many advantages, corresponding
    to no need to have a loading part, etc., it is notably famous for its advantages in enhancing exercise performance.
    Creatine is amongst the most well-researched and effective sports dietary supplements to exist at present.

    It’s probably the greatest supplements to increase energy, energy, and peak
    efficiency for any athlete at any stage. It primarily promotes lean muscle growth and improved efficiency with out causing
    extreme water retention. People use creatinol-O-phosphate supplements
    as a outcome of they consider these dietary supplements make them stronger,
    more muscular and extra in a place to recover shortly during and after strenuous
    exercise.
    These amplifying results could be brought on by the elevated complete creatine store in working muscles.
    Even although vegetarians had a greater enhance in excessive vitality phosphate content,
    the IGF-1 ranges have been much like the amount observed within the non vegetarian groups.
    These findings do not support the noticed correlation pattern by which a
    low important amino acid content of a typical vegetarian food regimen should cut back IGF-1 production [33].
    Most creatine analysis initially targeted on creatine’s role in train efficiency, training adaptations, and safety in untrained and trained healthy individuals [1].

    Creatine supplementation has been reported to increase muscle creatine and PCr ranges, improve acute train capability,
    and enhance training adaptations [44,sixty six,sixty nine,seventy two,73,74,seventy five,seventy six,seventy seven,seventy eight,seventy nine,80,eighty one,82,83,eighty four,85,86,87,88,89,90,ninety one,ninety two,93,ninety four,95,96].
    Ergogenic benefits have been reported in men and women from kids to aged populations,
    though nearly all of studies have been carried out on men [74,111,113,124,a hundred twenty five,126,127,128].

    Samples had been run in duplicate to confirm outcomes if the observed values were exterior control values and/or medical norms based on commonplace procedures.
    Therefore, creatine monohydrate is the recommended kind
    for the overwhelming majority of people. Creatine monohydrate is the most common, cheapest, and well-researched type of creatine.

    It’s no marvel why athletes, weightlifters, and fitness
    enthusiasts have been utilizing creatine for many
    years. Phosphocreatine can anaerobically donate a phosphate group to
    ADP to kind ATP in the course of the first 5
    to eight seconds of a maximal muscular effort. [citation needed]
    Conversely, extra ATP can be used throughout a period of low effort to convert creatine back to phosphocreatine.
    As we now have mentioned, creatine is a complement with an extended and wealthy history of utility, particularly its monohydrate.

    It seems to be best at helping athletes experience bursts of velocity
    and vitality, particularly over brief durations. This software reveals its usefulness in actions
    like racing and weight lifting when the physique must carry out higher, stronger and sooner over
    a short period (x). Since creatine pulls water into your muscle cells, some customers
    report bloating, puffiness, and even momentary
    weight achieve. This isn’t dangerous at all, but it is quite irritating if you’re making an attempt to keep lean.
    Creatine ingested by way of supplementation is transported into the cells completely by
    CreaT1. Nevertheless, there is another creatine transporter Crea T2,
    which is primarily lively and present in the testes [12].
    Creatine uptake is regulated by numerous mechanisms, particularly phosphorylation and glycosylation in addition to extracellular and intracellular levels of
    creatine.
    Traditionally, this calculation relies on BM as a contributing issue [13,14,24],
    however could also be improved by considering FFM instead of complete BM.
    Cohen’s d for related variables between baseline,
    placebo, and creatine circumstances. All visits to the laboratory involved the identical experimental procedures described beneath.

    It is the most extensively used type of creatine, and the
    form most utilized in analysis, which is a robust indication of its
    effectiveness and safety. Kre-alkalyn contains alkaline powder
    in order to modify the pH stability, in contrast to many different
    forms of creatine which don’t have this. Research has discovered that it’s more helpful to supplement with creatine immediately after a exercise due to its optimistic impact on power and body composition.
    Creatine is a naturally-occurring natural acid that serves
    as an power buffer and energy shuttle in tissues,
    similar to mind and skeletal muscle, that exhibit
    dynamic vitality requirements. Proof, deriving from a wide range of scientific domains, that mind bioenergetics are altered in melancholy and associated problems is growing.
    Scientific studies in neurological circumstances corresponding
    to PD have indicated that creatine may need an antidepressant
    effect, and early medical research in depressive disorders—especially MDD—indicate that creatine might have an necessary antidepressant impact.
    An individual’s muscle fiber type may also dictate
    the response of creatine uptake [14]. Brault and Terjung [14] examined rat hindlimbs and their response to creatine supplementation. Creatine uptake rates and
    CreaT protein content were observed to be biggest in highly oxidative muscle
    and lowest in glycolytic fibers, whereas, CreaT mRNA was not considerably different between all fiber types.

    However, some studies have shown that consuming 2–3 g/day of creatine for 30
    days can also successfully improve muscle creatine stores
    [46,49]. When creatine supplementation is mixed with heavy resistance
    coaching, muscle insulin like growth issue (IGF-1) concentration has been shown to increase.

    Compared to placebo, creatine groups produced higher increments in IGF-1 (78% Vs 55%) and body mass (2.2 Vs 0.6 kg).
    Moreover, vegetarians throughout the supplemented group had the largest improve of lean mass in comparability with non vegetarian (2.4 and 1.9 kg respectively).
    Changes in lean mass had been positively correlated to
    the modifications in intramuscular total creatine shops which have been also correlated with
    the modified levels of intramuscular IGF-1. The authors suggested that the rise in muscle IGF-1 content material in the creatine group could possibly be because of the higher metabolic demand created
    by a more intensely performed training session.
    Of this, about 2/3 of creatine is certain with inorganic phosphate (Pi) and saved as phosphocreatine (PCr) with the remainder saved as free creatine
    (Cr). The whole creatine pool (Cr + PCr) is about 120 mmol/kg of
    dry muscle mass for a 70 kg particular person who maintains a diet that includes
    pink meat and fish. Vegetarians have been reported to have muscle creatine and PCr stores about 20–30%
    decrease than non-vegetarians [46,47]. The body breaks down about 1–2% of creatine in the
    muscle per day into creatinine which is excreted in the urine [46,forty eight,49].
    Degradation of creatine to creatinine is greater in people with
    larger muscle mass and people with larger bodily exercise ranges.
    Subsequently, a normal-sized particular person might have to devour 2–3 g/day
    of creatine to take care of normal creatine stores relying on diet, muscle mass, and bodily activity
    levels. The best and rapid method to enhance muscle creatine shops is to ingest 5 g of creatine monohydrate
    4 instances every day for 5–7 days (i.e., 0.three g/kg/day) [46,49].

    In addition to that, it is wealthy in nutritional vitamins such as nutritional vitamins A, E, B3, B12, and B6, minerals
    like iron, zinc, and selenium. Individuals were given weekly questionnaires on how
    nicely they tolerated the complement, how nicely they adopted the supplement protocol, and
    if they skilled any medical problems/symptoms in the course of
    the examine. Compliance to the supplementation protocol was monitored by delivering empty weekly supplement containers, complement logs and verbal affirmation. After
    finishing the compliance procedures, subjects got the required dietary supplements and dosages for the next supplementation period.
    Muscle biopsies were obtained using a modified Bergstrom needle
    biopsy method following standard procedures [42].

    Just Lately, Braggion et al. [67] also observed that coaching carried out
    on a ladder (3×/week for 12 weeks) increased collagen fibers in the soleus
    skeletal muscle of ovariectomized rats. The upregulation of collagen turnover
    as a outcome of extended training might be brought on by the increased exercise of fibroblasts involved in muscle hypertrophy and regeneration [67,68].
    This change may provide mechanical support to muscle fibers, as
    resistance training promotes a excessive contractile demand to multiple skeletal muscular
    tissues, regardless of different histological, biochemical, and
    metabolic properties [51]. The examine could lack enough energy to
    detect a big impact of 15-s work relative to FFM.
    At the group level, creatine supplementation does not reduce glycolysis and lactate production however could barely dilute lactate concentrations.
    It is very really helpful for weightlifters,
    bodybuilders, and sports requiring explosive energy, such
    as sprinting, soccer, and basketball. When evaluating the efficacy of creatine phosphate relative to creatine monohydrate,
    it is important to consider their roles in the body’s vitality techniques.
    Creatine phosphate acts as a rapid power reservoir, particularly during high-intensity actions.
    The physique’s creatine phosphate shops are limited and can be depleted shortly with intense exercise.

    Little research supports these assertions immediately, however arginine’s NO-boosting effect
    improves muscle growth in young rats, whereas KIC and AKG have
    muscle-building or muscle-supporting effects of their very
    own. It Is definitely believable that these (and probably other) NO complement combos might improve pump or muscle mass
    for some. Additionally, the solubility of creatine monohydrate dietary supplements
    is poor in comparability with Kre-Alkalyn, which dissolves completely in water and is considered superior
    on this regard. As is the case with many supplements, the advantages that include regularly
    taking creatine will not be permanent.
    Creatine promotes muscle growth, endurance, and restoration from exercise
    classes. An elevated fee of ATP regeneration helps assist muscle perform during exercise, which might help you prepare more durable for more extended
    intervals with out experiencing fatigue. Creatine Nitrate is bonded to a nitrate group to doubtlessly improve solubility thus higher bioavailability, sound familiar?
    One Other benefit of including a nitrate group to creatine, is an increase in oxygen ranges to the bloodstream for larger muscle pump.

    [12] Creatine monohydrate and Kre-Alkalyn had been compared in a double-blind placebo examine with 32 resistance-trained individuals.
    Both teams increased muscle creatine, fat-free mass and power with no significant difference between the 2 groups.
    This implies that Buffered Creatine just isn’t superior to creatine monohydrate, it simply has a a lot bigger price ticket.

    In addition, creatine salts must outperform the bodily mixture of CM and
    the corresponding acid, showing synergistic effects. Just like creatine
    monohydrate, nitrate also works effectively to spice up
    the efficiency. As talked about, Creatine nitrate
    is more soluble in water than creatine monohydrate, that means that it’s more simply absorbed
    into the bloodstream. In a research, it is
    proven that regular supplementation will increase the quantity of intracellular creatine,
    which boosts the cell’s capacity to generate adenosine triphosphate.

    Moreover, some research in cell tradition has indicated that combining creatine and sodium might also enhance creatine uptake via the manipulation of increasing the gradient
    during which the CreaT functions [27]. The current literature is very preliminary in relation to inspecting how creatine supplementation affects CreaT expression while concomitantly following a resistance coaching protocol.
    Earlier analysis has concluded that there
    isn’t any gender distinction within the complete
    creatine content in muscle, both before or after supplementation. Though this means that CreaT activity
    is unlikely to differ between genders, no analysis has
    directly investigated this. Murphy et al. selected to focus upon whether there is
    a distinction between genders in CreaT mRNA and protein in healthy,
    younger adults [21].
    While creatine retention was significantly larger in the CM
    and dextrose group, no vital differences were seen between the CM
    and effervescent TCC groups. These findings suggest that while consuming
    a comparatively small amount of dextrose with CM can improve whole body creatine retention, supplementation of TCC in an effervescent form doesn’t augment whole body creatine retention greater than CM
    alone (Fig. 9). As an introduction about creatine’s potential position in well being and disease, the following offers a basic overview of creatine’s metabolic function, purported advantages throughout the lifespan, and potential therapeutic purposes.
    Moreover, we provide reasonable conclusions about the state
    of the science on creatine supplementation. We hope that this review and
    particular concern will assist readers and medical practitioners higher
    perceive the security and efficacy of creatine supplementation in a wide range of populations and provide recommendations
    about future research wants. These who want to shortly increase muscle creatine content material might consider what’s often recognized as a loading
    section.
    Although there’s less research obtainable on creatine HCL compared to monohydrate, preliminary studies and anecdotal proof recommend that it could require a decrease dosage to achieve
    similar advantages because of its enhanced absorption rates.

    You might be pondering, ‘these benefits sounds compelling’, and you’d be
    right, they are. Nevertheless, the overwhelming majority of research on creatine’s
    efficacy, together with its security profile and long-term results on muscle progress
    and efficiency, has been carried out utilizing creatine monohydrate.
    Consequently, whereas Creatine HCL presents an intriguing various, notably for individuals who
    may not tolerate monohydrate well, the foundational benefits
    of creatine supplementation as a whole, stay consistent across each types.
    By replenishing ATP levels extra effectively, creatine supplementation allows athletes, bodybuilders and on an everyday basis lively people to maintain greater performance levels.
    This interprets to important improvements in strength, energy
    output, and muscle endurance, which might help you push tougher throughout workouts and recuperate faster between units (Wu, S
    et al., 2022).

    References:

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    References:

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