Delirium can be the first sign of neuromuscular respiratory pump failure

It is not unusual for a serious attack of hypercapnia to present with DELIRIUM. In a young adult especially, an attack of delirium can be misdiagnosed as a psychiatric condition. This is what I think happened to Jerry when he became very sick suddenly at the age of 21.

Without careful assessment, delirium can easily be confused with a number of primary psychiatric disorders because many of the signs and symptoms of delirium are also present in conditions such as dementia, depression, and psychosis. DELIRIUM, ONDRIA C. GLEASON, M.D., University of Oklahoma College of Medicine, Tulsa, Oklahoma Am Fam Physician. 2003 Mar 1;67(5):1027-1034.

There was no careful medical assessment when Jerry was taken to the emergency department. There was no measurement of his ability to breath. There was only fear and a medical type of profiling on the part of the staff …… This kind of medical profiling involves sedating the patient and then sending him for psychiatric help and giving more sedation and pretending that this is the correct treatment. Jerry most likely needed some assistance with ventilation, perhaps until he recovered from his illness [a minor respiratory and G.I. infection] or perhaps lifelong non invasive assisted ventilation. The psychiatrists often admit the patient, leaving lots of time to thoroughly assess him for illness or injury requiring medical care, but they do not do this, not even when that patient is admitted for a lengthy period in a hospital…in a hospital where there are many many doctor specialists to help. The psychiatric profiling works to prevent any investigation into the patients birth history, paediatric medical history, family medical history or presenting medical illness that the patient is too sick to mention. This medical profiling is especially common when a young adult man or woman is delirious and agitated . [it is true that delirium is very scary to witness]…

I think that Jerry had unknown neuromuscular injuries or chest cavity rib problems from his difficult birth and possibly from further injuries/accidents in childhood.

Jerry might even have a congenital myopathy. Jerry’s grandmother had 5 children- all of whom had neuromuscular disorders at birth, including his mother. One baby died at birth of additional heart problems, and another died as a toddler, also of heart complications. The three survivors also had congenital myopathy. They were “floppy ” infants. Jerry thought that his mother had had Rickets in childhood, but upon reflection, she and her siblings most likely had a congenital myopathy. Jerry might have had a mild version. Who knows?

Congenital myopathy is a term sometimes applied to hundreds of distinct neuromuscular disorders that may be present at birth, but it is usually reserved for a group of rare, inherited, primary muscle disorders that cause hypotonia and weakness at birth or during the neonatal period and, in some cases, delayed motor development later in childhood.

The 3 most common types of congenital myopathy, in order, are
• Central core and multiminicore myopathies (core myopathies)
• Centronuclear myopathy
• Nemaline myopathy

The types are distinguished primarily by their histologic features, symptoms, and prognosis. Treatment of congenital myopathy is supportive and includes physical therapy, which may help preserve function. Sometimes symptoms do not manifest until adulthood.

Weakness is nonprogressive, and life expectancy is normal.

By Michael Rubin , MDCM, Weill Cornell Medical College
Last full review/revision Jan 2019| Content last modified Jan 2019
https://www.merckmanuals.com/professional/pediatrics/inherited-muscular-disorders/congenital-myopathies

Doctors treating adolescents and young adults should be familiar with a spectrum of paediatric neurological diseases that are not typically encountered in the adult.

Unfortunately adult doctors often do not consult with paediatricians and psychiatrists do not consult, period.

One evening Jerry became sick, with some minor GI or respiratory infection, suddenly felt more unwell, had trouble breathing and did not know why. He didn’t look so good, he didn’t feel so good and became restless, irritable then psychotic and combative. An acute attack of respiratory distress is a very real possibility, especially in a person with unknown, unlooked for neuromuscular weakness. The Richmond Agitation-Sedation Scale (RASS) is used to assess the need of critically ill patients for sedation. Jerry was very agitated and very ill and needed to be assessed for delirium.

Delirium and confusional states are among the most common mental disorders encountered in patients with medical illness, …….They are associated with many complex underlying medical conditions and can be hard to recognize. https://www.uptodate.com/contents/diagnosis-of-delirium-and-confusional-states

In adults, the Richmond AgitationSedation Scale (RASS) provides a single tool that is intuitive, easy to use, and includes both agitation and sedation. The RASS has been shown to be both reliable and valid in critically ill adults with and without mechanical ventilation and sedating medications.

Richmond Agitation-Sedation Scale (RASS)

Ranks agitation and possibility for sedation

Combative+4

Very agitated+3

Agitated+2

Restless+1

Alert and calm0

Drowsy-1

Light sedation-2

Moderate sedation-3

Deep sedation-4

Unarousable sedation-5

MANAGEMENT

  • Patients with a RASS of -3 or less should have their sedation decreased or modified in order to achieve a RASS of -2 to 0.
  • Patients with a RASS of 2 to 4 are not sedated enough and should be assessed for pain, anxiety, or delirium. The underlying etiology of the agitation should be investigated and appropriately treated to achieve a RASS of -2 to 0.

CRITICAL ACTIONS

  • A RASS score should be obtained on all hospitalized patients and at regular interval in all mechanically ventilated patients.
  • Patients with a RASS of 2 to 4 are not sedated enough and should be assessed for pain, anxiety, or delirium. The underlying etiology of the agitation should be investigated and appropriately treated to achieve a RASS of -2 to 0 https://www.mdcalc.com/richmond-agitation-sedation-scale-rass

Psychiatrists, for some reason, NEVER assess for critical illness in young adults even though anyone can suddenly become critically ill. Psychiatrists NEVER assess for delirium. Psychiatrists ASSUME that the agitation and altered mental status is psychological. They make lots of mistakes and people often don’t get better. Critical illness has a high level of morbidity and mortality.

Jerry was taken to hospital where he was sedated and then everything went wrong. He should have been assessed for delirium instead of for a psychiatric disorder. He should have been assessed for a neuromuscular weakness affecting his ability to breath, he should have been diagnosed with a neuromuscular respiratory pump disorder causing hypercapnia. No one asked his parents about possible neuromuscular disorders in the family.

Unfortunately, we are still, in the 21 st century, learning about neurological emergencies affecting the ability to ventilate.

Disorders of the peripheral nervous system, respiratory muscles, and chest wall lead to an inability to maintain a level of minute ventilation appropriate for the rate of carbon dioxide production. Concomitant hypoxemia and hypercapnia occur. Examples include Guillain-Barré syndrome, muscular dystrophy, myasthenia gravis, severe kyphoscoliosis, and morbid obesity. Which disorders of the peripheral nervous system (PNS), respiratory muscles, and chest wall lead to respiratory failure?
Updated: Apr 07, 2020

Author: Ata Murat Kaynar, MD; Chief Editor: Michael R Pinsky, MD, CM, Dr(HC), FCCP, FAPS, MCCM https://www.medscape.com/answers/167981-43844/which-disorders-of-the-peripheral-nervous-system-pns-respiratory-muscles-and-chest-wall-lead-to-respiratory-failure

Respiratory insufficiency due to neuromuscular disease is unique and differs from other types of respiratory failure.

Namely, it is characterized by ventilatory failure that manifests as hypercapnia (so-called type 2 respiratory failure or pump failure) and must be distinguished from hypoxic respiratory failure associated with intrinsic lung disease (type 1 respiratory failure).3 

The mainstay of treatment for neuromuscular respiratory failure is ventilation, as opposed to oxygenation; both noninvasive and invasive positive pressure can be considered.1,4

Other signs and symptoms of neuromuscular respiratory failure include changes in vital signs (i.e., tachycardia, tachypnea), breathlessness, staccato speech, use of accessory breathing muscles, orthopnea, paradoxical breathing, and weakness (particularly in neck and bulbar regions).1,4………………………………….One challenge to treating neuromuscular respiratory weakness is its association with a broad range of complex neurological conditions; thus, providers must be proficient in recognizing neuromuscular diseases based on clinical history and exam. Acute inflammatory demyelinating polyneuropathy (AIDP), also known as Guillain-Barré syndrome, is a prototypic neuromuscular disorder in which approximately 25% of patients develop respiratory failure. This condition is characterized by subacute ascending numbness, paresthesias, flaccid weakness, and areflexia. Diaphragmatic failure can occur suddenly, so it is important for clinicians to recognize the diagnosis and initiate early ventilator support.5,6……………………………….Specialized knowledge of neuromuscular diseases and hypercapnic respiratory failure is required to be able to accurately identify and manage this condition..Acute Neuromuscular Respiratory Weakness Due to Acute Inflammatory Demyelinating Polyneuropathy (AIDP): A Simulation Scenario for Neurology Providers MedEdPORTAL. 2019; 15: 10811. Published online 2019 Mar 1. doi: 10.15766/mep_2374-8265.10811PMCID: PMC6415011PMID: 30931390 Roshni Abee Patel,1,*Leighton Mohl,1Glenn Paetow,2 and  Samuel Maiser3,4

It is not unusual for a serious attack of hypercapnia to present with DELIRIUM.

Without careful assessment, delirium can easily be confused with a number of primary psychiatric disorders because many of the signs and symptoms of delirium are also present in conditions such as dementia, depression, and psychosis.

When Jerry unexpectedly became combative, paranoid and psychotic, he was brought to hospital. Emergency room doctors most likely noticed that his breathing was abnormal. Abnormal breathing is an important sign. Inadequate ventilation can lead to delirium. . Delirium in a young adult is immediately misdiagnosed as schizophrenia or manic depressive insanity without ever giving a second thought to the possibility of neuromuscular respiratory failure.

If anyone had bothered to examine Jerry’s vital signs including respiratory rate and pattern, they would have been shocked to find that he had Cheyennes-Stoke breathing.

Cheyne Stokes breathing is a type of abnormal breathing. It’s characterized by a gradual increase in breathing, and then a decrease. This pattern is followed by a period of apnea where breathing temporarily stops. The cycle then repeats itself.

Normal breathing, the process of moving air in and out of the lungs 12 to 20 times per minute, is something most people seldom think about. [Remember to read about Paula’s “normal” abnormal breathing.

However, abnormal breathing like Cheyne Stokes is serious and may be frightening

Causes of Cheyne Stokes breathing

Cheyne Stokes is usually related to heart failure or stroke. It may also be caused by:

  • brain tumors
  • traumatic brain injuries
  • high altitude sickness
  • encephalitis
  • increased intercranial pressure
  • chronic pulmonary edema

People who are dying often experience Cheyne Stokes breathing. This is a natural effect of the body’s attempt to compensate for changing carbon dioxide levels. While it may be distressing to those who witness it, there’s no evidence Cheyne Stokes is stressful for the person experiencing it. WHAT???? [ what about Cheyne Stokes associated with fear,anxiety, distress, agitation and psychosis????]

https://f36d41a36b7f6355d60d149a6ac8e6b6.safeframe.googlesyndication.com/safeframe/1-0-37/html/container.html

Jerry told the medical staff that his head felt like it was exploding. Most likely he was trying to explain the sensation of increased inter cranial pressure. No one listened. No one at all….

Kussmaul breathing vs. Cheyne Stokes

Both Kussmaul breathing and Cheyne Stokes breathing are characterized by fast breathing and too much carbon dioxide in the body, but that’s where their similarities end. Kussmaul breathing doesn’t alternate between fast and slow breathing or cause breathing to stop like Cheyne Stokes does. Instead, it’s characterized by a deep, rapid breathing pace throughout its duration. 

Kussmaul breathing is often caused by late-stage diabetic ketoacidosis. Diabetic ketoacidosis is a metabolic condition caused by a lack of insulin and too much glucagon in the body. Glucagon is a hormone produced by the pancreas that increases blood sugar. Kussmaul breathing may also be present in people with kidney failure. powered by Rubicon Project

Other abnormal respiration

Other types of abnormal respiration cause fast or slow breathing, such as:

Hyperventilation

When someone breathes deeply and too fast, it’s called hyperventilation. It leads to elevated levels of oxygen and low levels of carbon dioxide in the blood. The condition is often caused by anxiety, stress, or a panic attack. It may also be caused by excessive bleeding, heart disease, or a lung disease such as asthma. 

Left unchecked, hyperventilation may cause:

  • dizziness
  • lightheadedness
  • fainting
  • weakness
  • confusion
  • numbness in your arms or mouth
  • muscle spasms
  • chest pain
  • fast heart rate

Hypoventilation 

When someone breathes too slowly or too shallowly, it’s called hypoventilation. It leads to low oxygen levels and high levels of carbon dioxide in the blood. Hypoventilation may be caused by lung problems that obstruct the lower airways, such as emphysema, cystic fibrosis, or bronchitis.  Note from the writer of this Blog – Notice they forget to mention Neuromuscular respiratory pump failure which will cause only Hypercapnia and increased PC02.]

Symptoms of hypoventilation may include:

  • heart problems
  • being sleepy during the day
  • stomach problems
  • headaches
  • fainting

Obstructive sleep apnea

This condition causes you to stop breathing for 10 seconds or more while you sleep. Although everyone’s breathing pauses occasionally during sleep, people with obstructive sleep apnea stop breathing at least five times per hour. In severe cases, people may stop breathing every minute. 

Obstructive sleep apnea can happen to anyone, but it’s most common in people who are obese. Symptoms may include:

  • daytime sleepiness
  • waking up short of breath
  • morning headaches
  • mood changes
  • difficulty concentrating

Sleep apnea is treated with CPAP therapy and lifestyle changes such as weight loss. Left untreated, obstructive sleep apnea may lead to heart problems, and even death.

Outlook

Cheyne Stokes is serious. Since abnormal breathing often happens during sleep, it may be difficult to diagnose. [ A sleep study known as polysomnography is needed to diagnose sleep-related Cheyne Stokes and other forms of sleep apnea.- What????? ] ………..OR THE DOCTOR COULD TRY TO COUNT YOUR RESPIRATIONS FOR ONE MINUTE WITH THE HELP OF A STOPWATCH and would then definitely see the disturbed breathing without using any fancy or expensive toys.!!

Consult your doctor if you have symptoms of Cheyne Stokes, sleep apnea, or another type of abnormal breathing.……..

{ From the writer of this BLOG…. Here is why the above statement is often a RIDICULOUS statement!!…. You cannot know if you have abnormal breathing, especially if it is due to polyneuropathy or nerve damage [which is painless] and you are overcome with delirium and are not in your right mind}…..

https://www.healthline.com/health/cheyne-stokes Medically reviewed by Adithya Cattamanchi, MD — Written by Annette McDermott — Updated on April 28, 2017

Abnormal breathing and mental confusion are linked!

to be continued…

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