©2005 by Alan Cocchetto, NCF Medical Director

 
Let me begin by saying that I don't find the task of writing this column particularly easy - discussing the description and findings of a longtime PWC/ME who died recently. It seems like
every time you look closely at someone with this disease, you see immense suffering. As I have noted in previous columns that I have written, there unfortunately appears to be no limit as to
the human toll that this disease is capable of exacting on patients.

 

When I first began to read the autopsy report and death certificate for this PWC/ME, something initially caught my eye. On a line that read "Enter other significant conditions contributing to death..." were the words "Chronic Fatigue Syndrome and Fibromyalgia" written by the medical examiner. Perhaps this would help change the minds of people who don't
believe in the seriousness of this disease - the doubters. As to this PWC/ME's determined cause of death: Seizure disorder, rhabdomyolysis, acute renal failure and shock.

 

Our patient is a longtime PWC/ME who had been seen by a battery of physicians. Like this patient, many have been on various pain medications that are quite common for those with this disease. Upon arrival at the hospital, they developed multisystem organ failure secondary to severe rhabdomyolysis. Acute renal failure and subsequent shock led to their death.

 

Other observations that were made during the autopsy examination:

* Externally:
   Edema of the lower extremities was found.

* Internally:

Pulmonary - The right lung weight was approximately twice that of the left lung. The right lung was found to have many adhesions along with fluid in that lung.

Gastrointestinal - The liver had marked congestion. There was evidence of ischemic (insufficient blood supply) bowel. (Intestinal ischemia is the death of part of the intestine after its blood supply is cut off. The hallmark of intestinal ischemia is abdominal pain. Vomiting, diarrhea, and in some cases, fever, are also seen.)

Genitourinary - The right kidney appeared shock-like and pale.

Head and Central Nervous System - There was a cystic defect in the right occipital lobe. Furthermore, the white matter surrounding this defect appears puckered and has a brownish discoloration.

* Microscopic description:

Lung - There were small emboli scattered throughout the arteries. The alveolar spaces were filled with inflammatory cells. There was marked congestion and there were scattered fibrotic nodules within the lymph nodes. One nodule was calcified.

Liver - There was marked congestion of the sinusoids.

Spleen - There was marked congestion of the red pulp.

Kidney - There was mild inflammation.

Bowel - Ischemic

Bladder - Hyperplastic epithelium

Thyroid - There were varied sized colloid filled follicles. There were scattered dystrophic calcifications and calcifications of
small arterial walls.

Head - Cystic region of right occiput showed areas of degeneration with multiple blue lamellar bodies. There were degenerated astrocytes. These findings are non-specific and can be seen in a variety of degenerative disease processes.

 

The medical examiner's opinion was that this patient died as a result of an underlying undiagnosed seizure disorder with a subsequent fall and rhabdomyolysis that led to multisystem organ failure and ultimately to their death. The examiner's pathologic diagnoses included (1) epilepsy with (A) rhabdomyolysis secondary to seizure, (B) acute renal failure, (C) congested liver as well as (2) right pleural effusion with adhesions and pulmonary edema.

 

Let me discuss a few of the major findings from this medical report since it may help to assist in our understanding of what happened to this PWC/ME:

 

Epilepsy has been recognized for over two-thousand years. It is derived from the Greek word "epilepsia" meaning "to come upon, to be grabbed hold of or thrown down, to attack, to
seize hold of." In 1861, Hughlings Jackson first developed the theory that seizures were caused by excessive discharge of the gray matter of the brain. Today, epilepsy is viewed as a symptom of disturbed electrical activity in the brain caused by a wide variety of disorders.

 

Epilepsy is a general name given to the wide range of symptoms that reflect the many functions of the brain in a pathologically disturbed manner. It is a collection of many different types of seizures that vary widely in severity, appearance, cause, consequence and management. Epilepsy implies a periodic recurrence of seizures with or without convulsions. Seizures that are prolonged or repetitive can be life threatening. Seizures occur because small numbers of neurons discharge abnormally. Anything that disrupts the normal homeostasis of the neuron and disturbs its stability may trigger abnormal activity and seizures.

 

Many factors have been shown to precipitate seizures in susceptible individuals. Hyperventilation may precipitate absence seizures. Sleep, sleep deprivation, sensory stimuli
and emotional stress may initiate seizures. However, the most clearly established risk factors for epilepsy are severe head trauma, central nervous system infections and stroke. Certainly,
anyone who has personally known many PWC/ME's will tell you stories of patients who have had seizures. This is not uncommon with this disease and may be indicative of metabolic or physical imbalances that exist in the brain.

 

Rhabdomyolysis is the breakdown of muscle fibers resulting in the release of muscle fiber contents into the circulation. Some of these are toxic to the kidney and frequently result in kidney damage.

 

Myoglobin is an oxygen-binding protein pigment found in the skeletal muscle. When the skeletal muscle is damaged, the myoglobin is released into the bloodstream. It is filtered out of
the bloodstream by the kidneys. Myoglobin may occlude the structures of the kidney causing damage that may lead to kidney failure. In addition, myoglobin breaks down into potentially
toxic compounds that also cause kidney failure as well. This destructive process may ultimately lead to shock because of alterations in circulating fluid volume and reductions in blood flow to the kidneys. The general symptoms of rhabdomyolysis include abnormal urine color, muscle tenderness or weakness of the affected muscles, muscle stiffness or aching as well as generalized weakness. Additional symptoms include unintentional weight gain, seizures, joint pain and fatigue.

 

Acute kidney failure is defined as the sudden loss of the ability of the kidneys to excrete wastes, concentrate urine and conserve electrolytes. The general symptoms associated with kidney failure include urination that can be excessive at night, swelling of the ankle, feet or leg, generalized swelling and fluid retention, decrease in sensation especially in the hands or feet, changes in mental status or mood, hand tremors, flank pain, fatigue, ear noise/buzzing, breath odor and high blood pressure.

 

Shock is a life-threatening condition that occurs when the body is not getting enough blood flow. This can damage multiple organs. Shock requires immediate medical treatment and
can get worse very rapidly. Thus shock can be caused by any condition that reduces blood flow. Symptoms associated with shock are anxiety or agitation, confusion, low or no urine output, bluish lips and fingernails, dizziness or light-headedness, profuse sweating - moist skin, shallow breathing, chest pain and unconsciousness.