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Curren Foundation

The Basics of PNS and PCD

Paraneoplastic Syndrome (PNS) describes a broad group of uncommon disorders in which a person’s immune system responds to cancer by attacking not only cancerous cells but also healthy cells. A useful analogy might be that of “friendly fire” in warefare: one side’s own bombers, for example, drop bombs not on the enemy but mistankly on its own troops; or a police officer is shot by another officer by accident. The following are among the types of PNS that have been identified:

Paraneoplastic Cerebellar Degeneration

PCD describes a particularly devastating disorder that is a subset of PNS. It is associated with ovarian and other gynecological cancers, as well as breast cancer, some blood cancers, and small cell lung cancer. It is confirmed by a finding of the anti-yo antigen. It is most prevalent in women. 

 

In the case of PCD, “killer T cells” attack both the underlying cancer and the cerebellum, the part of the brain that governs motor skills and muscle functions (essentially all body movements – walking, speaking, swallowing, hand movements, maintaining trunk posture, and even eye movement). At an advanced stage, a victim can be totally incapable of communicating, eating, walking, and eyesight is blurred. The combination of these impairments leads to dizziness. Involuntary body movements are also commonplace, ranging from trembling to more severe forms, and the victim has no ability to control those movements.

 

The victim’s other mental attributes (the cognitive functions an dmemory, for example) are generally unimpaired. PCD leads to the terribly sad circumstance of a patient knowing exactly what is happening to her but being unable to do anything about it or even to let anyone know ow she feels or what she needs. The comfor that many critically ill patients may find in conversation with loved ones and the sharing of memories, does not occur. Alas, a Lou Gehrig’s disease presents similar problems for its victims. 

Limbic Encephalitis

This is inflammatio affecting a region of the brain known as the limbic system, which controls emotions, behaviors and certain memory functions. People with this disorder may experience personality changes or mood disturbances, memory loss, seizures, hallucinations, or drowsiness.

Encephalomyelitis

This syndrome refers to inflammation of the brain and spinal cord. There may be a wide variety of symptoms and signs depending on the area affected. 

Opsoclonus-myoclonus

This syndrome includes rapid, irregular eye movements (opsoclonus) and involuntary, chaotic muscle jerks (myoclonus) in your limbs and trunk caused by cerebellar dysfunction. 

Stiff Person Syndrome

Previously called Stiff Man Syndrome, this syndrome is characterized by progressive, severe muscle stiffness or rigidity, mainly affecting your spine and legs. It may also cause painful muscle spasms. 

Myelopathy

This term refers to a syndrome of injury limited to the spinal cord. Depending on the level of spinal cord injury, you may have changes in bowel and bladder function, and severe weakness and numbness up to a certain level in your body. If the level of injury includes your neck, you can have severe disability affecting all four limbs.

Lambert-Eaton Myasthenic Syndrome

This is a syndrome caused by disrupted communication between nerves and muscles. Signs and symptoms include pelvic and lower extremity muscle weakness, fatigue, difficulty swallowing, difficulty speaking, irregular eye movements, and double vision. Autonomic nervous system problems can include dry mouth and impotence.

Myasthenia Gravis

Myasthenia Gravis is also related to disrupted communcation between nerves and muscles. It’s also characterized by weakness and rapid fatigue of any of the muscles that are under voluntary control, including muscles in your face, eyes, arms and legs. The muscles involved in chewing, swallowing, talking, and breathing may be affected as well. 

Neuromyotonia

Also known as Isaac’s Syndrome, Neuromyotonia is characterized by abnormal impulses in nerve cells outside the brain and spinal cord (peripheral nerve hyperexcitability) that control muscle movement. These impulses can cause twitching, muscle rippling that looks like a “bag of worms,” progressive stiffness, muscle cramps, slowed movement and other muscle impairments.

Peripheral Neuropathy

This condition refers to patterns of damage to nerves that transmit messages from the brain or spinal column to the rest of your body. When damage involves only the sensory nerves of the peripheral nervous system, you can have pain and disturbances in sensation anywhere in your body. 

Dysautonomia

Dysautonomia refers to a wide range of signs and symptoms resulting from injury to the nerves that regulate involuntary body functions (autonomic nervous system), such as heart rate, blood pressure, sweating, and bowel and bladder functions. When this part of the nervous system is affected, common symptoms are low blood pressure, irregular heartbeat, and trouble breathing.

The consequences of PNS range from mild to extremely severe, depending upon the severity and duration of the attack and the type of cells attacked. 

The Importance of Early Diagnosis

Early detection of PNS symptoms can be pivotal in the detection of cancer as the symptoms often precede other cancer indicators. Detection and treatment of the underlying cancer is necessary to the immune system to cease its attacks. 

Prolonged attack on the nervous system could lead to irreversible deficits including inability to communicate verbally or in writing, walking, seeing clearly, swallowing, and more. The victim, in severe cases, be totally dependent upon the care of others, sometimes around the clock. This is devastating not only to the victim but to her family.

and Rapid treatment

Time is of the essence, therefore, both in early diagnosis and speedy treatment of the cancer and parallel efforts to reduce attacks on the cerebellum. The earlier the diagnosis and treatment, the better are the chances of a successful outcome. This, in turn, requires much effort and careful coordination of all medical treatment. 

Signs and Symptoms of PNS

  • Unsteady or impaired walking and difficulty maintaining balance (ataxia)
  • Loss of muscle coordination in the limbs
  • Inability to maintain trunk posture
  • Dizziness
  • Loss of fine motor skills, such as picking up objects or handwriting
  • Difficulty swallowing
  • Slurred speech
  • Vision problems and/or involuntary eye movements (nystagmus)
  • Sleep disturbances