Paraneoplastic degeneration of the cerebellum is a disease of the nervous system in the course of which the destruction of Purkinje cells - the neurons of the cerebellar cortex, which are the largest neurons in the human brain. What are the causes and symptoms of paraneoplastic degeneration of the cerebellum? What is the treatment?

Paraneoplastic cerebellar degeneration(paraneoplastic cerebellar degeneration - PCD) is a disease of the nervous system in the course of which the destruction of Purkinje cells - neurons of the cerebellar cortex, i.e. cells capable of processing and conducting information in the form of an electric signal.

Paraneoplastic cerebellar degeneration belongs to the neurological paraneoplastic syndromes - a group of nervous system disorders that occur in cancer patients but are not caused by metastases or local direct tumor activity. Paraneoplastic degeneration of the cerebellum belongs to the so-called classic neurological paraneoplastic syndromes, i.e. those that often coexist with cancer.

Paraneoplastic degeneration of the cerebellum - causes

The causes of paraneoplastic degeneration of the cerebellum, as well as other diseases belonging to the group of neurological paraneoplastic syndromes, are unknown. It is believed that the antibodies that the body produces to fight cancer cells by mistake also attack and destroy structures of the nervous system - in this case, Purkinje cells. These are onconeural antibodies.

Paraneoplastic degeneration of the cerebellum is most often associated with anti-Yo onconeuronal antibodies and occurs in women with breast and ovarian cancer.

Other antibodies associated with paraneoplastic degeneration of the cerebellum are anti-Hu (found in patients with lung cancer), anti-CV2 / CRMP5 (in patients with small cell lung cancer), anti-Tr (in patients with Hodgkin's disease), anti-Ri (in patients with breast cancer, neoplasms of the genital tract, lungs and bladder), anti-mGluR1 (in patients with Hodgkin's disease).

Paraneoplastic degeneration of the cerebellum - symptoms

The disease usually has a sudden onset and causes symptoms such as:

  • progressive ataxia (ataxia) of the trunk and limbs
  • dysarthria - is a speech disorder. The so-called is characterized by dysarthriabeing slow, indistinct and aphonic (meaning noiseless)
  • nausea
  • dizziness
  • double vision
  • nystagmus in all viewing directions
  • swallowing disorders
  • emotional lability and memory loss (in about 20% of patients)

The disease leads to complete disability. Only about 30 percent. patients can walk unassisted, and many cannot write, eat or swallow by themselves.

Paraneoplastic degeneration of the cerebellum - diagnosis

In the event of suspicion of paraneoplastic degeneration of the cerebellum, blood tests are performed for the above-mentioned autoantibodies, especially anti-Yo antibodies. If anti-Yo antibodies are detected, screening should be performed to rule out ovarian cancer (the cause of 46% of anti-Yo-related PCD) and breast cancer (underlying 24% of cases of this syndrome), and other cancers ( associated with a specific antibody).

There is also a test of the cerebrospinal fluid, which shows pleocytosis (the presence of an increased number of cells of a given type), increased protein concentration, and in 60% of sick - presence of oligoclonal bands (normally they should not be present).

Tomography or other neuroimaging tests show no abnormalities as cerebellar atrophy occurs in the later stages of the disease.

Paraneoplastic degeneration of the cerebellum - treatment

The cancer should be identified and treated first.

The patient also undergoes immunomodulation treatment with intravenous immunoglobulins, steroids, or plasmapheresis, but this is usually not effective ( although some patients have been reported to improve their he alth).

Symptomatic treatment includes rehabilitation with speech therapists in cases of dysarthria and swallowing disorders, and the use of propranolol and clonazepam.

Bibliography:

1. Ruddy Kathryn J., Hochberg Fred H., Paraneoplastic neurological syndromes, transl. Daniluk I. http://www.czelej.com.pl/images/file/Onkologia/421-423%20od7563-008_book-4.pdf 2. Michalak S., Kozubski W.,Neurological paraneoplastic syndromes , "Polski Przegląd Neurologiczny" 2008, vol. 4, no. 1

Category:

Neurology