Home > Uncategorized > What is Cerebellar Cognitive Affective Syndrome?

What is Cerebellar Cognitive Affective Syndrome?

Editor’s note:  I have to admit that I had not heard of this disorder until I encountered a woman who has it.  This prompted me to do some research into this relatively new discovery.  Here’s what I found.

The cerebellum has traditionally been attributed primarily with aiding in the regulation of motor functions in the body.  Prior to the last few years, little attention has been paid to any effect it might have on non-motor activities.  However, in the late 1980s, a few scientists began to notice certain behaviors occurring in persons who had either sustained damage to the cerebellum, or who had been born with impairments in that region of the brain. (Recently, it has been recorded that these deficits are most likely caused by a dysfunction of the connections between the cerebellum and the cerebral cortex and the limbic system.)

It was noted that some patients who had sustained cerebellar degeneration, stroke, damage due to a traumatic injury, or removal of tumors, exhibited characteristic cognitive deficits which seemed to focus on difficulties with concept formation, learning of paired associations, and generalized intellectual slowing.  Cases were reported in both children and adults.  However, there continued to be very few descriptions of clinically relevant cases in the literature.

In 1998, Jeremy D. Schmahmann and Janet C. Sherman, of the Dept. Of Neurology, Massachusetts General Hospital and Harvard Medical School, published the results of a study in the journal Brain.  They studied 20 patients over a period of 7 years, aged 23 – 74 years, with various injuries to or disorders of the cerebellum.  They found several distinguishing features shared by the majority of these patients, including:

1.  Impairiment of executive functioning (planning, set shifting, abstract reasoning, verbal fluency, and working memory — often with perseveration, distractibility or inattention, visuo-spatial disorganization and impaired visual-spatial memory)
2.  Personality changes and blunting of affect, or disinhibited and inappropriate behavior.
3.  Difficulties with language production (including dysprosodia, aggramatism, and mild anomia).

Dr. Schmahmann and his colleagues also noted that the net effect of the above deficits often produced a general impression of decreased intellectual functioning, which was more pronounced in patients with bilateral damage or were in a more acute stage of the disease process.  Posterior lobe lesions were found to be particularly important in the generation of the syndrome, with the vermix being consistently involved.

Other researchers have documented the presence of the following symptoms in persons who have CCAS, to one extent or another:

1.  Distractibility
2.  HYperactivity
3.  Impulsiveness
4.  Disinhibition
5.  Anxiety
6.  Ritualistic and stereotypical behaviors
7. Illogical thought
8.  Lack of empathy
9.  Aggression
10.  Irritability
11.  Ruminative and obsessive behaviors
12.  Dysphoria and depression
13.  Tactile defensiveness and sensory overeload
14.  Apathy
15.  Childlike behavior
16.  Inabillity to comprehend social boundaries

While there are very few of these cases currently recorded in the literature, it is believed that more will be discovered in the coming years.  Most likely, there are a number of persons out there who have been diagnosed with other disorders, due to physician’s lack of familiarity with the sydrome as well as the nature and variety of symptoms.  Some have noted the similarity between behaviors exhibited by persons with CCAS and some psychiatric disorders.  However, like most new diagnoses, as time goes by there will likely be more individuals found who have the disorder.  This is much like Lewy Body Disease, which was not generally known prior to the last 20-30 years, but which now ranks as one of the more common forms of dementia.

There is currently no known treatment for CCAS, other than treatment of symptoms.  Some remedies that have been proposed include Cognitive Behavioral Therapy and Transcranaial Magnetic Stimulation.  Others have utilized exercises to relieve the motor symptoms, in the belief that the cognitive symptoms will also be improved.  It has been noted, with some persons, that some symptoms may improve in time without any direct intervention.

Much research needs to be done with regards to this disorder.

Schmahmann, Jeremy D., and Sherman, Janet C., Brain, 1998.

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