Emotional Communication Disorders in Cerebellar Disease
Emotional Communication Disorders in Cerebellar Disease
The cerebellum has been linked to cognitive and emotional functions and there is increasing
 evidence that damage to posterior portions of the cerebellum can result in frontal-executive,
 visuospatial, and verbal deficits, including dysprosodia, and affective changes including
 blunting of affect or disinhibited and inappropriate behavior. Based on preliminary clinical
 observations and tests performed in the investigator's clinic, disorders of emotional
 communication may also be associated with cerebellar dysfunction. Emotional communication
 includes the production and comprehension of facial and prosodic expressions and is critical
 to maintaining positive and supportive relationships. Deficits in emotional communication can
 have devastating effects on relationships and on quality of life for those affected. Although
 deficits in affect and prosody have been reported in association with posterior cerebellar
 disorders, there are currently no studies systematically investigating emotional
 communication in individuals with cerebellar dysfunction. It is known that the cerebellum has
 strong connections with the cerebral cortex, especially the frontal lobes, and that cortical
 damage from stroke or neurodegenerative disease can result in disorders of emotional
 communication. Impairments in the integrity of cerebellar-cerebral networks from cerebellar
 disease may produce similar deficits in emotional communication. The purpose of this study is
 to systematically investigate and describe deficits in emotional communication in a series of
 patients with cerebellar disease. Participants will be individuals diagnosed with posterior
 cerebellar degeneration or damage from a non-hemorrhagic infarction, and age-matched
 neurologically healthy controls. Assessment will include a battery of tests of
 neuropsychological function as well as tests of emotional communication. Comprehension of
 emotional facial and prosodic expressions will be assessed as well as production of emotional
 communication. The expected outcomes will be to identify and describe deficits in production
 and comprehension of emotional prosodic and facial expressions and to describe the
 relationship between deficits in emotional communication and cerebellar atrophy with magnetic
 resonance imaging imaging (MRI) using voxel based morphometry (VBM).
The specific aims of this proposal will investigate the hypothesis that cerebellar damage is
 associated with emotional communication deficits.

 -  Determine whether posterior cerebellar damage results in deficits in the production or
 comprehension of emotional facial expressions and/or vocal prosodic expressions

 -  Determine whether posterior cerebellar damage results in alterations in intensity of
 reaction to emotionally evocative pictures or emotionally evocative words

 -  Investigate the relationship between performance on tests of emotional communication and
 left versus right cerebellar atrophy with magnetic resonance imaging imaging (MRI) using
 voxel based morphometry (VBM) analysis

 All subjects (cerebellar and control) will be given the following testing in a single session
 of lasting 3 to 3 and a half hours. Included will be tests to assess reading ability, naming
 ability, several tests of memory, a test of ability to discriminate between faces, tests of
 mental flexibility, and standardized questionnaires for depression, anxiety and emotional
 empathy. The tests include:

 1. Wechsler Test of Adult Reading

 2. Montreal Cognitive Assessment

 3. Beck Depression Inventory - 2

 4. Wisconsin Card Sorting test

 5. Toronto Alexithymia Scale

 6. Multi-Dimensional Emotional Empathy Scale

 All subjects will also be given tests assessing emotional communication ability. Tests of
 recognition and expression of emotional tones of voice and facial expression will be
 performed. Expression of facial expressions and tones of voice will be video recorded so that
 blinded raters can compare the accuracy and intensity of facial expressions and tones of
 voice between the participants. Training for the raters will include familiarization with the
 descriptions of features for each emotion with respect to changes in pitch, loudness, and
 rate for prosody and changes in facial features for facial expression. Both intra- and
 inter-judge reliability will be calculated for these blinded judges. To supplement this
 perceptual analysis, the participant's responses will be recorded using VisiPitch IV
 software, a clinical instrument that performs sound analyses and calculates the variation in
 the range of fundamental frequency (i.e., pitch variation) across each sentence. The ability
 to manipulate pitch to produce varying prosodic contours is the most salient acoustical
 feature of affective prosody.

 We will also assess intensity ratings and descriptions of emotional pictures and words using
 materials that have established rating norms. Participants will be shown a series of pictures
 and asked to rate the emotional intensity of the picture. They will also be asked to give a
 brief description of the picture. They will then be given a list of words, one at a time, and
 asked to rate the emotional intensity of these words.

 Some of the cerebellar participants will also be asked to participate in a magnetic resonance
 imaging (MRI) scan of the brain if the testing listed above shows deficits in understanding
 or producing emotional facial expressions or tones of voice and if there are no reasons that
 would prevent them from being able to enter a scanner (determined by a standard MRI screen
 form). Healthy control participants may also be asked to participate in a magnetic resonance
 imaging (MRI) scan of the brain and if there are no reasons that would prevent them from
 being able to enter a scanner. The imaging data will be used to investigate if there is a
 relationship between our experimental subjects' performance on tests of emotional expression
 and left versus right cerebellar atrophy-injury in the cerebellar lobules with MRI imaging
 using a voxel based morphometry (VBM) approach. The same data from controls will be used for
 comparison. Neuroimaging data collection will be done using a Philips Achieva 3T scanner
 (Amsterdam, Netherlands) and a 32-channel SENSE head coil. Imaging collected will include
 anatomical T1-weighted imaging scans. Structural MP-RAGE T1-weighted scans will be acquired
 with 130-1.0 mm sagittal slices, FOV=240 mm (AP)-180 mm (FH), matrix= 256-192, TR= 9.90 ms,
 TE= 4.60 ms, Flip Angle= 8, voxel size= 1.0x 0.94x 0.94 mm. Structural data will be analyzed
 with FSL-VBM, an optimized VBM protocol carried out with FSL tools. First, structural images
 will be brain-extracted and grey matter-segmented before being registered to the MNI 152
 standard space using non-linear registration The resulting images will be averaged and
 flipped along the x-axis to create a left-right symmetric, study-specific grey matter
 template. Second, all native grey matter images will be non-linearly registered to this
 study-specific template and "modulated" to correct for local expansion (or contraction) due
 to the non-linear component of the spatial transformation. The modulated grey matter images
 were then smoothed with an isotropic Gaussian kernel with a sigma of 6 mm. Finally, voxelwise
 GLM will be applied using permutation-based non-parametric testing, correcting for multiple
 comparisons across space.
Inclusion Criteria:

 -  Men and women over the age of 18

 -  Individuals with cerebellar ataxia

 Exclusion Criteria:

 -  Neurological signs reflecting major pathology in extrapyramidal, brainstem, cortical
 and visual systems

 -  Symptomatic cerebellar ataxia from alcohol, toxins, vitamin deficiency, and
 paraneoplastic cerebellar degeneration

 -  Dementia

 -  Current symptoms of depression or severe anxiety

 -  Currently on any psychotropic medication other than anti-depressants or history of a
 psychosis

 -  Severe impairments of hearing or vision

 -  Reading level less than 10th grade and/or history of learning disability

 -  Chronic medical and neurological diseases other than cerebellar (e.g., cardiac
 failure, renal disease, hepatic failure, stroke)

 -  History of severe head trauma (e.g., coma for more than one hour)