Disorders of the Cerebellum: Ataxia, Dysmetria of Thought, and the Cerebellar Cognitive Affective Syndrome

2004 ◽  
Vol 16 (3) ◽  
pp. 367-378 ◽  
Author(s):  
Jeremy D. Schmahmann
Keyword(s):  
Cerebellar Cognitive Affective Syndrome ◽  
Dysmetria Of Thought

Cerebellar Cognitive Affective Syndrome in Costa da Morte Ataxia (SCA36)

2020 ◽  
Vol 19 (4) ◽  
pp. 501-509
Author(s):  
R. Martínez-Regueiro ◽  
M. Arias ◽  
R. Cruz ◽  
B. Quintáns ◽  
T. Labella-Caballero ◽  
...  
Keyword(s):  
Cerebellar Cognitive Affective Syndrome

Cerebellar cognitive-affective syndrome preceding ataxia associated with complex extrapyramidal features in a Turkish SCA48 family

Neurogenetics ◽  
2019 ◽  
Vol 21 (1) ◽  
pp. 51-58 ◽  
Author(s):  
R. Palvadeau ◽  
Z. E. Kaya-Güleç ◽  
G. Şimşir ◽  
A. Vural ◽  
Ö. Öztop-Çakmak ◽  
...  
Keyword(s):  
Cerebellar Cognitive Affective Syndrome ◽  
Extrapyramidal Features

scholarly journals Cerebellar Cognitive Affective Syndrome Presented as Severe Borderline Personality Disorder

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Danilo Pesic ◽  
Amir Peljto ◽  
Biljana Lukic ◽  
Maja Milovanovic ◽  
Snezana Svetozarevic ◽  
...  
Keyword(s):  
Borderline Personality Disorder ◽  
Personality Disorder ◽  
Clinical Manifestations ◽  
Early Learning ◽  
Borderline Personality ◽  
Cerebellar Cognitive Affective Syndrome ◽  
Resonance Imaging ◽  
Cerebellar Dysfunction ◽  
Affective Instability ◽  
Polysubstance Abuse

An increasing number of findings confirm the significance of cerebellum in affecting regulation and early learning. Most consistent findings refer to association of congenital vermis anomalies with deficits in nonmotor functions of cerebellum. In this paper we presented a young woman who was treated since sixteen years of age for polysubstance abuse, affective instability, and self-harming who was later diagnosed with borderline personality disorder. Since the neurological and neuropsychological reports pointed to signs of cerebellar dysfunction and dysexecutive syndrome, we performed magnetic resonance imaging of brain which demonstrated partially developed vermis and rhombencephalosynapsis. These findings match the description of cerebellar cognitive affective syndrome and show an overlap with clinical manifestations of borderline personality disorder.

25 Cerebellar cognitive affective syndrome – a case report of diagnosis and management using selective serotonin and norepinephrine reuptake inhibitors

2020 ◽  
Vol 91 (8) ◽  
pp. e18.1-e18
Author(s):  
B Sridharan ◽  
YW Liao
Keyword(s):  
Case Report ◽  
Emotional Regulation ◽  
Complex Disease ◽  
Functional Independence ◽  
Selective Serotonin ◽  
High Dose ◽  
Cerebellar Cognitive Affective Syndrome ◽  
History Of ◽  
Cerebellar Haemangioblastoma ◽  
Norepinephrine Reuptake

Objectives/aimsThe objective of this case is to describe and report on a case of Cerebellar Cognitive Affective Syndrome (CCAS) which improved after the use of high dose venlafaxine therapy. To our knowledge, there are only a few case reports discussing on the effectiveness of various drug therapies in CCAS patients and none had reported on the use of Selective Serotonin and Norepinephrine Reuptake Inhibitors (SNRI).MethodsWe describe a 55-year-old male with a history of partially excised grade 1 left cerebellar haemangioblastoma in 2005 followed by Gamma Knife in 2006.Unfortunately, he suffered a recurrence in 2018 requiring embolisation of the haemangioblastoma followed with surgery to resect the tumour. He presented to our specialist neuropsychiatric unit following episodes of new acute unprovoked behavioural changes after surgery. During these episodes, he would become verbally abusive, refuse to engage in any rehab activity, refuse any oral intake, become sexually disinhibited, and experience distressing hallucinations. On the neuropsychiatric ward, he continued to have episodes of severe emotional regulation difficulties. There were no obvious triggers identified and it was noted that the peaks and troughs in his behaviour lasted for a few days before self-resolving and did not follow any particular pattern. In between these episodes, the patient was pleasant, engaging in physiotherapy, and conversed normally with staff.ResultsHe scored a total of 4 out of 10 on the CCAS-scale. A diagnosis of CCAS was concluded given the extensive history of cerebellar injury, nature of presenting complain, and his CCAS-scale score. He was started on high dose SNRI (Venlafaxine XL 150 mg BD). On repeating the CCAS-scale after being on SNRI for 4 months, his score was worse at 6 out of 10. However, we observed a decrease in the frequency, duration, and severity of behavioural change after commencement of SNRI. He benefited greatly from physiotherapy on the ward however despite our best efforts functional independence was not regained. He had to be transferred with the aid of 2 members of staff and mobilises with the aid of an electronic wheelchair.ConclusionsCCAS is a complex disease and the management is yet to be agreed on by the neuropsychiatry community. Our case report illustrated the therapeutic benefit of venlafaxine in the treatment of severe emotional regulation difficulties associated with resection of a cerebellar haemangioblastoma. Furthermore, patients with cerebellar injuries should be managed in a multi-disciplinary manner with input from neurology, neuropsychiatry, neuropsychology, occupational therapist, and physiotherapist.

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