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.
The electrophysiological features of X-linked juvenile retinoschisis in a young male: a case report
