Acta Neurologica Belgica https://doi.org/10.1007/s13760-019-01265-6 NEURO-IMAGES Cerebellar cognitive affective syndrome after bilateral caudal paramedian midbrain infarction Kui Chen1,2 · Li Wu1 · Wei Chen1 Received: 27 August 2019 / Accepted: 12 December 2019 © Belgian Neurological Society 2020 Keywords Cerebellar cognitive affective syndrome · Ataxia · Midbrain infarction · Wernekink commissure syndrome A 71-year-old man complained of sudden-onset vertigo, unsteady gait and slurred speech with gradual worsening in one day. Upon admission to the hospital, neurological examination revealed dysarthria, vertical nystagmus, adduction impairments of both eyes and bilateral cerebellar ataxia. Cerebellar functions were semi-quantified by International Cooperative Ataxia Rating Scale (ICARS) and Brief Ataxia Rating Scale (BARS) with scores of 56/100 and 26/30 separately. Besides, somnolence, obvious memory loss and attention impairments were also noted through clinical examination. On the first night of admission, the patient developed more neuropsychic symptoms, including disorientation, emotional instability, and impulsive behavior. In the next few days, the patient’s mental state fluctuated daily, with new manifestations such as depression and persecutory delusion. His deficits in cerebellar function, cognition and affect reminded us the possibility of cerebellar cognitive affective syndrome (CCAS). This patient with an educational level of 15 years scored 61/120 in CCAS scale [1], and failed in six items including phonemic fluency, category switching, cube draw and copy, verbal recall, similarities and affect, supporting a diagnosis of definite CCAS. The cranial magnetic resonance imaging, performed 2 days after onset of the neurological symptoms, showed an acute infarction in bilateral paramedian region of caudal midbrain without any Kui Chen and Li Wu contributed equally to this work. * Wei Chen david_chen8106@hotmail.com 1 Department of Neurology, Shanghai Ninth People’s Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai 200011, China 2 Department of Neurology, Shanghai Tenth People’s Hospital, School of Medicine, Tongji University, Shanghai 200072, China supratentorial lesion (Fig. 1a and b). To clarify the cause of his mental disorder, 18F-fluorodeoxyglucose (FDG) PET was conducted and significant bilateral cerebellar hypermetabolism and frontal hypometabolism were disclosed (Fig. 1c and d). During hospitalization, antipsychotic medications (sertraline and quetiapine) provided some symptomatic relief for emotional instability and delusion. Two weeks later, the patient was discharged from the hospital to the rehabilitation center for further rehabilitation therapy. We present a case of Wernekink commissure syndrome with CCAS after bilateral caudal paramedian midbrain infarction. Wernekink commissure syndrome is a rare midbrain syndrome characterized by tetra-ataxia, eye movement disorders and palatal myoclonus, due to the lesion at the Wernekink commissure involving the decussation of superior cerebellar peduncle in midbrain [2]. However, rare previously reported patients with Wernekink commissure syndrome presented with cognitive or affective dysfunction. In this report, the patient presented with impairments in executive function, spatial cognition, linguistic skills and affect regulation, which were the core features of CCAS [3]. The typical CCAS is caused by the damage in the cerebellar posterior lobe, also called cognitive cerebellum, and reflect the dysmetria of cognition and affect [4]. In the past decades, cognitive and affective deficits have also been reported in cases with isolated brainstem damage, among which some cases disclosed frontal hypoperfusions through SPECT imaging [5]. It seems that the damage in the brainstem could lead to cortical dysfunction via destroying some association with cerebral network. In our case, the patient’s FDG PET imaging indicated functional suppression of frontal areas (Fig. 1c and d), which may be caused by crossed cerebellar–cerebral diaschisis after impairment of Wernekink commissure. Further studies to explore the role of brainstem, cerebellum and cerebrum in modulating cognitive and affective 13 Vol.:(0123456789) Acta Neurologica Belgica Author contributions KC: study concept and design, patient management, literature review, drafting/revising the manuscript. LW: patient management, literature review, revising the manuscript. WC: study concept and design, critical review of manuscript, final approval of the version to be published. Compliance with ethical standards Conflict of interest The authors declare that they have no conflict of interest. Ethical approval All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent Informed consent was obtained from this patient. References Fig. 1  Neuroimage summary of the patient. Cranial MRI revealed a hyperintense midbrain lesion on diffusion-weighted imaging (a white arrow) with decreased apparent diffusion coefficient (b white arrow).18F-FDG PET showed increased FDG uptake in the bilateral cerebellum (c marked in red) and decreased metabolism in bilateral lateral and paramedian frontal cortex (d marked in blue) (color figure online) processes would help to develop better treatment strategy for patients with midbrain damage. 13 1. Hoche F, Guell X, Vangel MG, Sherman JC, Schmahmann JD (2018) The cerebellar cognitive affective/Schmahmann syndrome scale. Brain 141(1):248–270 2. Zhou C, He Y, Chao Z, Zhu Y, Wang P, Wang X, Liu S, Han W, Wang J (2017) Wernekink commissure syndrome secondary to bilateral caudal paramedian midbrain infarction presenting with a unique “heart or V” appearance sign: case report and review of the literature. Front Neurol 8:376 3. Schmahmann JD, Sherman JC (1998) The cerebellar cognitive affective syndrome. Brain 121(Pt 4):561–579 4. Schmahmann JD (2019) The cerebellum and cognition. Neurosci Lett 688:62–75 5. D’Aes T, Marien P (2015) Cognitive and affective disturbances following focal brainstem lesions: a review and report of three cases. Cerebellum 14(3):317–340 Publisher’s Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.