Arts Nicolaas J M, van Dorst Maud E G, Vos Sandra H, Kessels Roy P C
Centre of Excellence for Korsakoff and Alcohol-Related Cognitive Disorders, Vincent van Gogh Institute for Psychiatry, 5803 DN Venray, The Netherlands.
Winkler Neuropsychiatry Clinic and Korsakoff Centre, Pro Persona Institute for Psychiatry, 6874 BE Wolfheze, The Netherlands.
J Clin Med. 2023 Mar 27;12(7):2511. doi: 10.3390/jcm12072511.
Alcoholic cerebellar degeneration is a restricted form of cerebellar degeneration, clinically leading to an ataxia of stance and gait and occurring in the context of alcohol misuse in combination with malnutrition and thiamine depletion. However, a similar degeneration may also develop after non-alcoholic malnutrition, but evidence for a lasting ataxia of stance and gait and lasting abnormalities in the cerebellum is lacking in the few patients described with purely nutritional cerebellar degeneration (NCD).
We present a case of a 46-year-old woman who developed NCD and Wernicke's encephalopathy (WE) due to COVID-19 and protracted vomiting, resulting in thiamine depletion. We present her clinical course over the first 6 months after the diagnosis of NCD and WE, with thorough neuropsychological and neurological examinations, standardized clinical observations, laboratory investigations, and repeated MRIs.
We found a persistent ataxia of stance and gait and evidence for an irreversible restricted cerebellar degeneration. However, the initial cognitive impairments resolved.
Our study shows that NCD without involvement of alcohol neurotoxicity and with a characteristic ataxia of stance and gait exists and may be irreversible. We did not find any evidence for lasting cognitive abnormalities or a cerebellar cognitive-affective syndrome (CCAS) in this patient.
酒精性小脑变性是小脑变性的一种局限性形式,临床上可导致站立和步态共济失调,发生于酒精滥用合并营养不良和硫胺素缺乏的情况下。然而,类似的变性也可能在非酒精性营养不良后发生,但在少数描述为单纯营养性小脑变性(NCD)的患者中,缺乏站立和步态持续性共济失调以及小脑持续性异常的证据。
我们报告一例46岁女性患者,因新型冠状病毒肺炎和持续性呕吐导致硫胺素缺乏,进而发生了NCD和韦尼克脑病(WE)。我们介绍了她在被诊断为NCD和WE后的前6个月的临床病程,包括全面的神经心理学和神经学检查、标准化临床观察、实验室检查以及多次磁共振成像检查。
我们发现存在持续性站立和步态共济失调以及不可逆的局限性小脑变性的证据。然而,最初的认知障碍得到了缓解。
我们的研究表明,不存在酒精神经毒性且具有特征性站立和步态共济失调的NCD是存在的,并且可能是不可逆的。在该患者中,我们未发现任何持续性认知异常或小脑认知情感综合征(CCAS)的证据。