Coordination and Cognition in Pure Nutritional Wernicke's Encephalopathy with Cerebellar Degeneration after COVID-19 Infection: A Unique Case Report.

BACKGROUND: 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). METHODS: 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. RESULTS: We found a persistent ataxia of stance and gait and evidence for an irreversible restricted cerebellar degeneration. However, the initial cognitive impairments resolved. CONCLUSIONS: 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.

The contribution of mamillary body damage to Wernicke's encephalopathy and Korsakoff's syndrome.

Histopathological alterations of the mamillary bodies are the most conspicuous and the most consistent neuropathological features of several disorders that occur after severe thiamine deficiency, such as Wernicke's encephalopathy and Korsakoff's syndrome. Moreover, they are among the few abnormalities that are visible to the naked eye in these disorders. With a lifetime prevalence of approximately 1.3%, Wernicke's encephalopathy is by far the most frequent cause of damage to the mamillary bodies in humans. Still, there is a persisting uncertainty with regard to the development and the clinical consequences of this damage, because it is virtually impossible to study in isolation. As a rule, it always occurs alongside neuropathology in other subcortical gray matter structures, notably the medial thalamus. Converging evidence from other pathologies and animal experiments is needed to assess the clinical impact of mamillary body damage and to determine which functions can be attributed to these structures in healthy subjects. In this chapter, we describe the history and the current state of knowledge with regard to thiamine deficiency disorders and the contribution of mamillary body damage to their clinical presentations.