Neural correlates of adaptive working memory training in a glycogen storage disease type‐IV patient

Abstract

Glycogen storage disease type‐IV has varied clinical presentations and subtypes. We evaluated a 38‐year‐old man with memory complaints, common symptoms in adult polyglucosan body disease subtype, and investigated cognitive and functional MRI changes associated with two 25‐sessions of adaptive working memory training. He showed improved trained and nontrained working memory up to 6‐months after the training sessions. On functional MRI, he showed increased cortical activation 1–3 months after training, but both increased and decreased activation 6‐months later. Working memory training appears to be beneficial to patients with adult polyglucosan body disease, although continued training may be required to maintain improvements.

Review of the Neural Processes of Working Memory Training: Controlling the Impulse to Throw the Baby Out With the Bathwater

“…Other fMRI studies have reported increased activation following WMT ( 43 , 53 , 55 , 58 , 61 , 64 ). Olesen and colleagues used 3 CogMed tasks in a small sample of 8 healthy participants to evoke increased frontoparietal activations five weeks after training.…”

“…In another case study in line with Westerberg and Klingberg’s study in 2007—using WMT alongside fMRI—a 38-year old man with glycogen storage disease type IV and memory complaints was given CogMed training ( 61 ). He completed CogMed over 25 sessions, with each session consisting of eight verbal and visuospatial tasks, the difficulty adjusted based on the man’s daily performance over 25 weeks.…”

“…Various neuroimaging studies reported—alongside neural changes—far transfer effects of WMT to other un-related cognitive domains ( 20 , 43 , 56 , 60 , 61 , 75 , 82 , 87 ). Using fMRI, Dahlin and colleagues demonstrated significant transfer gains of an updating task (memorizing letter sequences) to working memory performance on the n-back task that was mediated by pre-training striatal activation.…”

Characterization of cognitive impairment in adult polyglucosan body disease

“…Being informed about attentional, executive or mnestic deficits are of significant value for caregivers and patients, as even mild deficits can have profound effects on abilities of daily living, social life, and working ability. Furthermore, detailed neuropsychological evaluation enables patient-tailored cognitive training, which has been shown to be effective in APBD in one patient [7].…”

“…Over 25 years ago, Rifai and colleagues (1994) performed a non-systematic review of cognitive symptoms of 24 published APBD cases and observed varying cognitive deficits, mainly in the domain of memory, in about half of the reported cases. A substantial number of new cases of APBD have been published in the last decades, some of them explicitly addressing cognitive impairment in APBD and providing detailed but heterogeneous results of neuropsychological measurements [4][5][6][7][8][9][10][11].…”