Pancreatic encephalopathy: a 7-year follow-up case report and review of the literature

Abstract

Pancreatic encephalopathy is a rare complication of acute pancreatitis. Clinical features include focal neurological signs and acute onset of dementia. This picture can fluctuate over time: cyclic progression with remission and relapses has been described. We present the case of a 43-year-old man who, after an acute episode of pancreatitis, experienced five relapses, with alternating focal signs. The patient has improved, but cognitive impairment persists after a 7-year follow-up.

Severe diffuse white matter lesions in a patient with pancreatic encephalopathy

“…In order to gain insight into the pathogenic mechanism of such a rare disorder, brain MRI is one of the indispensable methods with which to study clinically recovered patients. However, only two cases with brain MRI performed have been reported, of which one had small spotty abnormalities scattered in cortical white matter [4] and the other showed cortical and subcortical atrophy 7 years after onset [6]. Here we demonstrate the first brain MRI with acute substantial white matter lesions.…”

“…Since Rothermich and Von Haam described the first reported cases in 1941 [1], less than forty cases have been reported so far [1,2,4,6]. In order to gain insight into the pathogenic mechanism of such a rare disorder, brain MRI is one of the indispensable methods with which to study clinically recovered patients.…”

Young-Onset Dementia

“…81,82 Rare genetic and metabolic disorders can cause ataxia and cognitive decline such as NIA, 65 acerulopasminemia, 66 hereditary hemochromatosis, 67 Niemann Pick type C, 83 adult GM2 gangliosidosis, 70 Gaucher disease, 71 metachromatic leukodystrophy, 84 X-linked adrenoleukodystrophy, 85,86 Krabbe disease, 87 pigmentary orthochromatic leukodystrophyhereditary diffuse leukoencephalopathy with spheroids, 72 adultonset leukoencephalopathy with vanishing white matter disease, 88 familial British dementia, 89 mitochondrial disease, 90 familial Danish dementia, 91 Kufs disease, 73 sialidosis, 92 Lafora body disease, 93 adult polyglucosan body disease, 94 and cerebrotendinous xanthomatosis. 74,75 The association with pyramidal features suggests VaD, other causes of stroke (see below), AD with cotton wool plaques typically associated with deletions in exon 9 of presenilin-1 gene, 95 multiple sclerosis, 40 vitamin B12 deficiency, 76 SCA 1, 3, and 17, 68 NIA, 65 mitochondrial disease, 90 Fahr disease, 63 exposure to organic solvents, 42 Whipple disease, 34 prion diseases, 29 neurosyphilis, 78 SSPE, 31 neurosarcoidosis, 96 pancreatic encephalopathy, 49 Krabbe disease, 87 metachromatic leukodystrophy, 84 X-linked adrenoleukodystrophy, 85,86 pigmentary orthochromatic leukodystrophyhereditary diffuse leukoencephalopathy with spheroids, 72 adult-onset leukoencephalopathy with vanishing white matter disease, 88 familial British dementia, 89 Kufs disease, 73 cerebrotendinous xanthomatosis, 74,75 polyglucosan storage disease, 94 and polycystic lipomembranous leukodystroph...…”

“…A fluctuating level of cognitive function may be suggestive of DLB, 37 recurrent complex partial seizures, 4 paroxysmal cardiac arrhythmia, 38,39 remitting and relapsing inflammatory condition such as multiple sclerosis, 40 VGKCAb, 7 recurrent exposure to toxins such as alcohol 41 or recreational drugs, 42 an intermittent metabolic disturbance such as recurrent hypoglycemic episodes in treated poorly controlled diabetes mellitus (DM) or due to insulinoma, 43 hyperammonemic encephalopathy due to liver disease, 44 ureterosigmoidostomy, 45 valproate therapies, 45 ornithine transcobalamine deficiency 46,47 or citrullinemia (argininosuccinate synthetase deficiency), 48 pancreatic encephalopathy, 49 porphyria, 50 or recurrent hypoxia in the context of obstructive sleep apnoea. 51 Anxiety, depression, 52 and conversion disorders 53 may also cause a variable cognitive function.…”

Neurologinė Patologija Sergant Kasos Ligomis

“…43 metų vyras po ūminio pankreatito patyrė penkis encefalopatijos su besikeičiančiais židininiais simptomais paūmėjimus; ligonio būklė gydant pagerėjo, tačiau kognityvinė disfunkcija išliko dar septynerius metus [19]. PE diagnostikoje svarbi MRT.…”