No abstract

Primary Whipple disease of the Central Nervous System is a rare entity whose outcome might be fatal if not promptly diagnosed and treated. Few cases are reported in the literature with heterogeneous clinical and radiological presentations which often make the diagnosis extremely challenging. We report a case of primary Whipple disease of the Central Nervous System presenting with rhombencephalitis in a female patient in immunosuppressive treatment for rheumatoid arthritis. We describe the management of our patient and discuss the features of this rare clinical entity.

Background and purpose: Late-onset (LO) and early-onset (EO) dementia show neurobiological and clinical differences. Clinical and 18 fluoro-deoxy-glucose positron emission tomography (FDG-PET) features of LO and EO posterior cortical atrophy (LO_PCA, EO_ PCA), the visual variant of Alzheimer's disease (AD), were compared. LO_PCA patients were also compared with a group of patients with LO typical AD (tAD).Methods: Thirty-seven LO_PCA patients (onset age ≥ 65 years), 29 EO_PCA patients and 40 tAD patients who all underwent a standard neuropsychological battery were recruited; PCA patients were also assessed for the presence of posterior signs and symptoms. Brain FDG-PET was available in 32 LO_PCA cases, 23 EO_PCA cases and all tAD cases, and their scans were compared with scans from 30 healthy elderly controls. Within the entire PCA sample FDG uptake was also correlated with age at onset as a continuous variable. Results:The main difference between the two PCA groups was a higher prevalence of Bálint-Holmes symptoms in EO cases, which was associated with the presence of severe bilateral occipito-temporo-parietal hypometabolism, whilst LO_PCA patients showed reduction of FDG uptake mainly in the right posterior regions. The latter group also showed mesial temporal hypometabolism, similarly to the tAD group, although with a right rather than left lateralization. Correlation analysis confirmed the association between older age and decreased limbic metabolism and between younger age and decreased left parietal metabolism. Conclusions:The major involvement of the temporal cortex in LO cases and of the parietal cortex in EO cases reported previously within the AD spectrum holds true also for the visual variant of AD.

A 49-year-old man was admitted in the Psychiatric Unit of our Hospital due to an acute episode of severe agitation and persecutory ideas forcing the wife to bring him to the Emergency Department (ED). His medical history was marked by a couple of similar accesses to the ED with acute agitation in the recent past. In addition, his wife reported in the last 2 years an inclination to retirement and depressive mood and a continuous decline in mood with apathy and inertia and deficits in episodic memory, with increasing forgetfulness despite maintaining good work functioning. Quite surprisingly, she added that the patient had started to follow a strict fruitarian diet with a highly predominant consumption of just apples. He did neither smoke nor drink alcohol. Notwithstanding, oral olanzapine (15 mg / day) was started with improvement of agitation; however, the patient showed a gradual cognitive worsening with disorientation and executive dysfunction.