Magnetic Resonance Imaging Diagnosis in Normal Pressure Hydrocephalus.

BACKGROUND: Idiopatic normal pressure hydrocephalus (iNPH) is a progressive neurologic syndrome featured by the triad of gait disturbance, mental deterioration and urinary incontinence, associated with ventriculomegaly and normal cerebrospinal fluid (CSF) pressure. The clinical presentation may be atypical or incomplete, or mimicked by other diseases, so conventional neuroradiologic imaging plays an important role in defining this pathology. iNPH pathophysiologic mechanisms have not yet been fully elucidated, although several studies have demonstrated the involvement of the glymphatic system, a highly organized fluid transport system, the malfunction of which is involved in the pathogenesis of several disorders including normotensive hydrocephalus. METHODS: Recent studies have shown how crucial in the diagnosis of this pathology is the definition of morphologic biomarkers, such as ventricular enlargement disproportionate to cerebral atrophy and associated ballooning of frontal horns; periventricular hyperintensities; and corpus callosum thinning and elevation, with callosal angle <90 degrees. RESULTS: Another interesting feature that is becoming a well-recognized factor to look for and useful for the diagnosis of iNPH is disproportionately enlarged subarachnoid space hydrocephalus, which includes enlarged ventricles, tight high-convexity and medial surface subarachnoid spaces, and expanded Sylvian fissures. A correct choice of MRI sequences is important for a proper characterization identification of others diseases that may underlie this pathology. Magnetic resonance imaging allows us to evaluate CSF flow, enabling us to define qualitative and quantitative parameters necessary for the purpose of accurate iNPH diagnosis. CONCLUSIONS: iNPH can represent a real diagnostic challenge; a proper correlation among clinical features, traditional MRI, and CSF dynamics analysis can lead to a correct diagnosis.

Psychological Aspect and Quality of Life in Porphyrias: A Review.

The World Health Organization (WHO) describes "health" as a state of physical, mental, and social well-being and not merely the absence of disease or infirmity. Therefore, a biopsychosocial approach should be considered as an integral part of patients' management. In this review, we summarize the available data starting from 1986 on the biological, psychological, and social aspects of porphyrias in order to provide a useful tool for clinicians about the missing knowledge within this field. Porphyrias are a group of rare metabolic disorders affecting the heme biosynthetic pathway and can be categorized into hepatic and erythropoietic. Here, a total of 20 articles reporting the psychological and the quality of life (QoL) data of porphyria patients affected by acute hepatic porphyrias (AHPs), Porphyria Cutanea Tarda (PCT), and Erythropoietic Protoporphyria (EPP) were analyzed. These 13 articles include reported quantitative methods using questionnaires, while the reaming articles employed qualitative descriptive approaches through direct interviews with patients by psychology professionals. We conclude that the use of questionnaires limits the complete description of all areas of a patient's life compared to a direct interview with specialists. However, only a combined use of these methods could be the best approach for the correct disorder management.