[Common clinical aspects of tuberous sclerosis and neurofibromatosis in Mashhad]

Since CNS is derived from ectoderm during the fetal period, there are some common congenital or hereditary conditions that affect both the skin and nervous systems. Such conditions are traditionally called neurocutaneous disorders or phakomatosis.In this study, we have reviewed the most common clinical features and paraclinical findings in neurofibromatosis [NF] and tuberous sclerosis. In this cross sectional study, 50 patients with tuberous sclerosis or NF were chosen among all the patients who referred to the neurological clinic of Shahrivar 17th Hospital during a ten-month period. They were physically examined to detect any suspicion of neuroectodermal syndromes employing paraclinic exams like CT scan, MRI and EEG. Information about patients' familial history, age, sex, clinical and paraclinical findings was collected to fill out the questionnaires. Finally, the data were analyzed with SPSS and SAS soft wares. 80% of our patients had NF. The ratio of males to females was calculated as 1.1. The average age for the emergence of the disease was 7.8 years old for tuberous sclerosis, 11.3 years old for NF1 and 59 years old for our only case of NF2. The most common manifestation of these syndromes was Cutaneus Lesions [88.9% in tuberous sclerosis and 100% in NF]. Seizure and mental retardation were also common features in tuberous sclerosis with frequencies of 88.9% and 75%, respectively. Paraclinical studies were helpful in diagnosing the 50% of tuberous sclerosis and the8.7% of neurofibromatosis cases. Clinical and paraclinical manifestations of neurocutaneous disorders in this study are similar to other studies in the world

[Clinical and paraclinical symptoms in Dementia]

Dementia is basically the disease of old people which is usually caused by a primary degenerative lesion or by a structural disease in the brain. In terms of etiology, dementia is associated with reversible and irreversible causes. Determination of the underlying causes of dementia may facilitate its treatment and prognosis. The purpose of this study is to evaluate clinical and paraclinical symptoms of dementia as well as to determine the most common causes. This is a cross sectional descriptive study on 100 patients with dementia who referred to Shahrivar 17[th] Hospital in Mashhad. First, the patients who were diagnosed as being infected by dementia were evaluated according to DSM-IV criteria. They were also enrolled in history taking and physical examinations as well as paraclinical managements like EEG, CT Scan and MRI. The obtained data were then recorded down in the questionnaires. 36% of the patients were females and 64% were males. Of this population, 43% had Alzheimer Disease [AD], 40% had Vascular Dementia [VaD], 9% Parkinson disease [PD] and 8% had other types of dementia while there was no case of Ferontotemporal dementia [FTD]. HTN, DM and Hyperlipidemia were found to have a fully significant effect on VaD. 100% with the history of TIA and 88% with the history of CVA had VaD which was meaningful, too. 55% of the VaD patients were identified with Gait disorder and 65% with Incontinence which is ststistically significant. Based on the results of MRI and CT Scan, 100% of the AD patients, 47.5% of the VaD patients and 88.9% of the PD patients were identified with cortical atrophy. In addition, it was found that hypocampus atrophy was present in 53.5% of the patients with AD and in 15% of the patients with VaD. In 90% of the VaD patients, hypodense foci was observed which is statistically significant, too. As the findings of the study suggest, the results of CT Scan and MRI was notably related to the type of dementia. Thus, it seems that using these two techniques can determine the existence of dementia in the early stages which can help with improving the quality and quantity of life in these patients. According to the frequency rate of VaD in our patients, it seems that the lack of or poor control of the risk factors like HTN, DM and hyperlipidemia can lead to the high frequency rate of vascular dementia