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Background: Dementia is a common neurogenerative disease among older adults. Therefore, they are more prone to potentially inappropriate medication (PIM), which is medication that causes more harm rather than protecting the health of an individual. Hence, the American Geriatrics Society (AGS) has recognized the risk of certain medication classes on this population and released PIM according to Beers criteria, which is a helpful guide for clinicians to ensure the safety of medication before it is prescribed. The aim of this study is to assess the prevalence of PIM use among older adults with dementia as a risk factor in comparison to other older adults without dementia. Methods: A retrospective study was conducted in an outpatient setting in a tertiary hospital targeting elderly patients aged 65 years old or over from January 2020 to September 2022. A total of 598 patients were screened, and 270 patients met the inclusion criteria. The eligible patients were then divided into two groups: 168 were in a non-dementia group and 102 were in a dementia group. Results: PIM use was reported in patients with and without dementia. The most inappropriate medication that was prescribed comprised atypical antipsychotics PIM for both patients with and without dementia. However, the prevalence was higher in the dementia group for quetiapine (75% vs 24% respectively), olanzapine (82% vs 17% respectively) or risperidone (92% vs 7%, respectively). Anticholinergics were highly prescribed in older adult without dementia as compared to dementia patient and was statistically significant for solifenacin (96% vs.3.6% respectively) and amitriptyline (88% vs 11% respectively). Conclusion: Among elderly patients in outpatient care settings, the prevalence of PIM use is considered high in dementia patients for antipsychotics, while a higher use of benzodiazepine and anticholinergics was found in non-dementia patients.
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Woodhouse-Sakati syndrome (WSS) is a rare autosomal recessive multi-system genetic disease caused by loss of function mutations in the DCAF17 gene on chromosome 2q31.1. The disease is characterized by gradual neurologic degeneration and polyendocrinopathy, particularly noteworthy for hypogonadism, beginning in early adolescence. Clinical features show wide variability with no clear genotype-phenotype correlation. The pathophysiology of WSS is unclear at present and no specific treatment is available other than hormone replacement which is administered in the course of individualized symptomatic multidisciplinary care. Genetic testing helps in confirming the diagnosis along with genetic counseling of the patient and family members. Here we report multiple cases of WSS in three siblings from a new Saudi Arabia family who were diagnosed with WSS as a consequence of a common founder mutation in the DCAF17 gene with DNA analysis showing a homozygous single nucleotide frameshift deletion (c.436delC) in exon 4 of the gene.
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BACKGROUND: Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. METHODS: This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann-Whitney U tests, as appropriate. RESULTS: A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25-53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). CONCLUSION: The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.