Studies of specific populations have provided invaluable knowledge about Parkinson's disease (PD), especially in the field of genetics. The present report systematically reviews the medical literature on PD in Arabs. Medline and Embase were searched, and 24 article were identified: genetic (n = 17), epidemiological (n = 3), and clinical series (n = 5). Both autosomal dominant and recessive forms of inherited PD are described, associated with four genes (Parkin, PINK1, LRRK2, and PARK9). The G2019S LRRK2 mutation is more common in both familial (37-42%) and apparently sporadic PD (41%) in North African Arabs than in Europeans and North Americans (2-3%). The incidence of PD is reported at 4.5 per 100,000 person-years and reported prevalence at 27 to 43 per 100,000 persons. Hospital-based clinical series suggest that parkinsonism is the commonest movement disorder. Clinical features of PD in Arabs are not significantly different from those reported elsewhere. PD was reported as the cause of dementia in around 7% of Arabs. The majority of studies relate to the role of genes in the etiology of PD in North African Arabs. Further genetic, epidemiological and clinical studies from the majority of Arabic countries may enhance our understanding of PD.

The phenotypic and genetic spectrum of ataxia with oculomotor apraxia (AOA) disorders is rapidly evolving and new technologies such as genetic mapping using whole exome sequencing reveal subtle distinctions among the various subtypes. We report a novel PNKP mutation in two siblings with progressive ataxia, abnormal saccades, sensorimotor neuropathy and dystonia consistent with the AOA type 4 phenotype. Laboratory evaluation revealed hypoalbuminemia, hypercholesterolemia with elevated LDL, elevated IgE levels and normal α fetoprotein levels. Eye movement examination demonstrated a marked saccade initiation defect with profound hypometric horizontal saccades. Vertical saccades were also affected but less so. Also present were conspicuous thrusting head movements when attempting to change gaze, but rather than an apraxia these were an adaptive strategy to take advantage of an intact vestibulo-ocular reflex to carry the eyes to a new target of interest. This is demonstrated in accompanying videos.

We report a consanguineous family with three affected siblings with novel mutation in the KCNJ10 gene. All three presented with central nervous system symptoms in the form of infantile focal seizures, ataxia, slurred speech with early developmental delay and intellectual disability in two siblings. None had any associated electrolyte abnormalities and no symptomatic hearing deficits were observed.

SummaryBrake fluid (glycol-based) toxicity is known to have a protean of central and peripheral nervous system manifestations. The principal component of this household poison is ethylene glycol. Toxic effect is generally attributed to peri-vascular deposition of calcium oxalate crystals in various tissues. However, clinical features resembling brain death have rarely been reported. We report a case of brake fluid toxicity simulating brain death in a 21-year-old healthy man who ingested it as a recreational agent. BACKGROUND

Objective:To assess the tolerability of propofol (PF) in Wada test in an Arab population with temporal lobe epilepsy (TLE).Methods:This observational study with consecutive sampling took place in King Fahad Medical City, Riyadh, Saudi Arabia. Nine consecutive patients with mean (+SD) age of 26 (+5.8) years, 6 males and 3 females, underwent Wada test between January 2009 and December 2012. Six of them had left TLE, and 3 had right TLE. Each patient received 10 mg of PF in the internal carotid artery (ICA). Right hemispheric injection was followed by left hemisphere injection after 30 minutes. During the procedure, EEG monitoring showed changes within 5-18 seconds of injection as hemispheric delta slowing. Neuropsychological tests were carried out for localization of memory and language.Results:We were able to lateralize speech dominance in 8 patients and memory dominance in 6 patients. Peri-procedural complications included transient euphoria (n=1), transient spasm of ICA (n=1), eye pain (n=1), facial pain (n=1), and generalized tremulousness (n=2). None of the patients exhibited a symptomatic drop in blood pressure.Conclusions:We found that PF is well tolerable for the Wada test, with minimally significant complications, although blood pressure should be closely monitored.