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Anxiety is among the most debilitating nonmotor symptoms of Parkinson's disease (PD). This study aimed to determine how PD patients with low and high levels of anxiety (LA-PD and HA-PD, respectively) compare with age- and sex-matched controls at the level of motor control of reach-to-grasp movements during single- and dual-task conditions with varying complexity. Reach-to-grasp movement kinematics were assessed in 20 LA-PD, 20 HA-PD, and 20 sex- and age-matched healthy controls under single- as well as easy and difficult dual-task conditions. Assessment of PD patients was performed during both the on- and off-drug phases. The results obtained during dual-task conditions reveal deficits in both reach and grasp components for all three groups (e.g., decreased peak velocity and delayed maximum hand opening). However, these deficits were significantly greater in the PD groups, especially in the HA-PD group. Although dopaminergic medication improved reach kinematics, it had no effect on grasp kinematics. The results of our study indicated that high levels of anxiety may enhance the inefficiency of upper limb motor control in PD patients, especially during high demanding cognitive conditions, and should, therefore, be considered in the assessment and planning of interventions for upper limb function in these patients.

Background and Objectives: To investigate the correlation between reach and grasp kinematic measures and clinical measures of gross and fine manual dexterity in patients with idiopathic Parkinson Disease (PD). Methods: Sixty patients (44 men, 16 women) with idiopathic PD (Mean±SD of age: 59.85±11.89 years) were recruited in this non-experimental cross-sectional study using a non-probability sampling method. Their gross and fine manual dexterity were assessed using the Box and Block Test (BBT) and Nine Hole Peg Test (NHPT), respectively. Also, reach and grasp kinematic measures (including normalized movement time, peak velocity, Maximum Grip Aperture [MGA], and percentage of movement time in which MGA occurred [PMGA]) were measured in the more affected hand. Results: The results showed a significant and moderate correlation between reach kinematic measures (i.e. normalized movement time and peak velocity) and both gross and fine manual dexterity assessed by BBT (P=0.000) and NHPT (P=0.000), respectively. However, there was not a significant correlation between grasp kinematic measures (i.e. MGA and PMGA) and gross manual dexterity measured by BBT (P>0.05) as well as fine manual dexterity assessed by NHPT (P>0.05). Conclusion: This study showed no to moderate correlation between reach and grasp kinematic measures and clinical measures of gross and fine manual dexterity in patients with PD, supporting the significance of multi-level evaluation using both clinical and kinematic measures in the evaluation of upper limb function in patients with PD.

Recent studies indicate that there is a synergic association between butyrylcholinesterase-K variant (BChE-K) and apolipoproteinE-epsilon 4 (ApoE-epsilon 4) to promote risk for Alzheimer's disease (AD). Most subsequently replicative studies have been unable to confirm these finding. We attempted to replicate this finding in 105 AD cases and age and sex matched 129 controls from Tehran population, Iran. The BChE genotype of patients were found to be significantly different from controls (chi(2) = 12.2, d.f. = 2, p = 0.002). The frequency of BChE-K allele was also found to differ significantly in cases compared to controls [24% versus 12% (chi(2) = 20.6, d.f. = 2, p0.001)] leading to an increased risk of AD in subjects with this allele (OR = 2.5, 95% CI = 1.64-3.8, p = 0.001). This risk was found to increase from (OR = 2.37, 95% CI = 1.3-4.2, p = 0.006) in subjects less than 75 years old to (OR = 3.16, 95% CI = 1.41-7.1, p = 0.001) in subjects 75 years and older. But, the ApoE-epsilon 4 allele association risk was found to decrease from (OR = 9.5, 95% CI = 3.74-24.1, p = 0.001) in subjects75 years to (OR = 1.36, 95% CI = 0.49-4.1, p = 0.58) in those subjects 75 years and older. Furthermore, we found a very strong synergic association between BChE-K and ApoE-epsilon 4 OR = 19.1 (95% CI = 428-85.45, p0.001). In spite of this, synergism decreased from OR = 36.2 (95% CI = 4.4-296, p = 0.001) in subjects75 year olds to OR = 6.2 (95% CI = 0.9-72.4, p = 0.06) in subjectsor =75 years. We have found that BChE-K and ApoE-epsilon 4 alleles act synergistically to increase the risk of the late-onset AD, particularly in age group75 years in Tehran, Iran.

We aimed to compare the outcomes of patients who underwent carotid artery stenting (CAS) followed by coronary artery bypass grafting (CABG) with the outcomes of those who underwent isolated CABG without carotid intervention.In this prospective cohort study, conducted between March 2007 and February 2010, all patients who had significant carotid artery stenosis (70%) and were candidates for CABG were included. The outcome measures, including 30-day post-stenting complications, cardiac surgery neurological complications, myocardial infarction (MI), and mortality rates, were assessed.A total of 112 patients underwent CABG without carotid artery intervention and 62 patients were scheduled for CAS + CABG. The death and MI or stroke rates in the CAS + CABG patients and isolated CABG group were 9.7% and 6.3%, respectively (P=.18). In the CAS + CABG group, 4 patients (6.4%) were complicated by ipsilateral stroke, 2 (3.2%) by MI, and 3 (4.8%) by death; 2 deaths had neurological causes and 1 death had a cardiac cause. In the isolated CABG group, 4 stroke cases (3.6%) were diagnosed in the postoperative period, 2 of them (1.8%) being ipsilateral. Also, 1 MI case (0.9%) and 4 deaths (3.6%) occurred after cardiac surgery; 2 deaths had neurological causes and the remaining 2 deaths resulted from other postoperative complications (mediastinitis and arrhythmia).The risk of ipsilateral stroke in the isolated CABG approach in patients with concomitant coronary and carotid stenosis is small, and there is no evidence that this risk is lessened by prophylactic CAS. Staged CAS + CABG may become the preferred option in patients with symptomatic bilateral carotid stenosis with stable cardiac status if it is conducted in a high-volume center by experienced operators.

The aim of this study was to investigate some of nongenetic factors associated with Parkinson's disease (PD) in a sample of Iranian patients. This case-control study included 75 patients with idiopathic PD and 75 control patients. The patients in the control group were found to have drunk more glasses of tea per day than the case group before the onset of their problem (p=0.019). Every extra glass of tea per day decreased the risk of PD by 0.8 times (OR=0.8, 95%CI=0.73-0.97, p=0.02). Each cup of coffee per week decreased the chance of developing PD by 0.5 times (OR=0.5, 95%CI=0.28-0.9, p=0.021). A previous history of evening work increased PD risk by 4.6 times (OR=4.6, 95%CI=1.29-16.86, p=0.019) while major stressful events increased it by 13.5 times (OR=13.5, 95%CI=4.7-38.1, p=0.0001). In conclusion, coffee and tea consumption may exert a protective effect against PD, while evening work and stress may be risk factors for the development of the disease.

Myotonic dystrophy type 1 (DM-1) is a multi-system disorder affecting the muscles, brain, cardiovascular system, endocrine system, eyes and skin. Diagnosis is made by clinical, electrodiagnostic and genetic studies. This study aimed to determine the correlation between CTG expansion and distribution of muscle weakness and clinical and electrophysiological findings. Genetically confirmed DM-1 patients presenting to Shariati Hospital between 2005 and 2011 were included in this study. Clinical, electrodiagnostic and genetic testing was performed and the correlation between CTG expansion and distribution of muscle weakness and clinical and electromyographic findings was studied. Thirty-three genetically confirmed DM-1 patients were enrolled. Myotonia, bifacial weakness and distal upper limb weakness were seen in all patients. Diabetes mellitus was found in one patient (3%), cardiac disturbance in eight (24.2%), cataracts in eight (24.2%), hypogonadism in five (15.2%), frontal baldness in 13 (39.4%), temporalis wasting in 14 (42.4%), temporomandibular joint disorder in seven (21.2%) and mental retardation in eight (24.2%). The mean number of CTG repeats, measured by Southern blot, was 8780 (range 500-15,833). A negative correlation was found between CTG expansion and age of onset. Temporalis wasting and mental retardation were positively correlated with CTG expansion. No relationship was found between weakness distribution, electromyographic findings, other systemic features and CTG expansion. In this study of DM-1 in Iran, we found a correlation between CTG expansion and age of onset, temporalis wasting and mental disability. No correlation between CTG expansion and electrodiagnostic and other clinical findings were detected.

Background: Wilson’s disease (WD) is a treatable autosomal recessive metabolic disorder which could lead to protean hepatic or neurologic manifestations. WD could mimic many neurologic disorders and is often diagnosed with a long delay. This study describes central nervous system manifestations of a group of Iranian patients with neurologic WD. Methods: Data from case records of patients with neurologic WD presenting at a referral university hospital and a private clinic in Tehran from 1984 to 2004 were analyzed. Results: Fifty patients from 44 unrelated families with WD were identified, whose mean duration of follow-up was 51.8 (±58.5) months. The median age of onset of neurologic symptoms in 37 patients with primary neurologic or simultaneous hepatic-neurologic presentation was 16 (10–38) years, whereas in 13 patients with prior hepatic damage, this was 18.5 (11–34) years. The 6 most common manifestations were dysarthria (80%), drooling (48%), tremor in limbs (44%), abnormal gait (44%), psychiatric and/or sleep symptoms (44%), and dystonia in limbs (42%). Conclusion: Neurologic WD has heterogeneous manifestations and should be considered in young patients presenting with dysarthria, drooling, any kind of movement disorders or psychiatric symptoms.

Acetylcholine receptor (AChR) deficiency is the most common form of congenital myasthenic syndrome (CMS). Most AChR deficiencies are caused by mutations in the coding region of the AChR epsilon subunit.1 We report an Iranian Muslim family from the province of Eastern Azerbaijan (Maragheh) in which three of five offspring of consanguineous parents had early onset CMS arising from a newly described mutation in the epsilon subunit of the AChR; this mutation has been identified homozygously in all the three sibs. This is the first report of an AChR epsilon subunit mutation in Iran. The affected brother (case 1) was 23 years old and the affected sisters were 19 (case 2) and 16 (case 3) years old. The unaffected brother was 21 and the healthy sister was 13 years old. There was no history of miscarriage or infant mortality in the family, although, according to verbal accounts from the mother, fetal movements were decreased in all the affected sibs and case 1 had a difficult labour. The clinical diagnosis of CMS in this family had been made first in 1996. All cases presented with neonatal hypotonia, regurgitation, ptosis (case 1 developed ptosis at the age of 6 months), and delayed motor milestones. The course of the disease had been slowly progressive, transiently exacerbated by stress. Generalised weakness was more severe in the afternoons. Treatment with prednisone for …

Background Myasthenic crisis is the most serious life-threatening event in generalized myasthenia gravis (MG) patients. The objective of this study was to assess the long-term impact of thymectomy on rate and severity of these attacks in Iranian patients. Methods We reviewed the clinical records from 272 myasthenic patients diagnosed and treated in our neurology clinic during 1985 to 2002. Fifty-three patients were excluded because of unconfirmed diagnosis, ocular form of MG, contraindication to surgery, concomitant diseases and loss to follow-up. The Osserman classification was used to assess the initial severity of the disease. Frequency and severity of the attacks were compared between two groups with appropriate statistical tests according to the nature of variables. Multivariate logistic regression analysis was used to assess the predictors of myasthenic crisis in the group of patients without thymoma. Results 110 patients were in thymectomy group and the other 109 patients were on medical therapy. These two groups had no significant differences with respect to age at onset, gender, Osserman score in baseline and follow up period. 62 patients (28.3% of all 219 patients) had reported 89 attacks of myasthenic crisis. 20 patients of 62 (32%) were in thymectomy group and 42 (68%) were in the other group. There was significant difference between the two groups in number of patients with crisis (P = 0.001; odds ratio = 2.8 with 95% CI of 1.5 to 5.2). In addition, these attacks were more severe in group of non-thymectomized patients as the duration of ICU admission was longer and they needed more ventilatory support during their attacks. Regression model showed thymectomy and lower age at onset as two predictors of decrement in myasthenic crisis rate in non-thymomatous MG patients. Conclusions It is suggested that frequency and severity of myasthenic attacks as important endpoints in evaluation of MG patients. Thymectomy seems to have a preventive role on rate and severity of these attacks.

Intracranial chondromas/osteochondromas are rare benign lesions usually attached to dura, located over the convexity of the skull containing benign cartilaginous/bony components; however, other locations are probable [1]. Intracranial osteochondromas usually originate more commonly extradurally from cartilaginous remnants at the base of the skull and less frequently intradurally from the dura mater of the convexity or falx cerebri [1, 2]. When developing supratentorially, the parafalcine frontoparietal location is the most common site [1, 2]. A 60-year-old female was presented to our clinic with 10-year history of generalized tonic–clonic seizures controlled medically; also, the patient suffered from frequent abrupt episodes of restlessness and anxiety with the duration of 5 min. The neurological exam was normal. Previous imaging studies had revealed a large calcified lesion in the left middle cranial fossa originating from skull base (Fig. 1). The imaging characteristics of the tumor as well as its indolent course prompted us to the probable diagnosis of intracranial osteochondroma. Surgery was not recommended because of the eloquent location of the lesion and the possible occurrence of major deficits following the surgery; in addition, the seizure attacks were medically controllable. Typically, the histomorphology of chondromas/osteochondromas looks like mature hyaline cartilage devoid of anaplastic proliferation of chondrocytes or nuclear atypia, in conjunction with bony components and a lobular arrangement of clusters of lacunae containing single chondrocytes [3].

Parkinson's disease (PD) is a widespread progressive neural degenerative disease. Patients encounter problems in carrying out ordinary voluntary movements such as gaiting and writing. Effects of tremor, rigidity, and bradykinesia are seen in writing. Since the diagnosis of PD is based on the presence of cardinal symptoms, therefore voluntary movements like writing could be helpful as an indicator in this process. Handwriting analysis can be a suitable method to separate patients from normal individuals. We recorded handwriting data from 17 normal and 13 PD subjects, and then used mathematical analysis in order to extract proper features. At the end of the study, we used these selected features in a classifier. We achieved 93.89% accuracy in the test phase. Hence, this tool may be aid in the diagnosing of both PD and suspected PD individuals in the early stages of the disease.

Friedreich's Ataxia (FA) is the commonest genetic cause of ataxia and is associated with the expansion of a GAA repeat in intron 1 of the frataxin gene. Iron accumulation in the mitochondria of patients with FA would result in hypersensitivity to oxidative stress. Mitochondrial DNA (mtDNA) could be considered a candidate modifier factor for FA disease, since mitochondrial oxidative stress is thought to be involved in the pathogenesis of this disease. We studied 25 Iranian patients (16 females and 9 males) from 12 unrelated families. DNA from each patient was extracted and frequency and length of (GAA)(n) repeat was analyzed using a long-range polymerase chain reaction (PCR) test. Also we investigated impact of GAA size on neurological findings, age of onset and disease development. In order to identify polymorphic sites and genetic background, the sequence of two hypervariable regions (HVR-I and HVR-II) of mtDNA was obtained from FA patients harbouring GAA trinucletide expansions. Alignment was made with the revised cambridge reference sequence (rCRS) and any differences recorded as single base substitution (SBS), insertions and deletions. Homozygous GAA expansion was found in 21 (84%) of all cases. In four cases (16%), no expansion was observed, ruling out the diagnosis of Friedreich's ataxia. In cases with GAA expansions, ataxia, scoliosis and pes cavus, cardiac abnormalities and some neurological findings occurred more frequently than in our patients without GAA expansion. Molecular analysis was imperative for diagnosis of Friedreich's ataxia, not only for typical cases, but also for atypical ones. Diagnosis bases only on clinical findings is limited, however, it aids in better screening for suspected cases that should be tested. Our results showed that the rate of D-loop variations was higher in FA patients than control (P0.05). mtDNA deletions were present in 76% of our patients representing mtDNA damage, which may be due to iron accumulation in mitochondria.