Iranian clinical practice guideline for amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegeneration involving motor neurons. The 3–5 years that patients have to live is marked by day-to-day loss of motor and sometimes cognitive abilities. Enormous amounts of healthcare services and resources are necessary to support patients and their caregivers during this relatively short but burdensome journey. Organization and management of these resources need to best meet patients' expectations and health system efficiency mandates. This can only occur in the setting of multidisciplinary ALS clinics which are known as the gold standard of ALS care worldwide. To introduce this standard to the care of Iranian ALS patients, which is an inevitable quality milestone, a national ALS clinical practice guideline is the necessary first step. The National ALS guideline will serve as the knowledge base for the development of local clinical pathways to guide patient journeys in multidisciplinary ALS clinics. To this end, we gathered a team of national neuromuscular experts as well as experts in related specialties necessary for delivering multidisciplinary care to ALS patients to develop the Iranian ALS clinical practice guideline. Clinical questions were prepared in the Patient, Intervention, Comparison, and Outcome (PICO) format to serve as a guide for the literature search. Considering the lack of adequate national/local studies at this time, a consensus-based approach was taken to evaluate the quality of the retrieved evidence and summarize recommendations

Watershed Infarct in Beta-Thalassemia Major Patient

Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia. Method. We present an adult β-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct. Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct. Conclusion. Watershed infarct seems to be the cause of stroke in cases of β-thalassemia major with severe anemia. Blood transfusion can be applied in the setting of acute brain ischemia in such high risk patients

Watershed Infarct in Beta-Thalassemia Major Patient

Background. The mechanism of stroke in beta-thalassemia was reported previously as cardioembolic and hypercoagulable state. However, there is no report of watershed infarct in beta-thalassemia anemia. Method. We present an adult β-thalassemia major patient with manifest asymptomatic chronic left carotid occlusion who suffered watershed infarct. Result. In the presence of asymptomatic chronic left internal carotid occlusion, we assumed that severe anemia (hemoglobin = 3) at admission leads to watershed infarct. Conclusion. Watershed infarct seems to be the cause of stroke in cases of β-thalassemia major with severe anemia. Blood transfusion can be applied in the setting of acute brain ischemia in such high risk patients

Cerebrotendinous xanthomatosis and infertility: A case report

Abstract Cerebrotendinous xanthomatosis (CTX) is a lipid storage disorder that causes neurological, ophthalmic, vascular, and musculoskeletal disorders due to the deposition of cholesterol in the tissues. Hence, we report clinical and imaging of a 31‐year‐old mentally retarded man with cerebellar ataxia, bilateral swelling of the posterior aspect of Achill, and infertility

Supratentorial cortical ependymoma: An unusual presentation of a rare tumor.

Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma

Epidemiology and Clinical Features of Guillain-Barre Syndrome in Isfahan, Iran

Background: Guillain-Barre syndrome (GBS) is an immune-mediated peripheral neuropathy. We compared clinical, laboratory characteristics, and disease course of GBS subtypes in a large group of Iranian patients in Isfahan. Materials and Methods: We collected data from patients who were admitted to Alzahra referral university Hospital, Isfahan, Iran with a diagnosis of GBS. In this population-based cross-sectional research, characteristic of 388 cases with GBS between 2010 and 2015 were studied. Results: The current study recruited 388 patients with GBS including 241 males (62.1%) and 147 females (37.9%) with a mean age of 42.78 ± 21.34. Patients with polyradiculopathy had the highest mean age of 55.12 ± 20.59 years, whereas the least age was seen in acute motor axonal neuropathy (AMAN) with the mean of 36.30 ± 18.71 years. The frequency of GBS witnessed the highest frequency in spring with 113 cases (29.1%) and winter with 101 cases (26%). Patients' electrodiagnostic findings indicated that the highest frequency pertained to AMSAN with 93 cases (24%), whereas the least frequent diagnosis was acute Polyradiculopathy with 8 cases (2.1%). Most of the patients did not have any infections (53.6%) and among patients with infections, AMSAN had the highest frequency (22.9%) and finally, patients with AMSAN and AMAN had a higher length of stay. Conclusion: The study demonstrated incidence, sex distribution, preceding infection, and surgery similar to previous studies. However, our data differs from a study in Tehran that showed acute inflammatory demyelinating polyradiculoneuropathy is more prevalent than other types and we found a seasonal preponderance in cold months, particularly in axonal types

Normal Saline Versus Hypertonic 3% Saline: It’s Efficacy in Non-Acute Rhinosinusitis

Introduction: Sinusitis is one of the most common diseases in general and in otolaryngology practice, but the optimal therapeutic options have not yet been fully developed. This manuscript will try to compare normal saline nasal douching with hypertonic saline in reducing symptoms and improving its signs. Materials and Methods: One hundred and fourteen patients suffering from non acute rhinosinusitis, documented by history, physical examination and radiologic studies were divided into normal saline and hypertonic saline groups, each consisting of 57 patients. data were obtained by physical examination and a questionnaire.. Results:  Type of treatment had no significant effect on headache, morning dryness of mouth and pharynx and fatigue. Nontheless, nasal congestion, purulent discharge and postnasal discharge were reported to have improved in the group treated with hypertonic saline versus the normal saline group. Patient satisfaction also showed better scores in the hypertonic saline group. Conclusion: Hypertonic saline (3%) is more effective for nasal irrigation  than normal saline in chronic rhinosinusitis

The value of MUNIX as an objective electrophysiological biomarker of disease progression in chronic inflammatory demyelinating polyneuropathy

Introduction/Aims: Objective outcome measures to monitor treatment response and guide treatment are lacking in chronic inflammatory demyelinating polyneuropathy (CIDP). In this study we aimed to evaluate the motor unit number index (MUNIX) as an outcome measurement in patients with CIDP and determine the correlation of MUNIX with functional and standard electrodiagnostic tests in a single follow-up study. Methods: We evaluated MUNIX of the abductor pollicis brevis, abductor digiti minimi, and tibialis anterior (TA) muscles bilaterally. Muscle force was assessed by Medical Research Council Sum Score (MRCSS). Functional measures used were the Overall Neuropathy Limitation Score (ONLS) and the Rasch-built Overall Disability Scale (R-ODS) score at baseline and after 6 months of treatment. Standard electrophysiology was evaluated by the Nerve Conduction Study Score (NCSS). Results: Twenty patients were included at baseline, and 16 completed the follow-up study. Significant correlations were found between the MUNIX sum score and both MRCSS and NCSS at baseline, between both the pinch strength and grip and upper limb MUNIX at baseline and follow-up, and between MUNIX of TA and both lower limb MRCSSs with lower limb ONLS at baseline and follow-up. Significant correlations also were found between MUNIX sum score change and MRCSS change, R-ODS change, and ONLS change. Discussion: MUNIX changes correlated with strength and electrophysiological improvements in CIDP patients. This suggests that MUNIX may represent a useful objective biomarker for patient follow-up

Iranian clinical practice guideline for amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegeneration involving motor neurons. The 3-5 years that patients have to live is marked by day-to-day loss of motor and sometimes cognitive abilities. Enormous amounts of healthcare services and resources are necessary to support patients and their caregivers during this relatively short but burdensome journey. Organization and management of these resources need to best meet patients' expectations and health system efficiency mandates. This can only occur in the setting of multidisciplinary ALS clinics which are known as the gold standard of ALS care worldwide. To introduce this standard to the care of Iranian ALS patients, which is an inevitable quality milestone, a national ALS clinical practice guideline is the necessary first step. The National ALS guideline will serve as the knowledge base for the development of local clinical pathways to guide patient journeys in multidisciplinary ALS clinics. To this end, we gathered a team of national neuromuscular experts as well as experts in related specialties necessary for delivering multidisciplinary care to ALS patients to develop the Iranian ALS clinical practice guideline. Clinical questions were prepared in the Patient, Intervention, Comparison, and Outcome (PICO) format to serve as a guide for the literature search. Considering the lack of adequate national/local studies at this time, a consensus-based approach was taken to evaluate the quality of the retrieved evidence and summarize recommendations