Doppler microembolic signals in behcet's disease with nervous system involvement

Background: Behcet's disease [BD] is a multisystemic inflammatory vasculitis of unknown etiology, and vasculitis being its major pathological feature

Objectives: We studied the prevalence of microembolic signals [MES] in patients with diagnosis Behçet's disease. We also tried to determine the frequency of MES in BD patients with or without neurological involvement

Materials and Methods: This study enrolled 40 patients who fulfilled the diagnostic criteria of International Study Group for BD during 2012 to 2013. Bilateral transcranial Doppler ultrasound of the middle cerebral arteries was performed by multigate method. MES were identified based on the criteria of International Consensus group on Microembolus Detection

Results: We found MES in none of our patients with BD. We measured intima-media thickness in all patients

Conclusion: It seems that in our population core histopathologic phenomenon to be other than MES andvasculiticphenomenonmay be implicated as a pathophysiologic factor for central nervous system involvement.The clarification of this subject needs further investigations on Iranian BD patients

Neuroacanthocytosis in two brothers: an ultra-rare cause of movement disorder

We report a rare genetic disorder case of neuroacanthocytosis with clinical profile [oro-lingual-facial abnormal involuntary movements, neuropathy] and typical magnetic resonance findings [cerebral atrophy, bilateral caudate nuclei atrophy with dilated anterior horns of the lateral ventricles], positive family history in his brother and acanthocytosis in peripheral blood smear

novel c.4822>T mutation on SPG11 in an Iranian patient marked by hereditary spastic paraparesis and skeletal deformity: an incidental finding or a true association

Hereditary spastic paraplegias are highly heterogeneous neurodegenerative disorders with some special mutations. We report on a patient with pescavus, distal a myotrophy, hyper extended fingers, and pectus excavatum. Neurological examination showed that he had proximal lower limbs weakness with a positive Gower sign, exaggerated lower limbs deep tendon reflexes with spasticity, distal muscle was ting, bilateral horizontal nystagmus [direction change], and positive Romberg sign. A novel mutation in SPG11/spatacsin was detected through genetic analysis. Magnetic resonance imaging showed normal whole spine and brain anatomy

Anti-N-methyl-D-aspartate-receptor encephalitis, a challenge for neurologists or a chameleon for psychiatrists: a case report

Anti-Af-Methyl-D-Aspartate-Receptor [NMDA-R] encephalitis is a new autoimmune disorder, often paraneoplastic in nature, presenting with complex neuropsychiatric symptoms. Diagnosed serologically, this disorder is often responsive to immunosuppressant treatment, We here in report the case of a 56-year-old man with anti NMDA-R encephalitis presenting initially with disorientation and hallucination. He later developed bilateral ophthalmoplegia and spastic tetraparesis. Neurological examination showed mild consciousness disturbance and bilateral ophthalmoplegia on admission, spastic tetraparesis with limbs hyperreflexia. Cerebrospinal fluid samples showed mild pleocytosis. MRI disclosed some small hypersignal lesions in the FLAIR. Anti-NMDA-R antibody was diagnosed upon detection of antibody in the serum

Psychiatrists and neurologists should pay more attention to the cranial, mental and behavioral involvement due to this potentially fatal disease

Cerebral venous-sinus thrombosis: risk factors, clinical report, and outcome. a prospective study in the north east of iran

Introduction: Cerebral venous-sinus thrombosis [CVST] is a life threatening condition that needs rapid diagnosis and treatment. It appears comparatively more common in Middle East and South Asia

Objectives: To determine the demographic, clinical patterns, etiologies and prognostic factors of CVST in the North East of Iran

Materials and Methods: All adult patients admitted with a documented diagnosis of CVST from January 2011 to March 2012 in an academic hospital in the North East of Iran, entered this prospective descriptive study. The patients' demographic characteristics, clinical presentations, laboratory and brain imaging findings, treatment options were also studied. Follow-up visits were performed at month 1, 6, and then at month 12 using modified Rankin Scale [mRS]. Findings were analyzed using descriptive tests and Chi square test in SPSS software version 21

Results: Sixty patients [13.3% men, 86.7% women] with mean age of 38.11+/-11.30 years were identified. Fifty one cases [85%] had a clinical picture of increased intracranial pressure. Causes included positive antiphospholipid antibodies in 3.3%, protein C, S and anti thrombin III deficiency in 5%, 1.7% and 3.3%, polycythemia in 1.7%, infections in 1.7%, postpartum in 9.6% of women, and using Oral Contraceptive Pills [OCPs] in 65.38%. We found 10% mortality rate on discharge and 11.9% within 30 days and 42.7% rate of death or dependency at month 12

Conclusion: The findings of the study indicate that the use of OCPs was a main factor associated with CVST especially in association with inherited hypercoagulable state

White matter diseases yes, multiple sclerosis no, sjogren - larsson syndrome: another differential diagnosis of multiple sclerosis

Sjogren-Larsson Syndrome [SLS] is an inherited autosomal recessr neurocutaneous disorder with congenital ichthyosis, spastic diplegia quadriplegia and mental retardation

We report a case of Sjogren-Larsson Syndrome with clinical profile [ment retardation, ichthyosis, spastic diplegia] and MRI findings such as seen i multiple sclerosis [MS]

So this rare syndrome can be another differenti; diagnosis of MS