[Frequency and clinical characteristics of amyotrophic lateral sclerosis [ALS] disease in patients referred to neurology department of ghaem hospital]

ALS is a neurodegenerative disease due to degeneration of both upper and lower motor neurons [UMN and LMN]. There is no treatment for this disorder and it is important to distinguish from other conditions that may mimic ALS. This is a descriptive study since September 2002 until September 2004 among the patients whom referred to neurology department of Ghaem hospital. 21 patients were studied [14 men]. Mean age was 41.58 years. UMN and LMN symptoms were the presenting complaint of our patients except 5 cases with bulbar onset. Symptoms began before age of 40 in 40% of our all patients, and 27% of Charcot type of ALS. 15 Cases had charcot type of ALS, 4 cases had juvenile ALS, 1 case had Kennedy syndrome and finally 1 case had Madras motor neuron disease [MMND]. In this study we found the first case of MMND in Iran. There was a large proportion [40%] of cases with age of onset below 40 years in comparison with previous studies, more epidemiological and toxicological studies should be done

[Cerebral venus thrombosis in association with HSV1 encephalitis report of 1 case]

Encephalitis due to herpes simplex virus [HSV] is the most important cause of fatal Sporadic encephalitis that prompt diagnosis is necessary because there is effective antiviral treatment for this disorder. In the fatal cases, severe meningoencephalitis is associated with diffuse destructive changes in the brain tissue and is following the reactivation of latent virus.Recent studies demonstrate increased hypercoagulopathy state due to HSV infection in the endothelium and footprints of virus has been found in the vascular thrombosis. In this issue, one of the very rare manifestations of HSV encephalitis, which has so far been reported in a neonate, is presented. The patient presented with the signs of encephalitis including stupor state and agitation, headache, seizure and neck stiffness. The diagnosis of HSV encephalitis is confirmed by the PCR test of CSF fluid .MRI and MR venography of the patient's brain demonstrated the thrombosis of sagital, rectus and lateral venous sinus. The patient survived with the specific treatments. Herpes simplex virus type 1 can be a cause for the development of cerebral Venus thrombosis. This case report is a conformation for the studies demonstrating hypercoagulopathy state in the setting of HSV infection