Interferon-beta in pediatric multiple sclerosis patients: safety in short-term prescription

None of the approved immunomodulatory drugs in adults Multiple Sclerosis [MS] patients have been officially approved for the pediatric patients and are currently used off-label in this population. In this study, we evaluated the effectiveness and tolerability of intramuscular interferon beta1-a [Avonex[registered]] and subcutaneously injected interferon beta1-b [Betaferon[registered]] in children with definite relapsing-remitting MS [RRMS]. Thirteen patients aged younger than 16, who were recently diagnosed with definite RRMS according to the McDonald's criteria, were enrolled in this study. Six patients were treated with Avonex[registered] 30 micro g, intramuscularly every week, and seven patients were treated with Betaferon[registered] 250 micro g, subcutaneously every other day. All patients were treated with adult doses; initially interferon-beta was prescribed with half dose, and it was increased to full adult dose steadily. Eleven girls and two boys, mean [SD] age of 14.7 [1.9] years, were studied. Following nine months of using interferon-beta, nine patients [69.2%] had no relapses and the remaining four, experienced only one relapse. The mean EDSS score was decreased significantly after the study period. The present study provides reasonable data for the use of interferon-beta in Pediatric MS due to lack of short-term complications and safety. Studies with larger sample size and longer follow up duration are required to shed light on the long term impact of the interferon-beta therapy in children

Isaac's syndrome associated with CIDP and pregnancy

Neuromyotonia with all its synonyms is a disorder of peripheral nerve hyperexcitability characterized by regular or irregular myokymia, muscle cramps and stiffness, delayed muscle relaxation after contraction, and hyperhidrosis associated with well-described spontaneous electromyographic features. Herein, we report clinical and electrodiagnostic findings of a pregnant woman with neuromyotonia who also suffered from chronic inflammatory demyelinating polyneuropathy. We treated the patient with plasma exchange, 50 mL/kg [twice weekly, for six weeks]. After two weeks of treatment, cramps and stiffness were substantially reduced. After four weeks, she looked normal with a relatively smooth gait. After eight weeks, the patient was entirely well with no cramps or stiffness. Repeat EMG showed no myokymic discharges. After four months she was in good health and the plasma exchanges and only visited the patient regularly. One year later, we repeated a five-day course of plasma exchange to overcome mild recurrence of myokymia in her thighs. Now, after four years, she is healthy without any disability or problem. The patient's child has been healthy throughout without any evidence of neuromyotonia

Malignant cutaneous melanoma associated with cerebral venous sinus thrombosis

Cerebral venous sinus thrombosis [CVST] is an infrequent disease [representing around 1% of all strokes] manifested by clotting of blood in cerebral venous or dural sinuses as well as cortical veins. Herein, we describe an unusual case of malignant melanoma presenting with CVST in whom the diagnosis of CVST was suspected on the basis of clinical findings along with brain CT scan, and finally was confirmed by brain MRI and magnetic resonance venography. Intravenous heparin treatment was started immediately; but unfortunately the patient died one week after hospitalization. We suggest that the hypercoagulable state provoked by malignant melanoma generated the CVST as brain MRI did not indicate any evidence of brain metastasis; therefore, direct invasion of the sinus by the tumor is less likely; on the other hand, we should not omit the possible role of microscopic sinus metastasis

Cerebral venous thrombosis associated with oligodendroglioma and pregnancy

Until now, cerebral venous sinus thrombosis [CVST] was principally an autopsy diagnosis; however, with the introduction of MRI and angiography, as well as enhanced clinical attentiveness, it is now reliably diagnosed during life. Herein, we describe a case of CVST accompanied by oligodendroglioma and pregnancy. In our patient, the following factors contributed to the formation of CVST: First, the pregnancy state, which is a known risk factor for developing venous thrombosis; and secondly, the oligodendroglioma could have changed the architecture of adjacent sinus [right lateral sinus] and provocation of the development of clot in the sinus

Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome in a patient with Thalassemia intermedia

Here, we present the first instance of Guillain-Barr‚ syndrome variant in a patient with beta thalassemia and iron overload who had a history of transfusion before the onset of symptoms. Our patient was a 50- year-old Persian woman with history of intermediate thalassemia who had been treated with pack cells because of low hemoglobin level. Ten days after transfusion, she developed numbness of arms, left sided ptosis, and afterwards dysarthria, dysphagia, and bilateral ptosis. Electrodiagnosis on day 12 revealed reduced repetition of f-waves in the upper limbs and reduced recruitment with 1+ fibrillation in facial muscles. Electromyography and nerve conduction velocities in the limbs were normal. After excluding other causes and according to electrodiagnosis, the pharyngeal-cervical-brachial variant of Guillain- Barre syndrome was considered and plasma exchange began. Following exchanges, significant clinical improvement was attained. Iron overload and possible transmission of infections from blood products might have contributed in the development of syndrome