Coexistence of myasthenia gravis and amyotrophic lateral sclerosis in a Bosnian male: an unusual clinical presentation.

PURPOSE: Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are two different diseases. The coexistence of both of them is extremely rare and represents a diagnostic challenge which requires thoughtful interpretation of clinical characteristics. CASE REPORT: We present the case of a 46-year-old Bosnian male who developed ALS five months after MG. Diagnosis of MG was based on elevated titers of anti-AchR antibodies, positive edrophonium test, and decremental responses on a repetitive nerve stimulation test while the diagnosis of ALS was based on clinical and neurophysiological findings: upper motor neuron signs in the lumbar region, lower motor neuron signs in the bulbar and cervical regions, generalized fasciculations and muscle atrophy and progressive asymetric muscle weakness together with active and chronic denervation in the cervical and lumbosacral region determined by electromyoneurography. CONCLUSIONS: The coexistence of MG and ALS is rare and request an adequate interpretation of clinical symptoms. The relationship between these two diseases in as interesting phenomen to present.

Clinicopathologic features in a case of intermuscular myopericitoma of thigh.

Myopericytoma is a benign tumor with the most common presentation as a well-circumscribed, slow-growing mass. It is frequently misdiagnosed as a sarcoma. We presented a 23-year-old patient with a history of a sciatic pain of the right leg. A careful physical examination discovered tumor-like mass in the posterior part of the thigh. Neurological finding showed a reduction of myotatic reflexes on the right leg with a weaker muscle strength on the right leg. The right leg musculature was slightly hypotrophic in the range of 2-3 cm comparing to left leg. Initially electrophysiological and radiological diagnostic with magnetic resonance imaging (MRI) of the lumbar spine, pelvis and thighs were normal. Magnetic resonance imaging of the right thigh discovered a slow growing 2.1 × 3.8 cm sized mass that was initially described by radiologist as a neurinoma. Patient was admitted to department of neurosurgery and operated on for a tumor removal. Tumor was located intimately to femur and sciatic nerve and after careful dissection completely removed. Patient was doing well after surgery and discharge after three days from the hospital. In the postoperative period the symptoms disappeared. Histopathology showed a myopericitoma. Postoperative MRI after three months of follow up showed no tumor residues, and after 6 and 12 months there was no tumor recurrence. Myopericytoma behave in a benign fashion, but, because local recurrences and rarely metastases may occur in atypical and malignant neoplasms, a careful follow-up after radical resection is recommended.

Plasmapheresis in neurological disorders: six years experience from University Clinical center Tuzla.

BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal blood purification technique that is designed to remove substances with a large molecular weight. The TPE procedure includes removal of antibodies, alloantibodies, immune complexes, monoclonal protein, toxins or cytokines, and involves the replenishment of a specific plasma factor. The aim of the study was to describe the clinical response to TPE in various neurological patients, and to assess the clinical response to this therapy. METHODS: The study was retrospective. We analyzed the medical records of 77 patients who were treated at the Department of Neurology, University Clinical Center (UCC) Tuzla from 2011 to 2016.   Results: 83 therapeutic plasma exchanges were performed in the 77 patients. There was a slight predominance of male patients (54.5%), with an average age of 51±15.9 years. The most common underlying neurological diseases were Guillain-Barré syndrome (GBS) (37.7%), then chronic inflammatory demyelinating polyneuropathy (CIDP) (23.4%), multiple sclerosis (MS) (11.7%) and myasthenia gravis (10.4%). Less frequent neurological diseases that were encountered were paraneoplastic polyneuropathies (5.2%), neuromyelitis optica (also known as Devic's disease) (3.9%), motor neuron disease (3.9%), polymyositis (2.6%) and multifocal motor neuropathy (1.2%). CONCLUSIONS: Six years experience of therapeutic plasma exchange in neurological patients in our department have shown that, following evidence-based guidelines for plasmapheresis, the procedure was most effective in patients with GBS, CIDP and myasthenia gravis.

[Obstetric lesions of brachial plexus]. / Porodajne lezije pleksusa brahijalisa.

BACKGROUND: Obstetrical brachial plexus lesion (OBPL) (also known in its various forms as Erb's palsy, Klumpke's paralysis, Erb-Duchenne palsy) complicates a very small proportion of births. Furthermore, it seems that more likely many cases recover with little in the way of remaining deficit, but it is equally certain that some cases will not recover. Electro diagnostic examinations are used as following the physical examination and can provide data on both the severity and timing of the injury. The initial study usually is performed 2-3 weeks after injury, when signs of enervation are seen in children with moderate or serious injuries. The incidence of obstetric brachial plexus palsy varies from 0.4 to 1 case per 1000 new born children. This incidence has remained unchanged since the beginning of this century despite current technologic advances. The aim of this paper is to review the literature and authors experience with OBPL. PATIENTS AND METHODS: The study included 30 children examined in last 10 years at Electro myoneurography laboratory, Department of Neurology, University Clinical Center Tuzla, Bosnia and Herzegovina. RESULTS AND DISCUSSION: Total out of 20 children (66.67%) were male, and 10 (33.3%) female; in 11 (36.67%) palsy was on left, and in 19 (63.33%) on right side. Erb's palsy was seen in 27 (90%) and total palsy in the remaining 3 (10%) limbs. Electromyography was very useful test not only for diagnosis, but also for evaluation of recovery. Good recovery was observed in majority of these children, but some children has difficulty with active shoulder abduction, forward flexion, symmetric elbow flexion and forearm supination at age of one year and more, and mild shortening and atrophy of the limb are also observed.