Okay Vural

Efficacy of intravenous magnesium sulfate in the treatment of acute migraine attacks.

Objective.—To study the efficacy and tolerability of 1 g of intravenous magnesium sulfate as acute treatment of moderate or severe migraine attacks.

Auditory event-related brain potentials in fibromyalgia syndrome

ObjectiveThe aim of this study was to investigate cognitive functions using auditory event-related brain potentials (ERP) in fibromyalgia syndrome (FMS).MethodsThe P300 component of ERP was studied in 36 female FMS patients and 22 control subjects. The short form 36 (SF-36) medical outcome study was used to determine quality of life. Number of tender points and disease duration were noted. Cognitive functions were evaluated with P300.ResultsThe symptoms were discrepant in FMS (P<0.001). The scores of the eight SF-36 subgroups in FMS patients were significantly lower than in the control group (P<0.001). Fibromyalgia syndrome patients had prolonged latency and reduced amplitude of P300 (P<0.001). No correlation was found between the subgroups of SF-36, tender point count, disease duration, and P300.ConclusionThe results of our study reveal that FMS affects quality of life and dysfunction in cognitive abilities can be determined by brain event-related potentials.

Evaluation of periodic leg movements and associated transcranial magnetic stimulation parameters in restless legs syndrome.

Restless legs syndrome (RLS), a sensorimotor disorder characterized by unpleasant sensations commonly localized in the legs, is frequently associated with periodic limb movements (PLMs) during sleep. We investigated the role of transcranial magnetic stimulation (TMS) and cortical silent period (CSP) duration as diagnostic and monitoring tools in 20 patients with primary RLS before and after 1 month of treatment and also studied 15 normal age‐ and gender‐matched subjects. Polysomnographic assessment was undertaken and the PLM index determined in 17 of the 20 patients. We also studied the correlation between sleep efficiency index and CSP duration because of the increasing severity of the sleep disturbance and PLMs in patients with RLS. Our results demonstrate that the duration of the CSP was reduced in patients with RLS, and that dopaminergic treatment normalized this duration. There was no correlation between the PLM index and CSP duration. It may be speculated that PLMs and the CSP are due to different inhibitory mechanisms and they may be used separately as diagnostic and monitoring tools in patients with primary RLS. Muscle Nerve, 2005

α-interferon and isoprinosine in adult-onset subacute sclerosing panencephalitis

We report eight patients with adult-onset subacute sclerosing panencephalitis (SSPE), of which, four were treated with oral isoprinosine and four with intraventricular α-interferon plus oral isoprinosine. One of the four patients treated with oral isoprinosine died within two months, and the disease progressed in three patients. Of the four patients treated with oral isoprinosine plus intraventricular α-interferon, one showed mild progression, one remission, and the remaining two showed stabilization. The group of patients is relatively small, but our results suggest that treatment with oral isoprinosine plus α-interferon is effective for SSPE.

Essential palatal myoclonus in monozygotic male twins

Sirs: Palatal myoclonus (PM) is an uncommon movement disorder characterized by vertical oscillation of the soft palate at 1 to 3 Hz and usually bilateral. Occasionally some other brainstem-innervated muscles may be involved. Some authors call it a palatal tremor [10, 12]. We report monozygotic male twins with essential palatal myoclonus. To our knowledge this is the first report in which essential PM can be genetically linked. 39 year old twin brothers were admitted to hospital for treatment of involuntary movements of the soft palate and clicking in both ears. In their medical history it was learned that symptoms developed at the age of 14–15 years in one of them and at the age of 37 years in the other. The patients’ parents were healthy and unrelated. The patients’ general and neurological examination was unremarkable except easily audible clicking in time with the visible rhythmical movements of the soft palate. It was not associated with synchronized movements of eyes, tongue, larynx, diagram, intercostal muscles, extremity or trunk. The patient’s whose soft palate frequency had developed at the age of 14–15 years was 2Hz. symptoms, and was higher than his brother’s whose frequency was 1,5Hz. Chronic treatment with clonazepam, piracetam, valproic acid, amitriptiline and carbamazepine failed to improve PM in the first patient and clonazepam, carbamazepine, valproic acid were administered with no effect in the second. Because the different drugs did not improve the PM, five units of botulinum toxin (Botox®/Allergan) in 0.2 ml. saline solution were injected into each side of tensor veli palatini through a special needle under EMG guidance. The first patient reported some initial difficulty with swallowing, in the second patient there was no severe side effect. Three days after the injections PM and clicking began to diminish and had completely disappeared after 6 days in both of them. They remained free of the PM and clicking at the every three months followup visit for one year in the first patient and six months in the second. The investigations including routine blood chemistry, computed tomography (CT), magnetic resonance imaging (MRI), electroencephalography (EEG), somatosensory evoked potentials (SEP) and brainstem auditory evoked potentials (BAEP) were all normal. There are two forms of palatal myoclonus. Essential palatal myoclonus is characterized by a vertical oscillation of the soft palate at 1 to 3 Hz. unaltered by voluntary actions, and an annoying ear click. There are no other neurological signs or symptoms, and the pathophysiology of essential palatal myoclonus is unknown. Symptomatic palatal myoclonus is nearly always associated with syncronous movements of the eyes, larynx, pharynx, face, diaphragm, cervical and axial muscles. Some authors, however, point to difficulties in classifying their patients with palatal myoclonus as either the essential or the symptomatic type [1, 8, 9, 13]. Symptomatic palatal myoclonus produced by a lesion involving the pathway connecting dendate, red and inferior oliver nuclei. Olivary enlargement can be visible on MRI in some symptomatic cases [4, 5, 9, 13]. The patients may complain only of annoying ear click, the origin of which is uncertain, although many believe it arises from muscles contracting around the eustachian tube. The potential role of heredity in the etiology of essential palatal myoclonus is unknown. These monozygotic twin brothers with essential palatal myoclonus in which symptoms developed at the age of 14–15 years in one of them and at the age of 37 years in the other are of interest for suggesting the hereditary etiology of essential palatal myoclonus. In our monozygotic male twins the first symptoms of palatal myoclonus were noted 22 years later than in the other one. These findings indicate that there is a role of inheritance in the pathogenesis of essential palatal myoclonus but also that some other factors contribute to the clinical presentation of palatal myoclonus. Similar findings have been described in monozygotic twins with idiopathic dystonia and Huntington’s disease [3, 6, 11]. As the relationship between the disease and its genetic linkage could be confirmed in our patients, it might also be thought that the pathology may not be inherited. Successful treatment of essential palatal myoclonus by botulinum toxin has been reported several times [2, 7]. In our monozygotic male twins, five units botulinum toxin in 0.2 ml. saline solution were injected into each side of tensor veli palatini and both cases were rendered free of their myoclonus a few days after injection until 11 months in the first patient and 6 months in the second one. In conclusion, the essential LETTER TO THE EDITORS

Rhino-orbito-cerebral mucormycosis.

Sirs: Mucormycosis is usually a rapidly fatal disease that is caused by fungi of the class Phycomycetes. The fungus is saprophytic in the nasal cavity and paranasal sinuses and becomes pathogenic in some particular conditions. Uncontrolled diabetes mellitus, uraemia, leukaemia, lymphoma, septicaemia, AIDS, severe burns and long-term treatment with steroids predispose to mucormycosis [1, 2, 5–7]. We report an autopsyproven case of rhino-orbito-cerebral mucormycosis with unrecognised diabetes mellitus in which the course was rapidly progressive leading to death in 1 week. A 55-year-old woman was admitted to the hospital with swelling on the left side of her face since 1 week. Four days before admission, her left maxillary molar tooth had been removed. Her medical history was unremarkable, and she had no known history of diabetes mellitus. On examination, her body temperature was 38°C, she had hypaesthesia on the left side of her face, and there was swelling of the left eyelids; the rest of the examination including eye movement was normal. The pathological laboratory findings were as follows: white blood count 20,800/mm3, erythrocyte sedimentation rate 70 mm/h and blood glucose levels 232–355 mg/dl. One day after admission, she complained of diplopia and weakness of the right limbs. The next day, she developed nuchal rigidity, aphasia, right hemiplegia, total ophthalmoplegia of the left eye and gradual deterioration of the level of consciousness. An arterial blood gas specimen revealed an acidosis. Serum and cerebrospinal fluid (CSF) cultures did not reveal any micro-organisms. The CSF protein level was elevated, and there were 250 red and 20 white blood cells/mm3. Serology for HIV was negative. Cranial CT showed hypodense areas in the left temporal lobe. Antibiotics and heparin were instituted for the presumed cavernous sinus thrombosis, and insulin was given for diabetes mellitus. The next day, a progressive bluish discoloration of the skin on the left side of the her face was noticed (Fig. 1a). Histological preparations obtained from the nasal cavity and orbital lesion revealed nonseptate hyphae compatible with a mucormycotic infection. Subsequently, rhizopus was grown in culture. After diagnosis, treatment with IV amphotericin B 1 mg/kg per day was started. Within 1 day the patient was in deep coma, and the left facial skin lesion showed signs of necrosis (Fig. 1b). She died 4 days after hospitalization, and postmortem microscopic studies showed extensive fungal invasion of the periorbital soft tissues, nasal cavity, brain, and lung (Figs. 2, 3). The pathological hallmarks of mucormycosis are vascular invasion, ischaemic infarction, and haemorrhagic necrosis. Septic thrombosis of the central retinal artery, cavernous sinus and internal carotid artery are reported. The site of onset is usually the nasal mucosa, and the disease spreads rapidly to the palate, orbit, and brain [3–5]. Early diagnosis is important and is based on the clinical features such as presence of diabetes mellitus; black eschar in the nose or face; and demonstration of nonseptate, irregularly wide fungal hyphae in the biopsy of nasal mucosa or facial lesions. Before admission to our hospital, the patient was not known to have diabetes mellitus, and there was a history of infected maxillary molar tooth extraction. In our patient, the diagnosis was suspected because of extensive necrotic lesions of the face and rapidly progressive clinical course in spite of antibiotic therapy and was confirmed by mycological examination. The rhino-orbito-cerebral form of mucormycosis has a poor prognosis despite therapy and is often fatal within a few days [3, 4]. The involvement of the central nervous system and the absence of surgical LETTER TO THE EDITORS J Neurol (1998) 245 :689–690

Acute Fulminant SSPE: Clinical and EEG Features

We report an autopsy-proven case of SSPE in which the course was rapidly progressive, leading to death in 2 months. The patient was admitted for investigation of visual symptoms. His examination revealed complete right homonymous hemianopia and myoclonic jerks in the right half of the body. EEG showed high voltage periodic slow-wave discharges that were often associated with jerks. Measles antibody was positive in serum and CSF. MRI showed hyperintensity in the white matter of the occipital poles. The patient became completely blind and had jerks in all four limbs. His condition continued to deteriorate and within 3 weeks he reached a state of deep coma. The myoclonic jerks became less marked and gradually completely ceased. The EEG showed diffuse slow activity without periodic complexes. The patient died approximately 2 months from onset of disease. Brain autopsy revealed severe neuronal loss with abundant inclusion bodies in the remaining neuronal and oligodendroglial nuclei, which was compatible with SSPE.

Median nerve somatosensory evoked potentials recorded with cephalic and noncephalic references in central and peripheral nervous system lesions.

Somatosensory evoked potentials (SSEP) to electrical stimulation of the median nerve by using cephalic and noncephalic references were studied to detect the generator sources of short latency evoked potentials in 29 patients with cerebral, brainstem, spinal and peripheral nerve lesions. Patients were divided into six groups according to the localization of their lesions: group 1: cortical and subcortical lesions, group 2: basal ganglion lesions, group 3: pons and mesencephalon lesions, group 4: diffuse cerebral lesions, group 5: cervical cord lesions, group 6: brachial plexus lesions. Potentials were recorded using cephalic and noncephalic references after median nerve stimulation. Evidence obtained from patients suggested the following origins for these short latency SSEPs: P9 may arise in brachial plexus, P11 in dorsal basal ganglions or dorsal column, P13 and P14 in the nucleus cuneatus and lemniscal pathways, N16 in subthalamic structures and most likely mid and lower pons, N18 from the thalamus and thalamocortical tract, and N20 from primary somatosensory cortex.

Auditory brainstem responses in children with congenital heart disease.

Abstract Background: Cyanotic congenital heart diseases usually lead to growth and developmental delay in children due to chronic hypoxemia and undernourishment that may affect the central nervous system. The auditory brainstem responses are determined to assess the maturation and function of the brainstem. Therefore, we used the auditory brainstem responses to investigate the effect of cyanotic congenital heart diseases on brainstem maturation.

SPECT and MRI findings in a case of extensive neuronal migration disorder

We report a 20-year-old male with epilepsy, mild mental retardation, growth asymmetry, and MRI and SPECT features of unilateral subcortical ectopic cortex. The neurological examination showed mild growth asymmetry, hemiparesis and hemihypoesthesia and pyramidal signs on the left side. EEG showed focal abnormality in the right frontotemporal region. MRI revealed pachygyria and severe heterotopia associated with some abnormalities of ventricles and cerebellum on the right. Cortical responses were absent on stimulation of the left median and tibial nerves. Central motor conduction time from cortex to left upper extremity was prolonged in magnetic stimulation test. SPECT using 99 mTc-HMPAO revealed increased perfusion of the right subcortical region as compared with those of overlying cortical mantle and opposite hemisphere. To our knowledge, there has been no report documenting such a large and extensive subcortical ectopic cortex which appears as a mass distorting and shifting the middle structure in an adult, such as in our case.