Neurological Disorders

Beraprost sodium is a new stable, orally active Prostaglandin I2 analogue. The aim of this study was to determine the effect of beraprost on cognitive dysfunction and locomotor impairment induced by bilateral common carotid artery occlusion in mice. We investigated the ameliorating effect of beraprost through PGI2 IP receptor by studying neurologic deficit assessment and T-maze testing in young and old male C57Bl/6 wild-type (WT) and IP receptor knockout (IP KO) mice following a 12 min bilateral common carotid artery occlusion (BCCAo) and 7 days of reperfusion. Beraprost reversed BCCAo induced cognitive impairment and neurological deficit in a dose dependent manner. Immunohistochemical studies showed attenuation of neuronal cell death, astrogliosis, microglial invasion, and myeloperoxidase (MPO) activity in both young and old WT mice after post treatment with beraprost. Moreover, after BCCAo, phosphorylated cAMP response element binding protein positive cell numbers were increased with beraprost treatment over vehicle treated controls. These results show that beraprost treatment attenuated cognitive dysfunction and neurological deficits induced by BCCAo, and suggest that this effect may be mediated by the neuroprotective effects of treatment.

Migraine is an extremely common disorder and has co morbidity with many neurological illnesses. After reviewing the neurophysiological and biochemical basis of the research findings and hypotheses of migraine and other neurological disorders I present to the best of my knowledge the first para nasal sinus nitric oxide mediated neurobiophysiological explanation for many neurological disorders. The etiology of neurological illnesses is mainly due to neurotransmitter imbalance, neurodegenerative changes acute and chronic inflammation, effects of hypo and hyper endothelial and neuronal NO levels and genetic predisposition. According to this para nasal sinus nitric oxide based description those effects are mainly brought on by the sinorhinogenic impulse distribution of the central nervous system except genetic predisposition. Moreover, avoidance of the central neuronal influence and stress to the brain in early childhood or young age caused by migraine would help to prevent the progression or aggravation of the neurological illnesses. Indeed this article explains a new pathophysiological initiation between sino rhinogenic nitric oxide effects and neurological disorders and provides an etiologically important Neuro vascular impulse generating pathway to cause or aggravate neurological disorders. Therefore the patients who are clinically suspected of having migraine headache and neurological disorders or along with susceptible neurological disorders should receive comprehensive sinorhinological examination and evaluation based on the sinus hypoxic nitric oxide phenomena. A standard surgical and medical management of migraine that links with the sinus hypoxic nitric oxide theory are suggested to be used for even neurological disorder as a new treatment of neurological illnesses and to prevent the dysfunction of central specific neural circuits.

Spinocerebellar ataxias are a group of phenotypically and genetically heterogenous disorders characterized by progressive degeneration of the cerebellum with overlapping symptoms. A novel form of SCA has been described with triplet repeat expansions in the 3 UTR of the SCA8 gene and is caused by expansion of a CTG/CTA repeat in the ataxin-8 opposite strand gene (ATXN8OS) located on chromosome 13q21. Analysis of CTA/CTG repeats in SCA8 gene was performed in 188 ataxia patients and 100 healthy volunteers without any neurological signs or family history. The repeat length was found to be highly polymorphic. We were unable to find any individual with pathogenic repeat length in SCA8 gene, when we used the already established pathogenic repeat criteria. However, repeats >35 (4.7%) were exclusively found in patients only and none of the controls suggesting that these repeat sizes could be pathogenic for our population. The frequency of LN alleles was also found to be higher than reported for other populations. The percentage of LN alleles at SCA 8 locus was 65% and 47% in patients and controls. In the present study three patients also exhibited repeats lower than the normal range and the pathological implications of these needs to be explored.

While Chiari I malformation is a relatively common finding on MRI imaging, the vast majority of patients are asymptomatic and those who do present typically follow a slow course of chronic or subacute progression of symptoms. Emergent deterioration of existing Chiari malformation is rare. We present two patients with previously undiagnosed Chiari I malformation presenting with acute deterioration, one as a result of trauma and the other due to meningitis. Both of the patients required emergent surgical intervention, and each experienced recovery by postoperative month three. We present these two cases and review the pertinent literature.

Cerebrovascular diseases cause brain degeneration and subsequent decline of cognitive functions. In this study, comprehensive magnetic resonance imaging approaches with both structural and blood flow imaging were used to characterize the white matter hyperintensity in the brain, cerebral blood flow, and obstruction of vertebral artery caused by stenosis in various cases of individuals with cerebral vascular and cardiovascular risks. It is demonstrated that vertebral artery stenosis characterized as vertebral artery narrowing and/or reduced blood flow velocity by MRI may be associated with the regional specific cerebral vascular comorbidities detected as white matter hyperintensity and reduction of cerebral blood flow. More specifically, unilateral vertebral artery stenosis led to asymmetric periventricular WMHs, while bilateral vertebral artery with lower blood flow led to symmetric periventricular WMHs. The comprehensive MRI protocol with functional and high resolution structural imaging sequences is capable of providing valuable information on blood flow supply in the vertebral artery and cerebrovascular ischemia in individuals having vertebral and cardiovascular abnormalities.

Encephalitis lethargica (EL) is a CNS disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism), and neuropsychiatric sequelae. It was first described in 1916, but only sporadic cases have been described since the 1916-1927. EL is a rare disease and difficult to confirm because no diagnostic criteria have been agreed. Its additional common features include oculogyric crises and ocular features (ophthalmoplegia and ptosis). However, no case of decreasing visual acuity has been previously reported. We report a case of EL with accompanying bilateral blindness. A 31-year-old female patient visited our emergency room complaining of a headache, fever, pharyngitis, and poor oral intake. After the fever and headache subsided, the patient experienced drowsiness and bilateral decreases in visual acuity. Brain magnetic resonance imaging (MRI) showed increased signal intensity on the hypothalamus on fluid attenuated inversion recovery (FLAIR) and T2 weighted images, which was compatible with EL. Although we critically discuss other potential etiologies, we conclude that her blindness constitutes an unusual presentation of EL.

Hair dye poisoning has been evolving as one of the significant causes of intentional self-harm in the developing world. Hair dyes contain Paraphenylene-Diamine (PPD) and a host of other chemicals that can cause laryngeal edema, severe metabolic acidosis, rhabdomyolysis and acute renal failure. We present the case of a young woman who presented with complaints of progressive upper and lower limb weakness following PPD poisoning. The patient was diagnosed with the critical illness neuropathy on EMG and by history and examination. The patient was treated symptomatically and did well. Awareness and knowledge of this disorder and intervention at the right time is essential for physicians, nurses and health care workers to ensure appropriate care and treatment of such cases.

Introduction: Paediatric neurovascular diseases include different patterns, such as cerebral aneurysms, arteriovenous or vein of Galen and cerebral cavernous malformations, as well as ischemic stroke and other infrequent clinical entities like Moyamoya disease. Management and treatment of this topic requires well-coordinated multidisciplinary care of pediatric neurosurgeons, interventional neuro-radiologists and paediatricians.

Materials and methods: We present six cases of neurovascular pathologies in paediatric patients and highlight the multimodal treatment and management strategy. This case-series included patients with Vein of Galen malformation, Arteriovenous-malformation-bleeding, cerebellar ischaemic stroke, dissecting PICA-aneurysm and one patient with a vascular compression of the vestibular nerve inside the internal auditory meatus.

Results: All patients show a good outcome because of a fast and interdisciplinary work and a close collaboration of the neurosurgeon, the neuroradiologist and the neuropadiatrician.

Discussion: A multimodal therapy-management with an effective interdisciplinary setting including the pediatric neurosurgeon, the interventional neuroradiologist and the neuropaediatricians is necessary to reach the best outcome of the patient. Paediatric neurovascular diseases should be treated in an experienced centre with good infrastructure and good interdisciplinary work.

Wernicke’s Encephalopathy (WE) is a preventable neurologic condition characterized by altered mental status, ophthalmoplegia, and ataxia. Although historically associated with alcoholism, a few authors have described WE in patients with non-alcohol related psychiatric disorders. We report herein the case of a 36-year-old young man with paranoid schizophrenia who was brought to hospital for confusion and difficulties with his vision. His roommate said he had gone about thirty days without eating ‘…because he was on a slimming cure’. History and physical examination suggested WE as a result of isolation and poor diet leading to nutritional deficiency. This was confirmed by brain magnetic resonance imaging showing classic thalamic, mammillary bodies and brainstem lesions. Of note, his cognitive profile was far more heterogeneous than what had classically been described in the literature and involved both cortical and subcortical pathology, generating memory but also significant executive deficits. Intravenous treatment with thiamine was given and our patient showed mild improvements in visual acuity and nystagmus. However, persistent cognitive and physical disabilities consistent with Korsakoff syndrome remained, and he now lives in a supervised home. This case illustrates the tragic consequences of nutritional deficiencies in a patient with paranoid schizophrenia. The threshold to suspect WE in schizophrenic patients should be lowered and in doubt prophylactic parenteral thiamine should be administered.

A 57 year-old woman searched for a neurological Service for the first time when she was 45-year-old, complaining about weakness in lower limbs for ten months period. The physical examination reported was of sensitive level at T5, reduced proprioception, Romberg’s sign and ataxic gait. A Work up discovered a low Vitamin B12 level of 64pg/ml. Vitamin B12 reposition was initiated, however the patient failed to follow up.