Multiple Sclerosis and Intracranial Hypertension Presenting as Paroxysmal Kinesigenic Dyskinesia

Author:  Dr. Daniel E Jacome

Background: Paroxysmal movements in the form of tonic spasms, dystonia, choreoathetosis and hemidyskinesia have been reported in patient with multiple sclerosis (MS). Intracranial hypertension with headache and papilledema resembling idiopathic intracranial hypertension (IIH) exceptionally occurs in patient with MS. Conversely, IIH may rarely present as movement disorder in children.

Aims of the study: Report an unusual patient with MS and intracranial hypertension anteceded by movement disorder.

Methods: Clinical examinations and ancillary diagnostic testing.

Results: A 54 year old patient presented one year earlier to her diagnosis of MS with symptoms of paroxysmal kinesigenic diskinesia (PKD). Her initial neurologic examination and brain MRI were normal. A year later her examination became abnormal and her MRI showed demyelination. Her cerebrospinal fluid contained oligoclonal bands and the opening pressure was markedly elevated, in the absence of headache and papilledema.

Conclusion: PKD may antecede MS and acephalgic intracranial hypertension without papilledema in exceptional patients.

Repulsive Guidance Molecule-a and Demyelination: Implications for Multiple Sclerosis

Authors: Kubo T, Tokita S, Yamashita T.

Drug development for neurodegenerative and neuroinflammatory diseases such as multiple sclerosis and traumatic brain injury is challenging. One promising strategy is to target a molecule with multiple biological actions affecting divergent pathophysiological disease phases simultaneously since these diseases arise from multiple pathological phases. In recent years, we pursued this strategy with a focus on multiple sclerosis and spinal cord injury and found that repulsive guidance molecule-a (RGMa) inhibits regeneration of injured CNS axons following spinal cord injury. We also found that RGMa enhances CD4(+) T cell activation facilitating CNS demyelination in an animal model of MS, mouse experimental autoimmune encephalomyelitis (EAE), which supports the idea that RGMa has distinct pathological actions. The multiple functions of RGMa in the CNS and the immune system would provide a therapeutic opportunity to concurrently block the autoimmune reactions and axon injury in neurodegenerative and neuroinflammatory diseases. In this article, we introduce the therapeutic potential of targeting RGMa as a novel intervention for MS and spinal cord injury.

Increased Risk of Multiple Sclerosis after Traumatic Brain Injury: A Nationwide Population-based Study

Authors: Kang JH, Keller J, Lin HC.

Previous data show conflicting results regarding the association between MS and prior brain trauma. This study aims to investigate the risk for MS following a traumatic brain injury (TBI) using a population-based dataset. This study used data from the National Health Insurance Research Database. In total, 72,765 patients with TBI were included as the study cohort, and 218,295 randomly selected subjects were matched with the study cohort by sex and age as controls. We traced each patient individually for a six-year period from their index health care utilization to identify those who received a subsequent diagnosis of MS. We used the Kaplan-Meier method and the log-rank test to compare the difference in six-year MS-free survival rates between the two groups. Stratified Cox proportional hazard regressions were computed to compare the risk of developing MS for these two cohorts. The incidence rate of MS was 10.51 (95% CI: 7.60-14.16) per 100,000 person-years in patients with TBI and 6.63 (95% CI: 5.30-8.20) per 100,000 person-years in patients without TBI. After censoring cases who died from non-MS causes, stratifying for hospitalization of cases as a proxy for severity, and adjusting for monthly income and geographic region of the community in which the patient resided, the HR of MS for patients with hospital-treated TBI injuries was 1.97 (95% CI=1.31-2.93, p<0.01) that of patients without TBI during the six-year follow-up period after index healthcare use. Our study concludes that patients with TBI are at higher risk for subsequent MS over a six-year follow-up period.

Neurosurgical treatment of tuberous sclerosis complex lesions

Author: Pascual-Castroviejo I.

BACKGROUND: Tuberous sclerosis complex (TSC) is an autosomal dominantly inherited syndrome. Renal disease is the main cause of death. Brain disorders are the origin of more frequent and severe problems, such as tumors, epilepsy, and mental retardation. Participation of neurosurgeons in the study and especially in the treatment of TSC patients is often required.

MATERIALS AND METHODS: Two types of pathological conditions mainly require neurosurgical interventions in TSC: subependymal giant cell astrocytomas (SGCA) and cortical tubers. SGCA are located in the cerebral region close to the foramina of Monroe, uni- or bilaterally, and originate in hamartomas that can grow slowly as well as rapidly, even suddenly, especially in cases with intratumoral cyst, causing increased intracranial pressure (ICP) with severe risk for visual loss and life. Neurosurgeons have to participate in the follow-up of the patients as soon as the risk of ICP exists to remove the tumor when the criteria of SGCA growth are present. The other intracranial lesions that require neurosurgical intervention by are the cortical tubers.

CONCLUSION: These dysplastic lesions are associated with TSC in almost the 100% of affected persons and are the cause of epilepsy in most patients. The seizures can be resistant to antiepileptic medication in many cases in which a tuber is identified as the origin of the focal seizures after functional studies, such as EEG, MR, PET, etc. In these cases, only surgical removal of the tuber and the perituberal epileptogenic foci can cure the epilepsy. Large tubers are more epileptogenic than smaller ones.

Etiologic features of newly diagnosed epilepsy: Hospital-based study of 892 consecutive patients in West China

Authors: Si Y, Liu L, Hu J, Mu J, Fang JJ, An DM, Zhao LL, Tian LY, Zhou D.

PURPOSE: We evaluated data from a large cohort of newly diagnosed epilepsy patients from the biggest epilepsy center in West China. The aim was to determine the most prevalent etiologic factors in this region.

METHODS: From May 2008 to May 2010, the clinical data of patients with newly diagnosed epilepsy were consecutively, systematically and prospectively recorded in a database. The data were analyzed according to sex, age, seizure type, etiology, and other factors.

RESULTS: The present study examined 892 patients with newly diagnosed epilepsy. Among these patients, 346 (38.8%) were confirmed as symptomatic, with the largest constituent ratio among the elderly (63.2%). In this symptomatic group, central nervous system (CNS) infections and traumatic brain injuries (TBI) were the two most common etiologies. When analyzed according to age bracket, cortical dysplasia, mesial temporal sclerosis, and CNS infection were the most frequent causes among young patients (<18 years). On the other hand, CNS infection and TBI were the two most common causes in patients between 18 and 60 years. Stroke was the most common cause of newly diagnosed symptomatic epilepsy in the elderly (>60 years).

CONCLUSIONS: More than 30% of newly diagnosed epilepsy cases were shown to be symptomatic by medical history as well as careful clinical and laboratory examination. Detailed epilepsy assessments are essential to formulate a therapeutic plan and to improve prognosis. The etiology spectrum found in this large cohort forms a comparative baseline for future studies.

The possible role of cranio-cervical trauma and abnormal CSF hydrodynamics in the genesis of multiple sclerosis

Authors: Damadian RV, Chu D.

UPRIGHT Multi-Position MR scanning has uncovered a key set of new observations regarding Multiple Sclerosis (MS), which observations are likely to provide a new understanding of the origin of MS. The new findings may also lead to new forms of treatment for MS. The UPRIGHT MRI has demonstrated pronounced anatomic pathology of the cervical spine in five of the MS patients studied and definitive cervical pathology in the other three. The pathology was the result of prior head and neck trauma. All eight MS patients entered the study on a first come first serve basis without priority, and all but one were found to have a history of serious prior cervical trauma which resulted in significant cervical pathology. The cervical pathology was visualized by UPRIGHT MRI. Upright cerebrospinal fluid (CSF) cinematography and quantitative measurements of CSF velocity, CSF flow and CSF pressure gradients in the upright patient revealed that significant obstructions to CSF flow were present in all MS patients. The obstructions are believed to be responsible for CSF "leakages" of CSF from the ventricles into the surrounding brain parenchyma which "leakages" can be the source of the MS lesions in the brain that give rise to MS symptomatology. The CSF flow obstructions are believed to result in increases in intracranial pressure (ICP) that generate "leakages" of the CSF into the surrounding brain parenchyma. In all but one MS patient, anatomic pathologies were found to be more severe in the upright position than in the recumbent position. Similarly, CSF flow abnormalities were found to be more severe in the upright position than in the recumbent position in all but one MS patient. Images of the MS patient anatomic pathologies and CSF flow abnormalities are provided with comparison images from normal examinees in Figures 1-15.


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