Neurological Disorders

There are a number of case reports in my publication history and I had the one or the other conversation with colleagues why to publish case reports. Case reports are not an essential step in someone’s career and are more or less a waste of time, is the main argument I was “accused” for. I disagree with this argument and I would like to explain why in this short editorial.

Alzheimer’s disease (AD) is one of the most devastating and debilitating diseases affecting the aging population. Dementia is the most widely known deficit in AD patients, typically starts with loss of recent memory (minutes to hours), and progresses to loss of long-held memories and unable to recognize their loved ones. People with AD eventually lose their ability to take care of themselves. As a consequence the cost of care is huge, and it also takes a tremendous emotional toll on the family of AD patients. Two hallmarks of AD are senile plaques and neurofibrillary tangles, which are composed of amyloid β (Αβ) and tau, respectively. Despite extensive search for effective treatment over the past decades, available drugs have limited efficacy without affecting the course of AD.

Objective: To test the hypothesis that strength will have a correlation with balance in individuals with Parkinson’s Disease (PD) and will not be associated with balance in our healthy controls. Design: Cross-sectional Setting: Tertiary care university hospital and Veteran’s Hospital. Participants: 27 individuals with a diagnosis of PD and 25 age and gender matched controls Intervention: Not applicable. Main Outcome Measures: Balance as measured by sensory organization test scores separated into trials 1-3 (static balance) and 4-6 (dynamic balance) and strength as measured by work and power in knee flexion and extension. Results: Strength and balance were not found to have a significant association in the control group. However, in the PD group dynamic balance was found to have significant associations with all measures of strength: work of knee extension, work of knee flexion, power of knee extension and power of knee flexion. Conclusions: Knee flexion and extension strength have a significant correlation with dynamic, but not static, balance in individuals with PD and no significant correlation was found between strength and balance in the control group.

Study design: Prospective observational study of consecutive patients. Objective: Body Mass Index (BMI) has recently been investigated for its association with adverse events during lumbar fusion surgery and remains a controversial issue. We attempt to evaluate the impact of BMI on the incidence of perioperative complications after elective, degenerative, posterior lumbar fusions in a surgery-naïve population. Methods: We prospectively studied 77 consecutive patients undergoing open posterolateral lumbar fusion procedures at a single tertiary care center. Perioperative adverse events were defined by previously published reports and collected during the inpatient stay. Inclusion criteria reflected patients undergoing elective instrumented lumbar fusions while patients with revision surgery or history of infection, trauma or tumor were excluded from the study. Results: There were 39 non-obese (BMI<30) and 38 obese (BMI>30) patients. Overall, 38% of patients suffered at least one complication. Forty one total complications occurred, 76% of those were minor and 24% were major complications. There was a significant difference in the rate of total complications between two groups with 13 adverse events in non-obese and 28 events in obese group (p=.028). Using a backward elimination multivariate stepwise regression model based on maximum likelihood, obesity was an independent predictor of total complications (p=0.02, OR 1.082, 95% confidence interval 1.012-1.157). Conclusion: Lumbar fusion surgery is one of the most common spinal procedures and continues to be on the rise over the past decade. This prospective evaluation of patients undergoing elective degenerative lumbar fusions correlated obesity with increased incidence of total perioperative complications. These results argue for a more conservative approach and stringent surgical selection in obese patients.

A 31 year old woman presented with severe frontal headache and blurry vision about 6 days after her normal delivery. Her blood pressure was 193/111 mmHg and her liver and renal function tests and coagulation studies was normal. Her CT Head showed bifrontal subarachnoid hemorrhage (SAH) and MR Angiogram showed characteristic “beaded” appearance of multiple branches of the middle and anterior cerebral arteries bilaterally. The patient was treated with nimodipine and normal saline infusions. Her repeat MRA after one week showed resolution of SAH with normal anterior, middle, and posterior cerebral arteries. The patient was diagnosed with Postpartum cerebral angiopathy.

Carbon Monoxide (CO) is a colorless and odorless gas which is nonirritating and is produced whenever there is incomplete combustion of any fossil fuel. There are diverse clinical effects of CO poisoning and they may be easily confused with other illnesses, such as nonspecific viral illness, benign headache, and various cardiovascular and neurologic conditions. Although initial neurologic manifestations may be subtle, but more profound exposure may produce altered mental status, confusion, syncope, seizure, acute stroke-like syndromes, and coma. Isolated seizures have been reported more often in pediatric patients. The cardiovascular effects of CO poisoning are manifested initially by tachycardia in response to hypoxia but more significant exposures may result in hypotension, arrhythmias, ischemia, infarction and even cardiac arrest. We present here an interesting case of one such patient who presented with features of severe myocardial depression and uncommon MRI findings secondary to carbon monoxide poisoning

False localising sign is a term coined to indicate a clinical sign which is not in concordance with expected anatomical localisation of pathology. Bilateral abducent nerve palsy is often considered to be false localising sign, a sign of raised intracranial pressure. However bilateral abducent nerve palsy may rarely occur in absence of raised intracranial pressure.

Alzheimer’s disease is a mental disorder that accounts for 60-70% of dementia. Its specific causes have not been determined to date. It is understood as neurodegenerative diseases which progressively gets worse over time. It is characterized by short term memory loss, language disorientation, mood disorders, lack of motivation and self-care, and as well as behavioral challenges. Management’s strategies of AD are limited and are not effective. Common therapeutic mechanisms target the inhibition of acetyl cholinesterase and N-methyl D aspartate (NMDA). Post mortem evaluation and Magnetic resonance imaging techniques have demonstrated similarities between Multiple Sclerosis and Alzheimer’s disease in pathogenesis of the disease through neurodegeneration and inflammation. Similarly, findings using Echo-Color Doppler technique and MRI along other techniques have demonstrated a strong association of development of Chronic CerebroSpinal Venous Insufficiency (CCSVI) with MS resulting from vascular damage of jugular veins leading to changes in the hemodynamics in the brain.

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Edaravone, a powerful free radical scavenger, is the drug available in the clinical practice for the treatment of cerebral infarction and amyotrophic lateral sclerosis. Recently, many generic Edaravone injections have been commercialized. Substitution of additives in generic injections has the potential effect of changing the antioxidant ability of the original Edaravone injection. We investigated the dissimilarity between original and generic Edaravone injections focusing on their free radical scavenging activities determined by electron spin resonance spectroscopy. There were no significant differences between the original and generic Edaravone regarding the antioxidant abilities toward the hydroxyl radical. However, the generics in which the additive L-cysteine was substituted with glycerin or citric acid showed significant reduction in their antioxidant activity toward superoxide (p <0.01). Our in vitro findings suggest that the antioxidant ability of generic Edaravone against the hydroxyl radical is equivalent to that of the original Edaravone and that substitution of additives in the generic Edaravone might change its antioxidant activity toward the superoxide.

Rheumatic manifestations have been reported to occur in as many as of 41% in patients with infective endocarditis (IE) [1]. Back pain is the second most common rheumatic manifestation of IE, although, the occurrence of both infective endocarditis and spondylodiscitis is rare, with an estimated general incidence of 2.5% [2,3]. Among such cases, Streptococcus bovis is the most frequently isolated etiologic agent due to its specific tropism for vertebra [4]. Streptococcus bovis endocarditis usually presents with an indolent course and tends to occur in older males with underlying colonic pathology [5]. Streptococcus bovis spondylodiscitis commonly affects the lumbar spine, has a slow and insidious onset and is usually managed successfully with intravenous antibiotics [6-8].

Progressive multifocal leukoencephalopathy (PML) is a frequently fatal demyelinating condition of the central nervous system in which reactivation of the human polyomavirus JC (JCV) leads to lytic infection of oligodendrocytes. JCV reactivation typically occurs in the setting of profound impairment of cellular immunity seen in conditions such as human immunodeficiency virus infection and acquired immune deficiency syndrome (HIV/AIDS), hematologic malignancies, autoimmune diseases, and treatment with immunosuppressive medications. However, an emerging body of literature suggests that minimal or occult immunosuppression may be sufficient for the development of PML in certain cases. We report the case of a 52 year old man diagnosed with PML without history of or risk factors for immunocompromise, with absolute number of CD4 + T cells below the lower limit of normal but not meeting criteria for idiopathic CD4+ lymphocytopenia (ICL), who was subsequently found to have evidence of hepatic cirrhosis of unclear etiology. This is only the eighth case published of hepatic cirrhosis as the main identifiable risk factor for the development of PML and informs the ongoing discussion on mechanisms of moderate immunocompromise sufficient to allow for occurrence of this disease.

Background: Recently it has been shown an increase in the interleukin 1 beta and nitrite levels in cerebrospinal fluid as a primary response of the immature brain to oxygen deprivation in newborns that suffered perinatal asphyxia, 30% to 40% of these patients later develop neurological abnormalities incluiding cerebral palsy. Formerly was shown that an increased enzyme activity of NOS2 is responsible for the increase in nitrite levels in cerebrospinal fluid. The NOS2A gene has a polymorphic microsatellite (CCTTT)n located at -2.6 Kb from the gene promoter. The expansion of this microsatellite to 13 or 14 repeats increases transcription of the NOS2A gene and triples the nitric oxide level under hypoglycemia and hypoxia conditions. The study aim was shown that the expansion of -2.6 Kb CCTTT microsatellite in the NOS2 gene promoter, constitutes a risk factor for developing cerebral palsy in newborns that suffered perinatal asphyxia Methods: Genomic DNAs purified from peripheral leukocytes of 48 ICP patients and 57 healthy children, the (CCTTT)n microsatellite expansion were amplified by PCR, purified from agarosa gel in micro-column method and sequenced using genomelab methods development kit cycle sequencing dye terminator in an automated CEQ8000 sequencer. Results: The presence of a 14-repeat is significantly associated with infant cerebral palsy (Fisher P value=0.0122). Multivariate analysis adjusted for age and sex confirmed the association with an increased risk of developing infant cerebral palsy (odds ratio, 1.78; 95% confidence interval, 1.150-2.752; P=0.01). Conclusions: Our findings suggest that an expansion to 14 repeats of the CCTTT microsatellite plays a key role in the development of cerebral palsy in children that suffered perinatal asphyxia.

Dacrystic seizures are rare clinical occurrences characterized by paroxysmal stereotyped crying with a special challenging differential diagnosis with psychogenic non-epileptic seizures (PNES). We present a report of a male 21-yearold patient with history of schizophrenia, agitation crises and apparent seizures comprising a myriad of consciousness, motor, visual and autonomic manifestations. The patient began to smoke cannabis at age 15 and at age 18 smoked crack for 6 months. The seizures began at approximately this time and were incapacitating. In intercritical periods, he was friendly, communicative and displayed hysterical personality traits, but examination indicated no negative or positive symptoms when abstinent. Cognitive assessments revealed presence of intellectual deficiency (current QI=75). During hospitalization, the patient was extensively examined, and the diagnosis of schizophrenia was ruled out. All antipsychotic drugs were gradually tapered off. Video- EEG monitoring captured a prolonged seizure compatible with a dacrystic seizure. A cranial MRI scan revealed discrete diffuse cerebral atrophy. Carbamazepine was introduced and titrated until 1,600 mg/day and patient has been seizure-free for about 12 months. This case illustrates the importance of differential diagnosis of putative psychogenic seizures. This is the first report of dacrystic epileptic seizures associated with PNES and comorbid abuse of crack.

Lipomas are very common benign tumors; however, only four cases of headaches caused by extracranial lipomas have been reported. Moreover, the headache phenotypes and clinical course have been poorly described. Herein, we describe a case of 50 year-old-man who developed new onset predominately right-sided frontotemporal cluster-like headache since age 48. In 2005, he had generalized headaches that improved after a resection of an occipital lipoma. Imaging showed regrowth of the lipoma at the C1-C2 spinal level. Treatment with Excedrin tabs and sumatriptan injections helped within 20 minutes. His symptoms were worse in the summer and spring. He spontaneously improved after a few months. We review cases of lipoma-induced headaches in the literature, several of which resemble a trigeminal autonomic cephalalgias and are associated with compression of trigeminal or cervical peripheral nociceptors. This case offers the opportunity to discuss the possible pathophysiological mechanisms underlying cluster-like headaches symptoms and the relation with pain referral from cervical structures to the head.