Neurolist Journal Watch

Neurology 03/24/2009

 

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Parkinson's Disease

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Comparison of functional MRI differences patients heterozygous for either Parkin or PINK1 mutations and controls revealed consistent recruitment of rostral supplementary motor area and rostral dorsal premotor cortex in patients heterozygous for the mutations. The study concludes that patients heterozygous for the genes linked to recessive inherited Parkinson's disease demonstrates increased recruitment of rostral supplementary motor area and rostral dorsal premotor cortex area in order to compensate for the mild dopaminergic deficits.

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Alzheimer's Disease

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MRI studies of 49 patients with mild cognitive impairment are compared with patients with mild Alzheimer's disease and controls to evaluate for presence of cortical changes typical of mild Alzheimer's dementia. Patients were then followed to see whether any of the patients with mild cognitive impairment later developed Alzheimer's disease. The goal of the study is to evaluate whether cortical changes on MRI can serve as a predicative tool for patients with cognitive impairment. Authors concluded that thinning of specific cortical areas in patients with questionable Alzheimer's disease or mild cognitive impairment do predict future development of Alzheimer's disease.

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CSF from 117 patients with Alzheimer's disease were obtained and analyzed for the evaluation of the relationship between the various biomarkers and cognitive functions in patients with Alzheimer's disease. Study found that clusters of biomarker levels in CSF correlates with the cognitive profiles of patients with Alzheimer's disease. The study also found that extremely high tau level and phosphorylation of tau protein at threonine 181 is associated with a specific cognitive profile not related to the disease severity.

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Cognition

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Neuropsychiatric evaluations were given to 83 patients with asymptomatic, severe unilateral internal cerotid artery stenosis to assess whether carotid stenosis can induce specific cognitive impairment. Study found decreased performance in phonemic verbal fluency in patients with left internal carotid stenosis and decreased cerebrovascular reactivity compared to controls. Meanwhile, patients with right internal carotid artery stenosis and decreased cerebrovascular reactivity scored less on Colored Progressive Matrices and Complex Figure Text Copy compared to controls. Authors suggested that change in cerebrovascular reactivity may result in impairment of cognitive functions related to the side of the carotid stenosis. 

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Vertigo

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Bone mineral densitometry of anterior posterior lumbar spine and femur were performed on 209 patients with idiopathic benign positional vertigo and 202 controls. The authors concluded that there might be possible association between osteopenia/osteoporosis and idiopathic benign positional vertigo.

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Multiple Sclerosis

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Optical coherence tomography (OCT) was used to evaluate 22 patients with neuromyelitis optica (NMO) and 47 patients with multiple sclerosis. The study revealed decreased mean retinal nerve fiber layer (RNFL) in neuromyelitis optica patients with history of optic neuritis. The superior and inferior quadrants are more affected. The authors concluded that optic neuritis in patients with neuromyelitis optica is associated with thinning of RNFL, with the superior and inferiot quadrants more involved. The authors also suggested increased axonal injury in NMO compared to MS. Finally, the authors concluded that OCT as a reliable way to distinguish optic neuritis caused by NMO or MS. 

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Niemann Pick Disease

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Horizontal saccadic and antisaccadic measurements were obtained through infrared limbus eye tracker in 3 patients with Niemann Pick Disease type C and 10 controls. The study found that patients with most severe biochemical, cognitive, and symptom deficit perform the poorest on the frontal and brainstem ocular measures. Meanwhile, patients with less severe illness was found to have abnormally reduced saccade latency. The authors concluded that ocular motor measures can be used as a tool to assess the disease progression in patients with Niemann Pick Disease type C.

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Amyotrophic Lateral Sclerosis

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In a survival study involving a cohort of 1188 patients in London, UK and 432 patients in Melbourn, Australia, patients with brachial amyotrophic diplegia and pseudopolyneuritic variant of ALS were found to have better survival than patients with typical ALS.

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Botulinum Toxin

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Review article discussing about the effect of botulinum toxin on the CNS and possible clinical significance.

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Case Report: 22 year old male with MS accidentally overdosed on 4-aminopyridine (4-AP, a potassium channel blocker) developed agitated behavior, fever, hypertension, tachycardia, and eventual abulia and bilateral medial temporal lobe hyperintensity on MRI studies mimicking that of limbic encephalitis.

 

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Case Report: 3 cases of patients with neuromyelitis optica (NMO) who are seronegative for the NMO-IgG and normal or non-diagnostic MRI. CSF studies, however, revealed presence of NMO-IgG in all three cases during NMO exacerbation.

 

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Case Report: Newborn child with multi-system disorder found to have mutations in the catalytical subunit of mitochondrial DNA polymerase gama gene (POLG-1).

 

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Neuroimaging: Nasal septal deviation with contact point is easily seen on MRI and might be associated with episodic or daily headache. However, many MRI with contact point were read as normal. The author described two such cases.

 

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Neuroimaging: MRI images and gross pathology picture of a 58 year old male with multiple, variable sized infarction due to the atrial-esophageal fistula developed after esophagastrorectomy and local radiation for esophageal carcinoma.

 

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 Neuroimaging: EEG and MRI study of a patient with seizure activity typically characterized by swinging movement of bilateral extremities and simultaneous vocalization was found to have seizure related hyperintensity in the R frontal primary motor cortex near the supplementary motor area (SMA).