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Diseases of the Nervous System
Published in George Feuer, Felix A. de la Iglesia, Molecular Biochemistry of Human Disease, 2020
George Feuer, Felix A. de la Iglesia
Methyl bromide is a colorless gas at room temperature and has been used as insecticide fumigant, refrigerant, and in fire extinguishers. Acute exposure to methyl bromide causes visual and speech disturbances, convulsions, and delirium. Chronic toxicity is associated with peripheral neuropathy, as well as cerebellar and pyramidal dysfunctions. In chronic conditions, the first signs are paresthesia of the limbs and deep reflexes which become sluggish or absent. Markedly altered gait and staggering are also found. Mental status and pyramidal signs remain normal. The recovery from these abnormalities is slow, sometimes between 2 to 8 months after onset of symptoms. Methyl bromide is toxic to the lungs and kidney also.
Diagnosis and differential diagnosis of Parkinson’s disease
Published in Jeremy Playfer, John Hindle, Andrew Lees, Parkinson's Disease in the Older Patient, 2018
Extra pyramidal signs (EPS) are common in elderly persons: EPS of variable severity were reported in 15% of community-based subjects who were 65 to 74 years old and in 52% of those over 85 years of age.4 Clinically evident parkinsonism (two or more of the cardinal motor signs) in a similar population is lower, at around 3%.5 This suggests a significant reservoir of subtle signs of parkinsonism in elderly subjects, who may or may not go on to develop IPD or another disorder. Parkinsonian signs may occur in association with mild cognitive impairment without evidence of overt neuro logical disorder6 and may predict incident dementia.7 Parkinsonism occurring in the context of dementia becomes increasingly common in the ninth decade.8
Practice Paper 10: Answers
Published in Anthony B. Starr, Hiruni Jayasena, David Capewell, Saran Shantikumar, Get ahead! Medicine, 2016
Anthony B. Starr, Hiruni Jayasena, David Capewell
Punch-drunk syndrome (also known as post-traumatic dementia or boxing encephalopathy) occurs after repeated blows to the head. Brain atrophy is a feature. Clinical features are cognitive impairment and personality deterioration with cerebellar, extrapyramidal and pyramidal signs. Occasionally, pathological jealousy and rage reactions are associated. Pellagra is a disorder caused by niacin (vitamin B3) deficiency. It is most common in South America, where maize forms the staple diet. The main physical features are weakness, dermatitis and diarrhoea. There can also be personality changes, progressing to dementia. Treatment is with niacin. Death can occur within a few years if the condition is untreated.
Atypical and early symptoms of sporadic Creutzfeldt – Jakob disease: case series and review of the literature
Published in International Journal of Neuroscience, 2021
Grammatiki Katsikaki, Ioannis E. Dagklis, Petros Angelopoulos, Dimitrios Ntantos, Angeliki Prevezianou, Sevasti Bostantjopoulou
The last case was a 57-year-old man who had been experiencing behavior and memory problems for a period of three-months, as well as confusional episodes. Later on, he showed rapid deterioration of cognitive function and gait instability, the MRI of the brain revealed chronic ischemic type lesions on the right side of the pons and two angiomas of the left frontal lobe, whereas the EEG was indicative of focal epileptic disorder which evolved on frequency and topography and eventually showed generalised distribution (Figure 2). He was initially treated as non-convulsive status epilepticus (NCSE) with aggressive antiepileptic medication, showing a consequent improvement in the EEG but without any clinical improvement. During the course of the disease the EEG changed into an encephalopathic pattern of sCJD. The disease deteriorated sharply with pyramidal signs, convulsions and ultimately mutism and death within 4 months of the onset of symptoms. In sequential MRI, there was a pathogenic restriction of diffusion in the frontal and parietal cortex, insula, as well as in the head of the caudate nucleus and the anterior part of the putamen on the left (Figure 1E,F). The pathological PrPsc was detected with real-time quaking-induced conversion (RT-QuIC) in the CSF.
Investigating TBP CAG/CAA trinucleotide repeat expansions in a Taiwanese cohort with ALS
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2021
Kang-Yang Jih, Kon-Ping Lin, Pei-Chien Tsai, Bing-Wen Soong, Yi-Chu Liao, Yi-Chung Lee
SCA17 is a dominantly inherited neurodegenerative disease, characterized by ataxia (95%), dementia (∼90%), and involuntary movements (∼70%) such as chorea and dystonia (5–7). Psychiatric symptoms, pyramidal signs, and rigidity are also common manifestations (8,9). SCA17 is caused by an abnormal expansion of the CAG/CAA trinucleotide repeats within TBP. It is categorized as a polyglutamine disease since both CAG and CAA encode glutamine and the expanded trinucleotide repeats will result in a mutant TBP protein with a prolonged polyglutamine expansion. The normal trinucleotide repeat length in TBP ranges from 25 to 40. Patients carrying the TBP mutant allele with a CAG/CAA repeat size greater than 49 would develop SCA17 with complete penetrance, whereas those with intermediate-length CAG/CAA repeats (41 to 48) are at risk of SCA17 with a reduced penetrance (10). Only few SCA17 patients with 41 − 43 CAG/CAA repeats have been reported (11–13), and the majority of SCA17 cases carry TBP alleles with 44 or more CAG/CAA repeats (6,7).
Superficial siderosis of central nervous system with unknown cause: report of 2 cases and review of the literature
Published in British Journal of Neurosurgery, 2019
Hao Chen, Hafiz Khuram Raza, Jia Jing, Xinchun Ye, Zuohui Zhang, Fang Hua, Guiyun Cui
SS-CNS is a rare disease of CNS which is defined as linear residues of blood degradation products specially hemosiderin in the superficial layers of the brain, spinal cord and central parts of cranial nerves. The most common causes of SS-CNS are aneurysm, trauma, tumor, and arteriovenous malformation. Although in most cases, there is no obvious source of bleeding. Its most common presenting features include sensorineural hearing loss, cerebellar ataxia, and pyramidal signs. It’s largely considered as a rare disease though the numbers of reported cases are increasing with the advancing MRI technology.1 It is being more frequently diagnosed in patients with sensorineural hearing loss.2 SS-CNS tend to be under-diagnosed in the absence of obvious cause of bleeding,