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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
CJD is a spongiform encephalopathy which causes abnormal signal in the thalamus, basal ganglia (particularly the caudate and putamen) and cortex with T2 hyperintensity and restricted diffusion. The condition causes myoclonus and dementia. The sporadic type is most common and tends to affect older age groups. CT findings are frequently of atrophy.
Dementia syndromes
Published in Bhaskar Punukollu, Michael Phelan, Anish Unadkat, MRCPsych Part 1 In a Box, 2019
Bhaskar Punukollu, Michael Phelan, Anish Unadkat
Characterized by memory changes, confusion and disorientation initially. As the disease advances, a slowly worsening dementia develops. Cerebellar ataxia and choreoathetoid movements may occur. Myoclonus occurs in around 80% of cases. Extrapyramidal signs, dysarthria, akinetic mutism and cortical blindness may develop.
The nervous system
Published in Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella, Essentials of Human Physiology and Pathophysiology for Pharmacy and Allied Health, 2019
Laurie K. McCorry, Martin M. Zdanowicz, Cynthia Y. Gonnella
MyoclonicPresents as sudden, brief twitches or jerks of arm and leg muscles. May be generalized in terms of presentation or limited to muscles of face, extremities or trunk
Opsoclonus Myoclonus Ataxia Syndrome Due to SARS-CoV-2
Published in Neuro-Ophthalmology, 2023
Josef Finsterer, Fulvio A. Scorza
Altogether, 29 articles reporting 45 patients with SARS-CoV-2 associated OMS/OMAS were retrieved (Table 1). Their ages ranged from 2 to 88 years (Table 1). Three patients were paediatric patients and the remainder adults. Gender was male in 32 patients and female in 13 patients. Opsoclonus alone or with other features was described in 30 patients. Myoclonus alone or together with other features was described in 43 patients. Opsoclonus was associated with myoclonus in 28 cases. Eighteen patients presented with opsoclonus, myoclonus, and ataxia; 11 patients with myoclonus and ataxia; ten patients with opsoclonus and myoclonus; and one patient with opsoclonus and ataxia (Table 1). Isolated myoclonus was described in four cases (Table 1), while isolated opsoclonus was reported in a single patient (Table 1). In 11 patients the OMS/OMAS was associated with cognitive impairment. One patient was extensively tested by means of the Wechsler Intelligence Scale for Children – Fourth Edition (WISC-IV) test, showing an inhomogeneous cognitive profile (verbal comprehension index 95, perceptional reasoning 56, working memory index 70, processing speed index: 47) resulting in an intelligence quotient of 58 (Table 1).
Position-dependent arm dyskinesia due to severe craniocervical malformation
Published in The Journal of Spinal Cord Medicine, 2022
Francisco de Assis Aquino Gondim, José Arnaldo Mota Arruda, David Nunes de Lima Junior, Florian P. Thomas
Myoclonus is a movement disorder characterized by brief, abrupt and involuntary contractions of a single muscle or groups of muscles.6 Spinal myoclonus can be divided into segmental and propriospinal myoclonus.7 Some phenomenological aspects observed in our patient resemble propriospinal spinal myoclonus (patient 6 in the series of Termsarasab et al.).1 Symptoms only occurred with neck flexion at specific angles, making a psychogenic causation unlikely and reinforcing the importance of brainstem and/or spinal cord traction, possibly with ephaptic activation of motor pathways.8 Similar malformations may lead to episodes of tetraparesis and/or apnea.9 The left arm dyskinetic episodes continued until her death, which may have resulted from sudden and more severe brainstem compression. Our findings also resemble paroxysmal dyskinesia- or dystonia-like movements in patients with MS “precipitated by sudden or voluntary movements and by mere intention to move”.3 While we did not rule out a varicella-induced disorder, the clinical course is not consistent with this condition.2 Other causes of spinal-generated myoclonus/movement are autoimmune (usually paraneoplastic)10 and tumors.11 In our patient there was no evidence for either of these conditions and craniocervical malformation was an obvious cause.
Diagnostic and therapeutic approach to drug-resistant juvenile myoclonic epilepsy
Published in Expert Review of Neurotherapeutics, 2021
Michele Ascoli, Giovanni Mastroianni, Sara Gasparini, Pasquale Striano, Vittoria Cianci, Sabrina Neri, Valentina Bova, Anna Mammì, Antonio Gambardella, Angelo Labate, Umberto Aguglia, Edoardo Ferlazzo
JME at onset must be carefully differentiated from Progressive Myoclonus Epilepsies (PMEs). PMEs are a heterogeneous group of epilepsies characterized by the occurrence of action myoclonus associated with variable degrees of cognitive deterioration, ataxia, and other different clinical features [24]. Consanguinity between parents, presence of positive family history for myoclonus or epileptic seizures, neurological signs or symptoms, such as action or stimulus-sensitive myoclonus, dysarthria, ataxia, impaired walking, instability upon standing up, extrapyramidal signs, should prompt the physician to specific work-up aimed to exclude PMEs. Myoclonus must be carefully researched during the neurological examination: in rest condition, during action execution, and in response to stimuli including light, noise, and mental stress. The evaluation of rest, action, and stimulus-induced myoclonus is conducted through different tasks. To better highlight the presence of action myoclonus, the patient may be encouraged to perform the following maneuvers: 1) write a sentence; 2) to complete the Archimedes spirals by connecting the dots with the right and then the left hand; 3) to pour an eight-ounce glass of water into an empty eight-ounce glass with his dominant; 4) to use a soupspoon to bring water from a cup to his mouth with his dominant hand.