Tinnitus and Hyperacusis
John C Watkinson, Raymond W Clarke, Christopher P Aldren, Doris-Eva Bamiou, Raymond W Clarke, Richard M Irving, Haytham Kubba, Shakeel R Saeed in Paediatrics, The Ear, Skull Base, 2018
Tinnitus may manifest itself as a train of rhythmical clicks or a buzzing or fluttering noise or sensation that is not synchronous with the pulse. These sounds are often related to myoclonic activity resulting in repetitive contractions of the middle ear muscles, but other muscles within the head and neck region can be affected. Currently there is no evidence to suggest that the middle ear is part of a systematic myoclonic disorder. Within the middle ear, contraction of the tensor tympani and/or stapedius muscle are often proposed to be the cause of either a clicking or buzzing noise respectively; however, whether the associated tinnitus is the direct result of muscle contraction noise, vibration of the tympanic membrane or movement of the ossicular chain remains unknown.203 The palatal muscles can also develop myoclonic contraction and this can produce sound that is audible to others. The clicking sound of palatal myoclonus is usually irregular with a frequency of one to two clicks per second. Involuntary palatal movements may be seen, either simply by inspecting the palate transorally or by visualizing the upper surface transnasally using a fibreoptic endoscope. Palatal myoclonus exists in two forms: symptomatic palatal myoclonus, which may be associated with lesions of the brainstem; and essential palatal myoclonus, which is usually idiopathic. It is generally essential palatal myoclonus that is associated with pulsatile tinnitus.
Dementia and lower urinary tract dysfunction
Jacques Corcos, David Ginsberg, Gilles Karsenty in Textbook of the Neurogenic Bladder, 2015
Dementia in Alzheimer’s disease is characterized by loss of memory, intellectual dysfunction, disturbances in speech such as anomia (difficulty in word finding), and various types of apraxia and agnosia. In practice, dementia can be indicated by test scores, such as 23 or lower of 30 points on the mini-mental state examination (MMSE). Emotional disturbances include depression in about 25% patients, with agitation and restlessness also being common. Motor signs are particularly rare early in the course of the illness. Typical Alzheimer’s disease patients have MMSE scores of less than 5 even though they can walk into the clinic without assistance. However, as the disease progresses, increased deep tendon reflexes and parkinsonian syndrome may develop. Myoclonus is occasionally reported. Decreased motivation and initiative are also significant features. In most advanced cases, abulia (loss of psychomotor activity) or apallic syndrome (akinetic mutism, vegetative state) occurs, making the patient totally dependent. As discussed earlier, prominent urinary disturbances do occur in Alzheimer’s disease but are very uncommon at an early stage.
Development of palliative medicine in the United Kingdom and Ireland
Eduardo Bruera, Irene Higginson, Charles F von Gunten, Tatsuya Morita in Textbook of Palliative Medicine and Supportive Care, 2015
Myoclonus is a sudden, shock-like involuntary movement or twitching caused by active muscular contractions, which may involve a whole muscle or may be limited to a small number of muscle fibers.  73 It has been postulated that generalized myoclonus is a type of tonic-clonic seizure.  74 Myoclonus is one of the more frequently observed features of OIN, and if present, there should be a high index of suspicion for the diagnosis. It has been described following administration of most commonly used opioids but is more common with opioids that have active metabolites such as morphine,  75,76 hydromorphone,  77,78 and meperidine.  79,80 However, myoclonus has also been described with fentanyl  74,75 and methadone. It has been proposed that myoclonus results from accumulation of neurotoxic opioid metabolites.  77 Myoclonus may occur more commonly in patients who have impaired renal functions.
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.
Nasal pregabalin overdose and myclonus: a new way of misuse
Published in Psychiatry and Clinical Psychopharmacology, 2019
H. Mihrimah Ozturk, Gülin Morkavuk
Myoclonus is involuntary, jerky, shock-like movement due to muscular contractions or sudden lapses of muscle contraction in active muscles. There are various causes of myoclonus and pathophysiological mechanisms associated with this symptom [4,5]. Metabolic and toxic etiologies such as renal/hepatic failure, hyponatremia, hypoglycemia, hyperthyroidism, head trauma can cause myoclonus. Also many antiparkinsonian, antidepressant, anxiolytics, antiepileptic, analgesic, anesthetic drugs may induce myoclonus [6]. Pregabalin was reported to increase spikewave activity in a dose-dependent way in an animal model study with rats. However, the mechanism of pregabalin-induced myoclonus remains unclear [7]. Herein, we present a case of a patient with myoclonus after high dose nasal pregabalin misuse.
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