Degenerative Diseases of the Nervous System
Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw in Hankey's Clinical Neurology, 2020
The rate of progression is variable with some patients progressing to dementia over a course of more than 10–15 years, and others within a few years. Many patients eventually display akinetic mutism in the final stages. Loss of language function to the point of muteness is uncharacteristic of AD and a distinguishing feature of FTD. Patients with isolated PPA may show isolated language impairment, without global development of dementia in as many as 50% of affected individuals. In those with concomitant MND, the time to death tends to be shorter, secondary to the more common sequelae of swallowing difficulty and aspiration pneumonia. Long-term prognosis for all cases is poor.
Non-Synonyms (Similar-Sounding)
Terence R. Anthoney in Neuroanatomy and the Neurologic Exam, 2017
Akinetic mutism-1* (A&V, p. 257–258, 333): An imprecise term, used to label various (but not all) persistent conditions in which a patient docs not speak nor move though apparently alert, intact sensorily, and capable of speech and movement.
Akinetic mutism and the story of David
Published in Neuropsychological Rehabilitation, 2018
Rebecca Sinden, Barbara A. Wilson, Anita Rose, Nimisha Mistry
Akinetic mutism (AM) is a rare syndrome characterised by reduced motor function including facial expressions, gestures and speech output but with some degree of alertness following damage to the frontal lobe and/or frontal subcortical circuits (Nagaratnam, Nagaratnam, Ng, & Diu, 2004). It is sometimes seen in patients who are recovering from a disorder of consciousness (DOC; Formisano et al., 2011). There are many causes of AM including infarction with frontal lobe or bilateral thalamus damage, hydrocephalus, anoxia, an anterior communicating artery aneurysm, traumatic brain injury, stroke, carbon monoxide poisoning or tumour (Herklots, Oldenbeuving, Beute, Roks, & Schoonman, 2016). The first documented case of AM was reported by Cairns, Oldfield, Pennybacker, and Whitteridge (1941). They described a 14-year-old girl who suffered from a cystic tumour of the third ventricle causing recurrent episodes of mutism and immobility. The most profound characteristics were: Mutism, loss of feeling tone, loss of emotional expression, of spontaneous and of most other voluntary movements, and total incontinence of urine and faeces … she was incapable of originating active manifestations of any kind, with the notable exception that ocular fixation and movement occurred in response to the movement of external objects and to sounds (Cairns et al., 1941, p. 279).
Rapidly progressive dementia–associated N-type voltage-gated calcium channel antibody encephalopathy
Published in Baylor University Medical Center Proceedings, 2020
Smathorn Thakolwiboon, Amputch Karukote, Gyeongmo Sohn, Walter R. Duarte-Celada, Parunyou Julayanont
A previously healthy 74-year-old, right-handed black woman presented with a 6-month history of progressive cognitive decline and behavioral changes. She had 12 years of education and was a retired farmer who had moved from Nigeria to the United States 5 years earlier. The patient lived independently without cognitive difficulty prior to the illness. Her family reported that she started having bizarre visual hallucinations and persecutory delusions 6 months prior to this presentation. Three months after the onset of psychosis, she developed progressive cognitive impairments characterized by short-term memory problem and inattention. She was brought to the emergency department by a police officer after she was found wandering around a street. She had no history of psychiatric illness or substance abuse and no known family history of dementia. Neurological examination showed no focal neurological deficits. There were no abnormal movements, extrapyramidal dysfunction, akinetic mutism, or visual impairment on the exam. During the bedside examination, she was verbally aggressive. She scored 16/30 on the Mini-Mental State Examination (Table 1). Apart from her low body mass index (17 kg/m2), her general physical examination was normal.
The Last Refuge of Privacy
Published in AJOB Neuroscience, 2019
Melissa D. McCradden, James A. Anderson
Consider patients presenting with catatonic symptoms. Catatonia is an eminently treatable condition but with a differential diagnosis comprising many clinically overlapping conditions, and with significant consequences should treatment proceed based on an incorrect diagnosis. For example, neuroleptic malignant syndrome (NMS) and antipsychotic-induced parkinsonism can be difficult to distinguish from catatonia, which is particularly problematic since pharmacologic treatment for catatonia (benzodiazepines) can greatly worsen parkinsonian symptoms and even provoke death in the case of NMS (Rasmussen, Mazurek, and Rosebush 2016). Disorders such as nonconvulsive status epilepticus, abulia and akinetic mutism, locked-in syndrome, and stiff person syndrome may also present similarly to catatonia, yet the discriminatory test—the electroencephalogram—can be unreliable, and neuroimaging is time- and labor-intensive. In all these conditions, complete neurological and physical examination is very difficult due to the nature of the patient’s condition. Catatonic patients respond dramatically to lorazepam, and earlier treatment produces much better results compared with when treatment is delayed (Rasmussen, Mazurek, and Rosebush 2016).