Encephalopathy With Akinetic Mutism in a Child With COVID-19 Infection: A Case Report.

INTRODUCTION: COVID-19 has been associated with neurological complications, including encephalopathy and akinetic mutism. CASE PRESENTATION: A 7-year-old unvaccinated boy presented with visual hallucinations, urinary incontinence, and akinetic mutism 13 days after he was exposed to COVID-19. He had minimal respiratory symptoms, including just 1 day of fever and cough. Evaluations showed slowing on electroencephalogram, normal cerebrospinal fluid, normal brain magnetic resonance imaging, and mild sinus bradycardia. He recovered rapidly to baseline after 5 days of intravenous methylprednisolone. DISCUSSION: COVID-19-related encephalopathy including akinetic mutism is usually found in older adult patients with more severe COVID-19 illness. Our case demonstrates that akinetic mutism can present in children with mild COVID-19 illness and that it can respond rapidly and completely to intravenous methylprednisolone. CONCLUSIONS: COVID-19-related encephalopathy may be immune mediated. A heightened awareness of its association with COVID-19 illness should lead to earlier diagnosis and consideration of immunomodulatory therapy.

Full recovery of Akinetic Mutism after corpus callosum infarction in post subarachnoid hemorrhage of ruptured distal anterior cerebral artery aneurysm.

The authors report a previously healthy 23-year-old male patient who presented with subarachnoid hemorrhage and was found to have a ruptured right distal anterior cerebral artery aneurysm. He was treated by endovascular coiling technique, which was uneventful perioperatively. After a few days of mechanical ventilation and upon extubation, he expressed symptoms of apathy, drowsiness, lack of motivation, and lack of spontaneous motor function consistent with akinetic mutism. The magnetic resonance imaging of the brain revealed infarction of the whole body of corpus callosum. He remained in akinetic mutism status for twenty-one days before he started to show improvement until he fully recovered in 3 months. The authors report a unique finding where akinetic mutism resulted from infarction of the corpus callosum rather than medial frontal lobe (cingulate gyrus).

Doença de Creutzfeldt-Jakob: relato de caso / Creutzfeldt-Jakob disease: case report

As doenças priônicas fazem parte do grupo das síndromes de demência rapidamente progressiva com neurodegeneração. Em humanos, a doença de Creutzfeldt-Jakob é a mais prevalente. Atualmente, seu diagnóstico pode ser baseado em uma combinação do quadro clínico, ressonância magnética e eletroencefalograma com alterações típicas, juntamente da detecção de proteína 14- 3-3 no líquido cefalorraquidiano. Este relato descreve o caso de uma paciente de 74 anos, natural de Ubá (MG), admitida em um hospital da mesma cidade com quadro de demência de rápida progressão, com declínio cognitivo, ataxia cerebelar e mioclonias. No contexto clínico, aventou-se a possibilidade de doença de Creutzfeldt-Jakob e, então, foi iniciada investigação para tal, com base nos critérios diagnósticos. Também foram realizados exames para descartar a possibilidade de doenças com sintomas semelhantes. O caso foi diagnosticado como forma esporádica de doença de Creutzfeldt-Jakob. (AU)

Prion diseases are part of the rapidly progressive dementia syndromes with neurodegeneration. In humans, Creutzfeldt-Jakob disease is the most prevalent. Currently, its diagnosis may be based on a combination of clinical picture, magnetic resonance imaging, and electroencephalogram with typical changes, along with the detection of 14-3-3 protein in cerebrospinal fluid. This report describes the case of a 74-year-old woman from the city of Ubá, in the state of Minas Gerais, who was admitted to a hospital in the same city with a rapidly progressive dementia, cognitive decline, cerebellar ataxia and myoclonus. In the clinical context, the possibility of Creutzfeldt-Jakob disease was raised, and then investigation was started for this disease, based on the its diagnostic criteria. Tests have also been conducted to rule out the possibility of diseases with similar symptoms. The case was diagnosed as a sporadic form of Creutzfeldt-Jakob disease. (AU)

Akinetic mutism following prefrontal injury by an electrical grinder a case report: A diffusion tensor tractography study.

RATIONALE: A 72-year-old male had suffered from head trauma resulting from injury to his frontal area by an electrical grinder while working at his home. PATIENT CONCERNS: He lost consciousness for approximately 10 minutes and experienced continuous post-traumatic amnesia. DIAGNOSES: He was diagnosed as traumatic intracerebral hemorrhage in both frontal lobes, intraventricular hemorrhage, and subarachnoid hemorrhage, and underwent decompressive craniectomy and hematoma removal. INTERVENTIONS: The patient's Glasgow Coma Scale score was 5. At 2 months after onset, when starting rehabilitation, he showed no spontaneous movement or speech; he remained in a lying position all day with no spontaneous activity. OUTCOMES: On 2-month diffusion tensor tractography, decreased neural connectivity of the caudate nucleus to the medial prefrontal cortex (PFC, Broadmann area [BA]: 10 and 12) and orbitofrontal cortex (BA 11 and 13) was observed in both hemispheres. LESSONS: Akinetic mutism following prefrontal injury.

Akinetic mutism and the story of David.

Following a description about the characteristics of akinetic mutism (AM) and how it differs from locked-in syndrome (LIS) and a disorder of consciousness (DOC), we present the case of David, a 71-year-old man with AM. David sustained a stroke following a middle cerebral artery (MCA) thrombus. A CT scan at the time detected old ischaemic infarcts affecting the right frontal lobe, the left occipital lobe and the left parietal lobe so David had bilateral brain damage. Initially thought to have a DOC, further neuropsychological assessments administered when David had improved a little, resulted in the diagnosis of AM. Although David spoke little, when he did speak, his words and phrases were well articulated, grammatical and with appropriate intonation. He was alert and visually aware and he was not paralysed. We discuss whether the diagnosis was correct and address the difficulties in assessing such patients.

Serotonin Syndrome Following Combined Administration of Dopaminergic and Noradrenergic Agents in a Patient With Akinetic Mutism After Frontal Intracerebral Hemorrhage: A Case Report.

BACKGROUND: Serotonin syndrome (SS) is a potentially life-threatening condition that can be caused by use of proserotonergic drugs. Several studies have reported that combined administration of various medications may induce SS. We report a case of SS in a patient who was being treated with dopaminergic and noradrenergic drugs. CASE PRESENTATION: A 55-year-old man with a right frontal intracerebral hemorrhage extending to the left cerebral hemisphere presented with clinical features of akinetic mutism. Three months after onset, dopaminergic (methylphenidate, levodopa/benserazide) and noradrenergic (atomoxetine) drugs were administered to enhance his cognitive function. His cognitive function gradually improved during 8 weeks of dose escalation. One day after the dose of atomoxetine was increased from 40 mg/d to 60 mg/d, the patient developed inducible clonus, rigidity, diarrhea, tachycardia, and hyperthermia, in keeping with a diagnosis of SS. The symptoms and signs suggestive of SS resolved on the day following cessation of all dopaminergic and noradrenergic drugs. CONCLUSIONS: This case demonstrates that medications generally known as dopaminergic or noradrenergic agents could have serotonergic effects via a mechanism that is yet to be fully elucidated. The clinical manifestations of SS can be diverse, ranging from mild to severe and potentially fatal symptoms. When administering a combination of catecholaminergic agents, clinicians should carefully monitor the patient's neurologic status for unexpected adverse reactions.

Gastrostomy in patients with prion disease.

Patients with prion diseases can live for long periods of time in a state of akinetic mutism given appropriate management of their symptoms. To study symptom support in these cases, we performed gastrostomies on 3 patients with V180I genetic Creutzfeldt-Jakob disease (CJD) who had become akinetic and mute, and compared them to 14 other similar patients being fed by tube. In the 3 gastrostomy cases, there were no direct complications due to the gastrostomy or tube feeding, nor were there episodes of discontinuation of tube feeding or initiation of continuous drip infusion due to severe complications. Antibiotics were administered for mild infections, a complication of CJD, with 0.2% and 8.8% of the total time after gastrostomy being used for intravenous or transluminal administration, respectively. We compared the present patient series with that of our previous report statistically, and found that patients undergoing gastrostomy required significantly fewer discontinuations of tube feeding than those who did not. No significant difference in antibiotic administration was found between groups, however. It is our conclusion that gastrostomy should be allowed for symptom support in akinetic patients with prion disease, but adequate informed consent must be provided to the patient's family.

Akinetic mutism in a patient with mild traumatic brain injury: A diffusion tensor tractography study.

OBJECTIVES: Akinetic mutism (AM) is characterized by a complete absence of spontaneous behaviour and speech. We report on a patient with AM associated with injury of the prefronto-caudate tract and prefronto-thalamic tract following mild traumatic brain injury (TBI), diffusion tensor tractography (DTT). CASE PRESENTATION: A 20-year-old man suffered from TBI resulting from a pedestrian car accident. Following the TBI, he developed abulia (decreased activity and speech) that worsened over approximately a year. His typical features of AM that remained stable from one year until two years after the TBI are: he showed no spontaneous movement or speech and remained recumbent with no spontaneous activity. RESULTS: On one-month DTT, the neural connectivity of the caudate nucleus to the medial prefrontal cortex was low in both hemispheres, and this neural connectivity was lower on two-year DTT. The orbitofrontal-thalamic tract was thin in the left hemisphere on one-month DTT, whereas this tract became thinner in both hemispheres on two-year DTT. CONCLUSIONS: Using serial DTTs, injuries of the prefronto-caudate tract and orbitofrontal-thalamic tract and degeneration of these injured neural tracts concurrent with aggravation of abulia to AM were demonstrated in a patient with mild TBI. ABBREVIATIONS AM akinetic mutism; BA Brodmann areas; CN caudate nucleus; CST corticospinal tract; CRT corticoreticulospinal tract; DTT diffusion tensor tractography; FAC Functional Ambulation Category; PFC prefrontal cortex; MMSE Mini-Mental State Examination; ROI region of interest; TBI traumatic brain injury.

Akinetic mutism revealing an ischemic stroke.

We describe a 44-year-old man who developed akinetic mutism following a cerebrovascular accident involving his left middle cerebral artery. We discuss this rare condition and its unusual clinical picture.

Recovery of akinetic mutism and injured prefronto-caudate tract following shunt operation for hydrocephalus and rehabilitation: A case report.

RATIONALE: A 76-year-old female patient was diagnosed with an aneurysmal subarachnoid hemorrhage following rupture of a right posterior communicating artery aneurysm. PATIENT CONCERNS: She was treated surgically with clipping of the aneurysmal neck. Six months after onset, when starting rehabilitation at our hospital, she showed no spontaneous movement or speech. DIAGNOSES:: aneurysmal subarachnoid hemorrhage following rupture of a right posterior communicating artery aneurysm. INTERVENTIONS: During 2 months' rehabilitation, her AM did not improve significantly. As there was no apparent change, she underwent a ventriculo-peritoneal shunt operation for hydrocephalus 8 months after her stroke. After the surgery, she remained in the AM state, but participated in a comprehensive rehabilitative management program similar to that before shunt operation. During 1 month's intensive rehabilitation, her AM gradually improved. At 9 months after onset, she became able to perform some daily activities by herself including eating, washing, and dressing. In addition, she could speak with some fluency. OUTCOMES: On 6-month DTT, the neural connectivity of the caudate nucleus (CN) to the medial prefrontal cortex (PFC, Broadmann area [BA]: 10 and 12) and orbito-frontal cortex (BA 11 and 13) was low in both hemispheres. However, the neural connectivity of the CN to the medial PFC increased on both sides on 9-month DTT. The integrity of the arcuate fasciculus (AF) was preserved in both hemispheres on both 6- and 9-month DTTs. LESSONS: Recovery of AM and injured PCTs was observed in a stroke patient.

Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt-Jakob disease.

BACKGROUND AND PURPOSE: Akinetic mutism is thought to be an appropriate therapeutic end-point in patients with sporadic Creutzfeldt-Jakob disease (sCJD). However, prognostic factors for akinetic mutism are unclear and clinical signs or symptoms that precede this condition have not been defined. The goal of this study was to identify prognostic factors for akinetic mutism and to clarify the order of clinical sign and symptom development prior to its onset. METHODS: The cumulative incidence of akinetic mutism and other clinical signs and symptoms was estimated based on Japanese CJD surveillance data (455 cases) collected from 2003 to 2008. A proportional hazards model was used to identify prognostic factors for the time to onset of akinetic mutism and other clinical signs and symptoms. RESULTS: Periodic synchronous discharges on electroencephalography were present in the majority of cases (93.5%). The presence of psychiatric symptoms or cerebellar disturbance at sCJD diagnosis was associated with the development of akinetic mutism [hazard ratio (HR) 1.50, 95% confidence interval (CI) 1.14-1.99, and HR 2.15, 95% CI1.61-2.87, respectively]. The clinical course from cerebellar disturbance to myoclonus or akinetic mutism was classified into three types: (i) direct path, (ii) path via pyramidal or extrapyramidal dysfunction and (iii) path via psychiatric symptoms or visual disturbance. CONCLUSIONS: The presence of psychiatric symptoms or cerebellar disturbance increased the risk of akinetic mutism of sCJD cases with probable MM/MV subtypes. Also, there appear to be sequential associations in the development of certain clinical signs and symptoms of this disease.

Reversible Changes of Brain Perfusion SPECT for Carbon Monoxide Poisoning-Induced Severe Akinetic Mutism.

PURPOSE: This study aimed to characterize changes in regional cerebral blood flow (rCBF) in patients who experienced carbon monoxide (CO) poisoning and subsequently developed severe delayed neuropsychiatric sequelae (DNS) with akinetic mutism. We determined whether these changes were reversible in parallel with improvements in neuropsychological function in response to treatment, including hyperbaric oxygen therapy. METHODS: Patients who developed severe DNS with akinetic mutism after acute CO intoxication between 2007 and 2011 were enrolled. Tc-ECD brain SPECT findings were compared between the patients with severe akinetic mutism and age-matched control subjects to characterize the pattern of rCBF. Perfusion SPECT was correlated with clinical outcomes after treatment with statistical parametric mapping (SPM8); the height threshold was P < 0.01 at peak level, and the corrected false discovery rate was P < 0.05 at the cluster level. RESULTS: Seven patients with akinetic mutism were analyzed. All patients had neurological symptoms caused by acute CO exposure, and all recovered to nearly normal daily function after initial treatments. In all cases, after a "lucid interval," DNS progressed to akinetic mutism. The SPECT images acquired at the onset of akinetic mutism demonstrated variable hypoperfusion in frontal-temporal-parietal regions, with the greatest severity in the left temporal-parietal regions. In parallel, we performed functional neuropsychiatric tests. After treatment, the brain SPECT showed significantly fewer hypoperfusion regions, and neuropsychiatric tests showed dramatically improved function. CONCLUSIONS: Our findings demonstrated both cerebral cortical and subcortical injuries in patients with CO-induced akinetic mutism. Improvement in rCBF correlated well with functional recovery after treatment.

Mumps encephalitis with akinesia and mutism.

Measles-rubella-mumps vaccination is routine in many countries, but the mumps vaccine remains voluntary and is not covered by insurance in Japan. A 5-year-old Japanese boy who had not received the mumps vaccine was affected by mumps parotitis. Several days later, he presented with various neurological abnormalities, including akinesia, mutism, dysphagia, and uncontrolled respiratory disorder. Mumps encephalitis was diagnosed. Despite steroid pulse and immunoglobulin treatment, the disease progressed. Magnetic resonance imaging showed necrotic changes in bilateral basal ganglia, midbrain, and hypothalamus. At 1 year follow up, he was bedridden and required enteral feeding through a gastric fistula and tracheostomy. Mumps vaccination should be made routine as soon as possible in Japan, because mumps encephalitis carries the risk of severe sequelae.