Akinetic mutism and gait disturbance in a patient with delayed post-hypoxic leukoencephalopathy.

We report on a patient with delayed post-hypoxic leukoencephalopathy (DPHL) who showed akinetic mutism and gait disturbance, neural injuries that were demonstrated on diffusion tensor tractography (DTT). A patient was exposed to carbon monoxide (CO) and rapidly recovered; however, two weeks after onset, he began to show cognitive impairment and gait disturbance. At six weeks after CO exposure, he showed akinetic mutism and gait inability. DTT at 6-weeks post-exposure showed discontinuations in neural connectivities of the caudate nucleus to the medial prefrontal and orbitofrontal cortex in both hemispheres. In addition, the corticoreticulospinal tract revealed severe thinning in both hemispheres.

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).

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.

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.

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.

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.

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.

[Akinetic mutism due to fulminant multilocular cerebral venous thrombosis, including the vein of Galen]. / Akinetischer Mutismus bei fulminanter multilokulärer Sinus- und Venenthrombose einschließlich der Vena Galeni.

Cerebral venous thrombosis may present with multifaceted symptoms and therefore be difficult to diagnose. Only few evidence-based data exist with respect to therapy and prognosis, especially concerning the deep cerebral venous system. A thrombosis of the vein of Galen is deemed to have a poorer prognosis. Our case report describes the local combined neuro-interventional therapy as an individual attempt to cure a patient with a fulminant disease course.

Akinetic mutism without a structural prefrontal lesion.

Akinetic mutism is characterized by profound apathy and a lack of verbal and motor output for action, despite preserved alertness. The condition usually follows bilateral damage to the medial frontal subcortical circuits. We report a 59-year-old right-handed woman who was admitted to the neurology ward with sudden-onset akinetic mutism. Her medical history included an ischemic stroke 3 years earlier, with residual anomia and mild agraphia but no motor dysfunction. On this admission, a cranial computed tomography scan disclosed an acute left superior cerebellar infarction embracing the vermis, and a prior left inferior parietal infarct. Electroencephalogram showed bilateral frontal delta-wave activity. Four weeks later, we performed a technetium-99m hexamethylpropyleneamine oxime single-photon emission computed tomography (Tc-HMPAO SPECT) scan to study the patient's frontal lobe function. The SPECT scan revealed the causative bifrontal hypoperfusion, more prominent on the right, while the structurally evident cerebellar infarction was predictably masked by subacute hyperperfusion phenomenon. Contralateral frontal diaschisis is an established sequela of cerebellar infarction. Because this patient also had lesions in the left parietal region, her left prefrontal area was critically deprived of its major reciprocally connected cortical counterparts (right prefrontal and left parietal), and also became dysfunctional. Her resulting bilateral frontal dysfunction is a common cause of akinetic mutism.

Delayed post-hypoxic leucoencephalopathy presenting akinetic mutism after traumatic cervical cord injury: a case report.

Delayed post-hypoxic leucoencephalopathy is a demyelinating syndrome characterized by acute onset of neuropsychiatric symptoms including parkinsonism or akinetic mutism that occurs days to weeks after recovery from cerebral hypo-oxygenation. We encountered a patient who presented with an akinetic mute state late after cervical cord injury without a definite hypoxic event.

[A case of intraventricular meningioma manifesting akinetic mutism after complicated management of hydrocephalus].

A 26-year-old male with large intraventricular meningioma developed acute hydrocephalus postoperatively, which was difficult to manage. After a repeated course of dilated and slit-like ventricle, he gradually presented a disturbance of consciousness, which was recovered by the use of bromocriptine, suggesting akinetic mutism. At the chronic stage, he suffered from symptoms of hydrocephalus again. Time-slip MRI revealed the stasis of CSF flow around the third ventricle. Neuroendoscopy disclosed that the foramen of Monro was occluded with membrane. After endoscopic third ventriculostomy as well as penetration of the foramen, the symptoms disappeared and hydrocephalus was resolved. This is a rare case of akinetic mutism after complicated management of hydrocephalus associated with intraventricular meningioma.

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.

Survival to akinetic mutism state in Japanese cases of MM1-type sporadic Creutzfeldt-Jakob disease is similar to Caucasians.

BACKGROUND: It is not known whether the clinical course of Japanese sporadic Creutzfeldt-Jakob disease (sCJD) cases differs from that of Caucasian sCJD cases. PATIENTS AND METHODS: To investigate the clinical course of Japanese sCJD, clinical findings from 29 patients with Japanese MM1-type sCJD were retrospectively evaluated and compared to Caucasian sCJD findings. RESULTS: Survival of Japanese MM1-type sCJD up to the time of akinetic mutism state is similar to that of Caucasian subjects. However, the total disease duration of Japanese patients was approximately three times longer. CONCLUSIONS: The present observations indicate that Japanese sCJD cases generally show a longer disease duration because of the longer survival period after reaching the akinetic mutism state.

Akinetic mutism--not coma.

We describe a case of akinetic mutism mistaken as coma. A 77-year old lady presented with apparent unresponsiveness. In fact she responded when stimulated. She subsequently developed movements typical of clonic perseveration, erroneously treated as seizures. She also had features of a frontal lobe syndrome. Initial CT scan showed no abnormality; it was only after an MRI scan that a diagnosis of bilateral paramedian thalamic infarction was made. MRI scanning should be considered early in the investigation of patients with atypical neurological presentation.

Inflammatory cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease.

BACKGROUND: Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile. CASES: We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis. CONCLUSION: Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.

Ocular dipping in Creutzfeldt-Jakob disease.

Ocular dipping refers to a slow downward deviation of both eyes followed by a quick return to the midposition after a brief delay. Two patients with rapid neurologic deterioration in Creutzfeldt-Jakob disease (CJD) displayed ocular dipping, which quickly evolved into sustained downgaze deviation. Ocular dipping may thus be a transitional sign in a vertical gaze disturbance.

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.