Akinetic mutism and status epilepticus due to Epstein Barr virus encephalitis.

Neurological complications of Epstein Barr virus (EBV) infection are infrequent and may include occasionally encephalitis, usually with a benign evolution. We here report on an aggressive case of EBV encephalitis in a 14-year-old boy with extensive basal ganglia involvement, and to a lesser degree of brain cortex who presented atypically with akinetic mutism and non-convulsive status epilepticus, requiring intensive care but showed a favorable outcome. EBV encephalitis is uncommon and its best management is unclear. Its pathophysiology is not well understood but could include autoimmunity. Onconeuronal and synaptic antibodies were negative in serum and cerebrospinal fluid, including the dopamine D2 receptor. To the best of our knowledge, this is the first report to evaluate antibodies to D2 receptors in EBV encephalitis. Corticosteroid therapy is usually recommended but the use of acyclovir is controversial. Intensive care is required in severe cases to assure a favorable outcome.

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.

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.

[Bromocriptine: could it be the cure for post-surgical akinetic mutism?] / Bromocriptina: podria ser la cura para el mutismo acinetico posquirurgico?

INTRODUCTION: Akinetic mutism is considered as an alteration of the motivational state of the person, which the patient is unable to initiate verbal or motor responses voluntary, even with preserved sensorimotor and surveillance functions. CASE REPORT: A 43 year-old male involved in a cerebellum arteriovenous fistula complicated with hydrocephalus, who responded dramatically to treatment with bromocriptine. CONCLUSION: Typically, akinetic mutism is described as a transient surgeries posterior fossa. However, it can also occur after multiple valvular failure in patients with hydrocephalus.

TITLE: Bromocriptina: podria ser la cura para el mutismo acinetico posquirurgico?Introduccion. El mutismo acinetico se considera una alteracion del estado motivacional de la persona, por el cual el paciente es incapaz de iniciar respuestas verbales o motoras de caracter voluntario, aun teniendo preservadas las funciones sensomotoras y de vigilancia. Caso clinico. Varon de 43 años, intervenido de una fistula arteriovenosa del cerebelo complicada con hidrocefalia, que respondio espectacularmente al tratamiento con bromocriptina. Conclusion. Tipicamente se ha descrito el mutismo acinetico como una complicacion transitoria de las cirugias de la fosa posterior. Sin embargo, tambien puede aparecer tras multiples fallos valvulares en pacientes con hidrocefalia.

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.

Serial diffusion-weighted MRI and SPECT findings in a Creutzfeldt-Jakob disease patient with V180I mutation.

We report serial changes of diffusion-weighted imaging (DWI) and single photon emission computed tomography (SPECT) in a patient with Creutzfeldt-Jakob disease with V180I mutation (CJD180). DWI abnormalities in our patient were more predominantly observed in the left cerebral cortex than left basal ganglia. Hemilateral abnormalities progressed over 5 months to involve the contralateral side with increasing DWI signals. At 6 months, SPECT showed hypoperfusion in the left parietal and frontal lobes and the hypoperfusion region spread to the bilateral basal ganglia, right parietal and frontal lobes. SPECT imaging revealed marked cerebral blood flow reduction, predominantly in the cerebral cortex corresponding to brain areas with high-intensity DWI signals. During the follow-up period of CJD180, DWI was more sensitive than conventional FLAIR and T2-weighted images (T2WI) to detect and monitor the progression of abnormal hyperintense lesions. We suggest that serial DWI and SPECT findings are useful for not only early diagnosis of CJD but also for monitoring disease progression.

Treatment of chronic akinetic mutism with atomoxetine: subtraction analysis of brain f-18 fluorodeoxyglucose positron emission tomographic images before and after medication: a case report.

Akinetic mutism is a rare, complex neuropathologic disorder. The pharmaceutical treatment of akinetic mutism typically includes dopaminergic agents, but the resulting therapeutic effects are often unsatisfactory, and it remains unclear whether late treatment using these medications is effective. We present a case study of a 53-year-old male patient who developed akinetic mutism for a period of 7 months after a subarachnoid hemorrhage. The hemorrhage was caused by a ruptured aneurysm in the right anterior communicating artery, followed by a secondary infarction in the territory of the right anterior cerebral artery. Baseline brain F-18 fluorodeoxyglucose positron emission tomographic images revealed decreased glucose metabolism in both frontal lobes. Treatment with atomoxetine, a selective norepinephrine reuptake inhibitor, for a period of 8 weeks led to a clinically significant improvement in the patient's cognitive function and activities of daily living. A subtraction brain positron emission tomographic analysis after atomoxetine medication revealed increased cerebral glucose metabolism in both the premotor and visual association cortices. Thus, we suggest that atomoxetine can be a useful therapeutic option in the treatment of chronic akinetic mutism.

Diminution of basal ganglia dopaminergic function may play an important role in the generation of akinetic mutism in a patient with anterior cerebral arterial infarct.

We report the clinical features and dopamine transporter [2-[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3.2.1]oct-2-yl]methyl](2-mercaptoethyl)amino]ethyl]amino]ethanethiolato(3-)-N2,N20,S2,S20]oxo-[1R-(exo-exo)]-[99mTc] technetium([99mTc]TRODAT-1) image finding in an 86-year-old woman with akinetic mutism due to infarction of bilateral anterior cerebral arterial territories. TRODAT-1 is a cocaine analogue that can be labeled with technetium-99m and bound to the dopamine transporter (DAT) site. It reflects primarily the integrity of presynaptic dopamine neuron terminals. With the evolution of the clinical features, the TRODAT SPECT images change from bilateral diminution of radioactivity uptake at the 81st-day check point to normal pattern at the 6-month one when the akinetic mute manifestations were nearly gone. This novel illustration suggests that the akinetic mutism caused by anterior cerebral arterial infarct is closely linked to the perturbation of the subcortical dopaminergic system. And the amelioration of the clinical features concordantly evolved with the restoration of the dopaminergic function.

Subarachnoid haemorrhage and akinetic mutism.

Two rare cases of akinetic mutism induced by aneurysmal subarachnoid haemorrhage are reported. The literature is reviewed, the possible pathophysiological mechanism of akinetic mutism is considered and the association between these two conditions is explored with an attempt to address the roles of medical treatment and surgery and to discuss prognosis.

Frontal lobe and cingulate cortical metabolic dysfunction in acquired akinetic mutism: a PET study of the interval form of carbon monoxide poisoning.

A middle-aged man suffering from acute carbon monoxide intoxication was clinically assessed to be in an akinetic and mute state. In order to elucidate regional cerebral disturbances, brain metabolism was investigated with fluoro-deoxyglucose positron emission tomography ((18)FDG-PET) 5.5 months after intoxication. Significantly reduced metabolic rates of glucose were revealed in selected brain regions, especially in both the frontal and anterior cingulate cortices, as well as in the subcortical white matter. Frontal and cingulate cortices showed a preserved metabolism of 35-53%, whereas the regional glucose consumption in cerebral white matter was reduced by more than 70%. In contrast, other areas of the brain such as the sensory-motor cortex, parts of the temporal lobes, basal ganglia and brainstem disclosed normal metabolic values. This lesion topography is discussed in relation to the development of akinetic mutism in the present case and in comparison with recent reports on the topic. Considering a plausible pathophysiology, akinetic mutism appears to be based on a different structural neuropathology when compared with the locked-in syndrome and the vegetative state. It is suggested that akinetic mutism is regarded as a specific condition characterized by injury of the frontal neuronal systems which promote executive functions.

[Significance of single photon emission computed tomography and akinetic mutism]. / Intérêt de la tomoscintigraphie de perfusion pour le diagnostic de mutisme akinétique.

Akinetic mutism is a reactive status with permanent opening of the eyes. The accountable lesions are always bilateral. The injured cerebral structures include the frontal gyri, the thalami or the mesencephalic areas. In one case of a 44-year-old patient, magnetic resonance imaging and computed tomography were not contributive. Tc99m brain SPECT imaging was performed and displayed bilateral frontal hypoactivity. This case suggests that this technique could be helpful for diagnosis when clinical features and radiological pattern are opposite.

Akinetic mutism: a report of three cases.

OBJECTIVES: To report 3 new cases of akinetic mutism, a clinical syndrome defined by silent immobility with preserved visual alertness not accountable by lesion of the areas and/or effector pathways of speech and voluntary movements. MATERIAL AND METHODS: Anatomopathological studies were performed in Cases 1 and 2; clinical follow-up, EEG, angiography and CT scans in Case 3. RESULTS: Case 1: Bipallidal necrosis; Case 2: Left pallidal necrosis with right frontoparietal cortico-subcortical infarction; Case 3: Striato-capsular infarction on the left side, involving the caudate nucleus and the anterior arm of the internal capsule, together with obstructive hydrocephalus. CONCLUSION: The roles of both globus pallidus and prefrontostriatal circuits in the onset of voluntary movements are discussed.

Assessment of brain perfusion by 99mTc-HMPAO SPECT in akinetic mutism due to high-dose intravenous methotrexate therapy.

Chemotherapy of the central nervous system may cause neurotoxicity in children with acute lymphocytic leukemia. We evaluated regional blood flow in a 6-year-old child presenting with akinetic mutism, using 99mTc-HMPAO single photon emission tomography (SPECT) following high-dose intravenous methotrexate therapy. While findings in X-ray computerized tomography were decreased density in bilateral basal ganglia and thalamic nuclei with diffusely decreased attenuation of the periventricular white matter, a global, frontal dominant profoundly abnormal perfusion pattern involving both gray and white matter was observed in the SPECT study. Treatment of the central nervous system with high dose intravenous chemotherapy may cause profound abnormalities in white and gray matter blood flow and early assessment of the neurotoxicity may be identified by 99mTc-HMPAO SPECT in the pediatric age group.

Role of cavum septum pellucidum in akinetic mutism of hydrocephalic children.

Two children 9 and 13 years of age with histories of aqueductal stenosis developed akinetic mutism after multiple shunt revisions. The girl had spontaneous relief of her akinetic mutism 1 month later, after parenteral antibiotics and multiple shunt revisions. The akinetic mutism of the boy was resistant to antiparkinsonian therapy before megadosage of bromocriptine (120 mg/day). Both children had a prominent cavum septum pellucidum (CSP), which has rarely been involved in the pathogenesis of akinetic mutism. Because of the rapid shrinkage of the girl's CSP after shunt revision, but not that of the boy even after stereotactic fenestration of the wall of his CSP, their eventual outcomes were different. Wide CSP (WCSP) may play an important role in the occurrence of akinetic mutism in hydrocephalic patients.

Computed tomographic findings in akinetic mutism.

Brain-injured children who progress from coma to a sleeplike state of akinetic mutism are often misinterpreted by their parents as having improved. In children with akinetic mutism caused by extensive destruction of the cerebral gray matter, the computed tomographic scan may demonstrate the irreversible nature of the pathologic changes.