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.

A Case of Abulia From Left Middle Cerebral Artery Stroke in an Adolescent Treated Successfully With Short Duration Olanzapine.

INTRODUCTION: Abulia is defined as a pathological state of amotivation, apathy, and global absence of willpower. It presents with a challenging array of overlapping symptoms, making effective identification and treatment difficult. CASE PRESENTATION: We describe the first known report of an adolescent with a ventricular assist device who developed abulia following a left middle cerebral artery (MCA) stroke who responded successfully to treatment with olanzapine. DISCUSSION: The neurobiological etiology of abulia is still unclear but is postulated to be related to deficits in the dopaminergic reward circuitry in the frontal-subcortical-mesolimbic regions. There have been reports of poststroke patients with abulia being treated by modulating this dopamine circuitry and in some cases with short-term low-dose olanzapine. CONCLUSION: Further research is needed to develop a better understanding of the pathophysiology of abulia leading to more effective treatment algorithms including more specific diagnostic tools and effective pharmacological interventions.

Rotigotine effect in prolonged disturbance of consciousness. Brief report of two cases.

Two patients with post-coma reactivation deficiency who showed a "dramatic" response to rotigotine therapy are described. They had suffered from prolonged coma due to lesions in the mesencephalic ventral tegmental area. The authors believe that rotigotine effect in these cases could be due to restoration of dopaminergic transmission in medial frontal areas previously "de-afferented" from the lesions. Some comatous patients may experience a prolonged difficulty in recovering a normal state of consciousness. This phenomenon may be due to dysfunction of amynergic activating pathways connecting brainstem to the frontal cerebral cortex. In particular, dysfunction of dopaminergic pathways from the mesencephalon to the frontal cortical areas may be responsible for clinical pictures characterized by preserved alertness and total loss of interactions with the surrounding environment; the so called "waking coma" cases.

On the pathophysiology and treatment of akinetic mutism.

Akinetic mutism (AM) is a rare neurological disorder characterized by the presence of an intact level of consciousness and sensorimotor capacity, but with a simultaneous decrease in goal-directed behavior and emotions. Patients are in a wakeful state of profound apathy, seemingly indifferent to pain, thirst, or hunger. It represents the far end within the spectrum of disorders of diminished motivation. In recent years, more has become known about the functional roles of neurocircuits and neurotransmitters associated with human motivational behavior. More specific, there is an increasing body of behavioral evidence that links specific damage of functional frontal-subcortical organization to the occurrence of distinct neurological deficits. In this review, we combine evidence from lesion studies and neurophysiological evidence in animals, imaging studies in humans, and clinical investigations in patients with AM to form an integrative theory of its pathophysiology. Moreover, the specific pharmacological interventions that have been used to treat AM and their rationales are reviewed, providing a comprehensive overview for use in clinical practice.

Akinetic mutism responsive to bromocriptine following subdural hematoma evacuation in a patient with hydrocephalus.

An 11-year-old girl with obstructive hydrocephalus developed akinetic mutism after treatment for hydrocephalus due to aqueductal stenosis by ventriculoperitoneal (VP) shunting. Bilateral chronic subdural hematomas developed about 2 months after insertion of the VP shunt and were evacuated. Postoperatively, the patient developed akinetic mutism, but her condition improved after administration of bromocriptine. Absence of abnormalities on dopamine transporter single photon emission computed tomography, lack of clinical response to levodopa treatment, and normal homovanillic acid concentration in the cerebrospinal fluid all indicated normal dopamine production. Pressure on the periventricular monoamine projections in the thalamus and hypothalamus without major dopamine deprivation in the striatum may have been the most important factors in the development of akinetic mutism in this patient.

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.

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.

[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.

Abulia: no will, no way.

Abulia refers to impaired ability to perform voluntary actions, show initiative, make decisions along with decrease in movements, speech, thought and emotional reactions. We describe here two patients who developed this condition following bilateral insult to different sites in the centromedial core of the brain, the first following the cerebral venous thrombosis and the second after the right ACA and MCA infarct. Both these patients improved following treatment with Bromocriptine. These cases are described for proper identification and management by the clinicians.

[Akinetic mutism related to hydrocephalus and cerebellar surgery treated with bromocriptine and ephedrine. A pathophysiological review]. / Mutismo acinético relacionado con hidrocefalia y cirugía cerebelosa tratado con bromocriptina y efedrina. Revisión fisiopatológica.

Akinetic mutism (AM) is a behavioral disorder characterized by impossibility to move or speak in awake patients. lt has been typically described as a transient disorder following posterior fossa tumour resection. Besides, AM may also appear after recurrent shunt failures in hydrocephalic patients, with no tendency towards improvement, either spontaneously or with shunt revisions. However successful treatment of this second type of AM has been achieved with bromocriptine. We present a patient who developed AM after a posterior fossa surgery complicated by ventriculitis and multiple hydrocephalic events. AM only improved with bromocriptine. We review AM pathophysiology. Although not well known, it appears to be quite different, depending on its cerebellar or hydrocephalic origin. Damage to dentate nucleus or its efferents (mainly of glutamate) should promote AM of cerebellar origin, while damage to paraventricular monoaminergic pathways could explain AM related to repeated shunt failures which has successful response to bromocriptine treatment. However, a more complete study of this disorder is required to ascertain its aetiology.

Chronic akinetic mutism after mesencephalic-diencephalic infarction: remediated with dopaminergic medications.

OBJECTIVE: Akinetic mutism (AKM) is an uncommon disorder with a complex neuropathology. There is no generally accepted treatment, and it is not known if late treatments are effective. The relationship between AKM and abulia is uncertain. METHODS: The effects of dopaminergic treatment of a patient with chronic AKM after discrete bilateral infarctions of the mesencephalic ventral tegmental area and the lateral hypothalamus were studied with motor measures, the Functional Independence Measure (FIM), and neuropsychological tests. RESULTS: Treatment with a combination of carbidopa/levodopa and pergolide produced prompt amelioration of AKM with dramatic and rapid improvement in FIM. An apathetic, amotivational state persisted despite resolution of akinesia and normal frontal executive functions. CONCLUSIONS: AKM may respond to dopaminergic treatment even after months of severe akinesia. The mechanism of abulia is more complex than simply a partial dopaminergic deficiency state and may persist even when AKM is treated and frontal cognitive functions are normal.

Relief of akinetic mutism from obstructive hydrocephalus using bromocriptine and ephedrine. Case report.

The case of a 20-year-old man with obstructive hydrocephalus who suffered multiple shunt failures and shunt revisions is presented. The patient gradually developed a clinical syndrome of akinetic mutism. This behavioral syndrome failed to respond to shunt revisions, but did improve after the administration of a combination of bromocriptine and ephedrine.

Bromocriptine-responsive akinetic mutism following endoscopy for ventricular neurocysticercosis. Case report and review of the literature.

A 26-year-old woman with obstructive hydrocephalus caused by a cysticercal cyst blocking the left foramen of Monro was initially treated with an external ventriculostomy and subsequent endoscopically assisted cyst resection and septostomy. Postoperatively, the patient developed the clinical syndrome of akinetic mutism, but her condition improved after the administration of the dopaminergic agonist bromocriptine. This result indicates that a disruption in the ascending dopaminergic pathway involved in behavior formation was likely the cause of these neurological symptoms.

Akinetic mutism: pharmacologic probe of the dopaminergic mesencephalofrontal activating system.

Four children who exhibited akinetic mutism during the course of their neurologic diseases were treated with bromocriptine. Reversal of the akinetic mute states was evident in all patients. Pathways of the dopaminergic neurons are reviewed and a clinically useful mechanism which causes akinetic mutism is postulated.

Parkinsonian syndrome complicating systemic lupus erythematosus.

Two girls with florid extrapyramidal parkinsonism complicating systemic lupus erythematosus (SLE) are reported. One patient (15 years old) presented with extreme rigidity, irritability, and mutism initially diagnosed as acute psychosis. Examination revealed severe extrapyramidal akinetic mutism, along with marked restlessness. CT and MRI imaging of the brain were unremarkable. EEG revealed moderate generalized disturbance of background activity. 99mTc-HmPAO SPECT cerebral scanning detected decreased regional cerebral blood flow at the basal ganglia. Dopamine-agonist drugs led to complete recovery after 3 months, along with normalization of EEG and SPECT alterations. The second patient (16 years old) was assessed for progressive bradykinesia and apathy impeding her active daily activities, and she was suspected to have developed depression. Neurologic assessment revealed a parkinsonian syndrome that was less severe than that of the first patient. The EEG showed mild disturbance of background activity, and 99mTc-HmPAO SPECT demonstrated impaired regional cerebral blood flow over the basal ganglia. A parkinsonian extrapyramidal syndrome complicating SLE should therefore be taken into account in any patient with SLE presenting with marked behavioral alterations, rigidity, or akinetic mutism.

[Effect of trihexyphenidyl-HCL on posttraumatic prolonged disturbance of consciousness: report of two cases].

Cases involving two patients who presented post-traumatic prolonged disturbance of consciousness (PTPDC), namely akinetic mutism, and recovered from it after treatment with trihexyphenidyl were reported. Case 1: A seventy-one-year-old farmer. Five months after head injury, when he was first admitted to us, he was stable with signs of oligokinesia, katatonic posture, speechlessness, rigid muscle tones and positive cog-wheel phenomenon. One week after administration of the drug, his speech and voluntary movement improved remarkably. Two months after the treatment, he was able to walk, and was discharged from the hospital. Case 2: A forty-six-year-old man sustained major head trauma. In the acute stage, he was comatose with decerebrate posture. On the 15th hospital day, he showed a state of akinetic mutism with normal sleep - wakefulness cycle. Evacuation of the collected subdural fluid was done one month after the injury, which resulted in no change in his clinical state. Five months after the injury, trihexyphenidyl treatment was begun. A few days after the treatment, his motor activity and his facial expression obviously improved. One week after, he mimicked the word 'o-ha-yo (good morning)' after the physician's greeting. CT scan and magnetic resonance imaging in the chronic state of these patients showed bifrontal cerebral white matter lesions, which indicated old cerebral contusion. No brain stem lesions were detected with these examinations. Our two cases clearly did not belong to the category of post-traumatic parkinsonism because of their clinical courses, and their features shown in radiological examinations. However the anti-parkinsonian drug, trihexyphenidyl was effective.(ABSTRACT TRUNCATED AT 250 WORDS)

[Akinetic mutism from recurrent hydrocephalus: successful treatment with levodopa, bromocriptine and trihexyphenidyl].

A case of akinetic mutism was reported with reference to a marked improvement by levodopa, bromocriptine and trihexyphenidyl. A 39-year-old male, first seen on February 2, 1981, had an occipitalgia, accompanied by nausea and vomiting. For several months before this consultation, the patient had suffered from asthenopia. Brain CT scan and cerebral angiogram demonstrated internal hydrocephalus due to aqueduct stenosis of unknown etiology. After a ventriculoperitoneal shunt operation on February 20, 1981, he completely recovered. Two years and a half after the shunt insertion he had no difficulty in his daily life. He reentered the hospital on December 21, 1983, because of personality change, mental deterioration and bradykinesia. Brain CT scan showed recurrent hydrocephalus resulting from shunt blockage. Following the shunt revision, hydrocephalus was resolved. Nevertheless, the patient did not return to his previous state. And he became bed-ridden, incontinent of urine, and unable to take fluids or foods, following which he went into a state of akinetic mutism. Other neurological findings were as follows: upward gaze palsy, impaired convergence, convergence nystagmus, plastic rigidity of neck and all four limbs, and diffuse hyperreflexia with right Babinski's sign. Abnormal involuntary movement was not seen. On March 27, 1984, levodopa therapy was instituted and on April 2, trihexyphenidyl was combined with levodopa. Shortly after administration of levodopa and trihexyphenidyl, akinetic mutism began to improve, but upward gaze palsy was not affected. He began to speak and could walk unassisted by the end of July.(ABSTRACT TRUNCATED AT 250 WORDS)