ABSTRACT
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.
Subject(s)
Akinetic Mutism/physiopathology , Basal Ganglia Diseases/physiopathology , Encephalitis, Viral/physiopathology , Epstein-Barr Virus Infections/physiopathology , Status Epilepticus/physiopathology , Adolescent , Akinetic Mutism/diagnostic imaging , Akinetic Mutism/immunology , Akinetic Mutism/therapy , Anticonvulsants/therapeutic use , Autoantibodies/immunology , Basal Ganglia Diseases/diagnostic imaging , Basal Ganglia Diseases/immunology , Basal Ganglia Diseases/therapy , Brain Edema/diagnostic imaging , Brain Edema/immunology , Brain Edema/physiopathology , Brain Edema/therapy , Chromonar , Electroencephalography , Encephalitis, Viral/diagnosis , Encephalitis, Viral/immunology , Encephalitis, Viral/therapy , Epstein-Barr Virus Infections/diagnosis , Epstein-Barr Virus Infections/immunology , Epstein-Barr Virus Infections/therapy , Glucocorticoids/therapeutic use , Humans , Magnetic Resonance Imaging , Male , Methylprednisolone/therapeutic use , Receptors, Dopamine D2/immunology , Recovery of Function , Status Epilepticus/immunology , Status Epilepticus/therapyABSTRACT
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.
Subject(s)
Akinetic Mutism/etiology , Intracranial Thrombosis/complications , Venous Thrombosis/complications , Adult , Akinetic Mutism/psychology , Akinetic Mutism/therapy , Catheterization, Central Venous , Cerebral Veins , Combined Modality Therapy , Female , Humans , Intracranial Thrombosis/psychology , Intracranial Thrombosis/therapy , Treatment Outcome , Venous Thrombosis/psychology , Venous Thrombosis/therapyABSTRACT
The toxicity of xenobiotics can result inrare disorders of consciousness, such as akinetic mutism and somnambulism as well as syndromes mimicking consciousness disturbances, such as locked-in syndrome and psychogenic coma. Akinetic mutism is a condition characterized by a lack of spontaneous movements and little or no vocalization. Somnambulism include performing of complex motor activity in an automatic manner during deep sleep, without any awareness of its execution. The locked-in syndrome is a state with quadriplegia coexisting with cranial nerves palsies and mutism, but with fully preserved consciousness. Psychogenic coma is a condition in which the patient has preserved level of consciousness and awareness, but does not communicate with theenvironment and does not exhibit the external manifestations of consciousness. This paper presents the etiology, clinical characteristics, as well as diagnostic and therapeutic issues for the above syndromes.
Subject(s)
Akinetic Mutism/chemically induced , Coma/chemically induced , Quadriplegia/chemically induced , Somnambulism/chemically induced , Xenobiotics/poisoning , Akinetic Mutism/diagnosis , Akinetic Mutism/therapy , Coma/diagnosis , Coma/therapy , Diagnosis, Differential , Humans , Quadriplegia/diagnosis , Quadriplegia/therapy , Somnambulism/diagnosis , Somnambulism/therapySubject(s)
Akinetic Mutism/etiology , Hydrocephalus/surgery , Parkinson Disease/etiology , Ventriculoperitoneal Shunt/adverse effects , Akinetic Mutism/diagnosis , Akinetic Mutism/therapy , Antiparkinson Agents/therapeutic use , Bromocriptine/therapeutic use , Child , Female , Humans , Parkinson Disease/diagnosis , Parkinson Disease/therapy , Physical Therapy Modalities , Treatment OutcomeABSTRACT
The physician must explain the treatment or procedure in detail including risks, benefits, and alternative options; the patient's choice must be voluntary; the patient must demonstrate his or her ability to understand the risks and benefits of their choice; and the patient must be able to manipulate information in a logical way. These criteria must be met in order for the process of informed consent to be valid.
Subject(s)
Choice Behavior , Informed Consent/psychology , Physician-Patient Relations , Physicians/psychology , Risk Assessment , Aged , Akinetic Mutism/diagnosis , Akinetic Mutism/therapy , Disclosure , Ethics, Medical , Humans , MaleABSTRACT
El mutismo acinético (MA) es un trastorno del comportamiento, caracterizado por la incapacidad para moverse o hablar en pacientes despiertos. Típicamente ha sido descrito como una complicación transitoria de la cirugía de tumores de fosa posterior. Sin embargo, el MA también puede aparecer tras múltiples fallos valvulares en pacientes hidrocefálicos. En estos casos, el MA no mejora espontáneamente, ni con revisiones valvulares, pero puede responder al tratamiento con bromocriptina. Presentamos una paciente con MA tras una cirugía de fosa posterior, complicada por una ventriculitis con dilataciones ventriculares repetidas, que sólo mejoró con bromocriptina. Revisamos la fisiopatología del MA. Aunque ésta no sea bien conocida, parece que la afectación del núcleo dentado y de sus eferencias (principalmente de glutamato) sería responsable del MA de origen cerebeloso, mientras que la afectación de las vías monoaminérgicas paraventriculares explicaría el MA relacionado con dilataciones ventriculares repetidas y que responde al tratamiento con bromocriptina. Aun así, se requiere un estudio más profundo de esta patología para aclarar su etiología
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
Subject(s)
Male , Female , Humans , Akinetic Mutism/diagnosis , Akinetic Mutism/physiopathology , Akinetic Mutism/therapy , Bromocriptine/administration & dosage , Bromocriptine/therapeutic use , Hydrocephalus , Ephedrine/therapeutic use , Cerebellum/surgery , Cranial Fossa, Posterior/surgery , Brain NeoplasmsABSTRACT
The case of a 21-year-old man with obstructive hydrocephalus who suffered multiple shunt failures is presented. The patient developed a syndrome of akinetic mutism that improved after administration of bromocriptine and metoprolol.
Subject(s)
Akinetic Mutism/etiology , Hydrocephalus/complications , Adult , Akinetic Mutism/diagnosis , Akinetic Mutism/therapy , Bromocriptine/therapeutic use , Cerebral Ventricles/pathology , Cerebrospinal Fluid Shunts , Humans , Hydrocephalus/diagnosis , Hydrocephalus/therapy , Magnetic Resonance Imaging , Male , Metoprolol/therapeutic use , Tomography, X-Ray ComputedABSTRACT
Stupor is a rare and dramatic presentation of a number of neurological and psychiatric conditions which can cause problems in both diagnosis and management. A case is described which illustrates these difficulties.
