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)

Neuroanatomical Mechanism of Cerebellar Mutism After Stroke

Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech due to cerebellar lesions. CM is often reported in children. We describe a rare case of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability, decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging revealed significant volume reduction of medial frontal projection fibers from the corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps major and minor and bilateral cingulum-angular bundles were not visualized. Cerebello-frontal pathway reconstructed from the FMRIB Software Library showed continuity of fibers, with decreased number of fibers on qualitative analysis. These results suggest that cerebello-frontal disconnection may be a neuroanatomical mechanism of CM. Damage of brain network between occipital lobe, cingulate and cerebellum caused by hemorrhage may also have role in the mechanism of CM in our case.

A case of Creutzfeldt-Jakob disease in a patient on hemodialysis

We report an unusual case of probable Creutzfeldt-Jakob disease (CJD) in hemodialysis patient. A woman 59 years of age with a past history of hypertension and end-stage renal disease presented with a stuporous state preceded by rapidly progressive cognitive dysfunction, myoclonus, and akinetic mutism. At first, the cause of the altered mental status was assumed to be uremic or hypertensive encephalopathy combined with fever. Proper managements, however, did not improve the neurologic symptoms. Diffusion-weighted magnetic resonance imaging revealed bilaterally asymmetric high signal intensity in both basal ganglia and cerebral cortices. Electroencephalography showed diffuse generalized theta-to-delta range slow wave and intermittent medium-to-high voltage complexes with a characteristic triphasic pattern on both hemispheres. Cerebrospinal fluid assay for the 14-3-3 protein was positive and diagnostic of CJD.

A Case of Keratitis Caused by Combined Infection of Multidrug-Resistant Acinetobacter Baumannii and Candida Parapsilosis

PURPOSE: To report a case of keratitis caused by Acinetobacter baumannii and Candida parapsilosis in a patient using 0.5% levofloxacin and 0.1% fluorometholone for an extended period of time. CASE SUMMARY: A 55-year-old male patient in the neurosurgery department with conjunctival injection and discharge in his left eye was consulted for ophthalmic evaluation and treatment. He was hospitalized in the state of akinetic mutism and given 0.5% levofloxacin and 0.1% fluorometholone for 2 years. On slit-lamp examination, a 3.5 x 4.5-mm dense stromal infiltrate with an overlying epithelial defect was observed. Corneal scraping culture revealed multidrug-resistant A. baumannii and C. parapsilosis. According to the susceptibility result, the patient was treated with topical 10% piperacillin/tazobactam and 0.125% amphotericin B hourly. The corneal ulcer healed gradually with corneal opacity remaining after 8 weeks of treatment. CONCLUSIONS: The authors of the present study report a case of treated multidrug-resistant A. baumannii and C. parapsilosis keratitis in a patient using topical antibiotics and steroid for an extended period of time.

Leukoencephalopathy and Akinetic Mutism in a Married Couple: A Probable Association with Oriental Medicine

BACKGROUND: Oriental medicines have been associated with severe psychiatric, neurological, and other adverse medical events. These medicines occasionally cause a typical reversible toxic encephalopathy, but most such cases are not recognized because these adverse events are complex and are associated with other systemic signs and symptoms. CASE REPORT: We describe a married couple with rapid progressive cognitive impairment and akinetic mutism after taking the same oriental medicines on the same day. Brain magnetic resonance images of the couple showed typical leukoencephalopathy in the periventricular white matter and basal ganglia regions, bilaterally. CONCLUSIONS: The development of neurobehavioral symptoms and toxic leukoencephalopathy in both patients following the ingestion of oriental medicines is suggestive of a cause-and-effect association, although such a relationship needs to be verified.

Encephalitis Lethargica With Catatonia

Encephalitis lethargica (EL) is an acute or subacute central nervous system (CNS) infectious disorder presenting with pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism), and neuropsychiatric sequelae. We report a 67-year-old man who had hypersomnolence, oculogyric crisis, akinetic mutism, parkinsonism, and malignant catatonia, which represented the stereotypic movement disorder with additional features of hyperthermia. His symptoms dramatically improved after steroid pulse therapy, indicating that EL syndrome could be a postinfectious neurological autoimmune CNS disorder.

Akinetic Mutism following Spontaneous Subarachnoid Hemorrhage: A Case Report / 대한뇌혈관외과학회지

Akinetic mutism (AM) is a rare complication of spontaneous subarachnoid hemorrhage (SAH). It is characterized by mutism and general hypokinesis in a patient superficially fully awake in that the eyes remain open and follow objects. Most common cause of AM in patients with SAH is secondary infarction in the distal anterior cerebral artery (DACA) territory. Sometimes post-SAH hydrocephalus has also been implicated in a delayed form of AM because of disruption of dopaminergic pathway. We report a case of 64-year-old woman who presented with sudden bursting headache without neurologic deficit. She was referred from the other hospital with a diagnosis of spontaneous SAH on head computed tomography (CT) which showed high density on sylvian, interhemispheric fissures and basal cistern. We performed computed tomographic cerebral angiography (CTA) and trans-femoral catheter cerebral angiography (TFCA), that revealed no definitive intracranial aneurysm, arterial dissection, or other vascular abnormality of the SAH. During admission period, she developed mutism and motor weakness of all limbs without spontaneous movement. We performed brain magnetic resonance (MR) image and RI cisternography. Also we prescribed levodopa. On the 33th hospital day, she had neurological recovery with drowsy mentality and grade 4 weakness of all limbs, but she was observed to suddenly develop general weakness and became cardiac arrest on the 55th hospital day. Efforts to resuscitate her failed, no more treatment could not be applied. In our case, we couldn't find infarction in the DACA terriority, midbrain or cerebellum except communicating hydrocephalus. This case may alert neurosurgeon to recognize the possibility of such rare complication after spontaneous SAH.

The Effect of Levodopa on Akinetic Mutism in Bilateral Anterior Cerebral Artery Infarction: A case report

Akinetic mutism is a clinical syndrome in which the patient is unable to speak (mutism) or move (akinesia). Various brain lesions can induce akinetic mutism. We attended a 71-year-old woman who presented with akinetic mutism caused by bilateral anterior cerebral artery infarction. The patient improved after the administration of levodopa com-bined with carbidopa, in response to visual and verbal stimuli. Increased verbal output and spontaneous motor activities were also noted. Levodopa may be helpful to the treatment of akinetic mutism.

Akinetic Mutism and Cognitive-Affective Syndrome Caused by Unilateral PICA Infarction

A 42-year-old man with left posterior inferior cerebellar artery (PICA) infarction presented with akinetic mutism and cognitive impairment. Initially he suffered from akinetic mutism and MRI-documented infarction in the distribution of the left PICA. Twelve days later he developed cognitive impairment. Neuropsychological tests were performed, with the results corrected for age and education being compared with published Korean norms. Impaired performances were evident on executive function testing, with difficulties in planning, abstract reasoning, set-shifting, and perseveration. Akinetic mutism and cognitive-affective syndrome may be a manifestation of unilateral PICA infarction.

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.

A Case of Akinetic Mutism Caused by Volume Change of Cerebral Ventricles

Akinetic mutism is a syndrome caused by various etiologies, and characterized by silent immobility and preserved alertness. The repetitive ventriculoperitoneal shunt for the recurrent hydrocephalus can be a forerunner of that. We present a man with akinetic mutism following two times of ventriculoperitoneal shunt revision. Akinetic mutism of the patient may be caused by the damage on the ascending dopaminergic projections. Symptoms were not alleviated by the normalization of ventricular size but by a large dose of bromocriptine.

Transient Akinetic Mutism Following General Anesthesia: A case report / 대한마취과학회지

A patient who had akinetic mutism was mistaken for a psychologic disorder or true coma. We experienced a case of reversible akinetic mutism and paraplegia for 5 hours after general anesthesia in a 28-year-old female who underwent debridement of chemical burn-induced skin necrosis on the left leg.

Transient Akinetic Mutism Following General Anesthesia: A case report / 대한마취과학회지

A patient who had akinetic mutism was mistaken for a psychologic disorder or true coma. We experienced a case of reversible akinetic mutism and paraplegia for 5 hours after general anesthesia in a 28-year-old female who underwent debridement of chemical burn-induced skin necrosis on the left leg.

Clinical manifestations and complications in carbon monoxide intoxication

Background ; Carbon Monoxide is colorless, ordorless, highly toxic gas produced by fire and by motor vehicles and appliances which use carbon-based fuels. In Korea, since coals were the main domestic fuel for cooking and for heating the floor, many cases of carbon monoxide intoxication have occurred annually. Until now, systemic complications & clinical manifestations as well as neurologic complications following carbon monoxide poisoning have rarely been reported. METHODS: I have researched systemic complications and clinical manifestations including neurologic complications from 1986 to 1997. I reviewed retrospectively medical records of 188 admitted patients to the Yonsei Medical Center due to carbon monoxide intoxication, and analyzed them according to the neurological, cardiovascular, respiratory, urogenital, dermatological and other systems. RESULTS: 1)The number of the admitted patients due to CO intoxication to Yonsei Medical Center between 1986 and 1997 has decreased compared to that between 1976 and 1981. 2)In this study, the cause of CO intoxication were furnaces in 170 cases(93.9%), fire accidents in 2 cases(1.1%), gas boilers in 2 cases(1.1%), LPG gas in 1 case(0.55%) and propan gas in 1 case(0.55%) in order. 3)Systemic complications and clinical manifestations after CO intoxication were 167(88.8%) nervous , 59(31.4%) musculoskeletal, 56(29.8%) respiratory, 49(26%) dermatologic, 44(23.4%) cardiovascular, 30(16.0%) genitourinary and 9(4.8%) other complications. Other systemic complications as well as neurological complications were not rare. 4)The clinical manifestations of neurological complications were decreased mental status(drowsy in 57 cases(30.3%), stupor in 63 cases(33.5%), semicoma in 33 cases(17.6%), coma in 13 cases(6.9%), alert in 22 cases(11.7%), focal sign related to the stroke in 16 cases (7.4%), incontinence in 22 cases(11.7%), seizure in 4 cases(2.4%), psychiatric behavior in 4 cases(2.4%), peripheral neuropathy in 4 cases(2.4%), bracheal plexopathy in 4 cases(2.4%), and torticollis in 1 case(0.5%). Delayed neurologic sequelae were akinetic mutism in 13 cases(76.5%), mental deterioration in 6 cases(35.3%), incontinence in 6 cases(35.3%), psychiatric behavior in 3 cases(17.6%), speech disturbance in 2 cases(11.8%), involuntary movement in 2 cases(11.8%), and depression in 1 case(5.9%) in order. 5)Cardiovascular complications showed tachycardia in 47 cases(28.3%), ischemic heart disease in 34 cases(18.6%), right bundle branch block in 6 cases(3.6%), left axis deviation in 4 cases(2.4%), atrial fibrillation in 3 cases(1.8%), sinus arrhythmia in 2 cases(1.2%), premature atrial contraction in 1 case(0.6%), premature ventricular contraction in 1 case(0.6%), primary atrio-ventricular block in 1 case(0.6%), prolonged QT in 1 case(0.6%). 6)Pulmonary complications were pneumonia in 23 cases(13.1%), pulmonary edema in 22 cases(12.6%), pneumonia with pulmonary edema in 3 cases(1.7%), and atelectasis, pulmonary hemorrhage, empyema, pleurisy in each 1 case(0.6%). 7)Azotemias were 26 cases(14.2%), clinically confirmed acute renal failures were 4 cases(2%) 8)Rhabdomyolysis was not rare as 63 cases(33.5%) compared to the previous report. 9)Dermatologic complications were erythema with bullae in 21 cases(11.9%), erythema without bullae in 14 cases(7.9%), bullae only in 10 cases(5.6%), laceration in 3 cases(1.65%), and ulceration in 1 case(0.55%). Conclusion ; We must consider other systemic manifestations as well as neurological symptoms in patients with CO intoxication since those are not infrequent in Korea.

A Case of Frontotemporal Dementia with Motor Neuron Disease

Dementia with motor neuron disease is a rare subtype of frontotemporal dementia. A 59-year-old woman presented with a twenty month history of abnormal behaviors and progressive cognitive decline. Abnormal behaviors consisted of emotional unconcern, persecutory delusion, distractibility, impulsivity, wondering and excessive laughing. Neuropsychological tests showed profound frontal lobe dysfunction out of proportion to amnesia, aphasia, and visuospatial dysfunction. Brain MRI showed subtle atrophy of both frontal lobes. However, brain FDG-PET showed glucose hypometabolism of bilateral frontal and anterior temporal lobes. An open brain biopsy of right frontal lobe demonstrated nonspecific findings including neuronal loss and microvacuolation with reactive astrogliosis in the absence of Alzheimer changes or Pick body. Four months later she developed amyotrophic lateral sclerosis starting with dysphagia. Her condition had continued to deteriorate, resulting in akinetic mutism and marked limb atrophy by the time she died of pneumonia three years postonset.

Rostral Midbrain Dysfunction Associated with Hydrocephalus Secondary to Periaqueductal Tumor

The authors report a case of rostral mesencephalic dysfunction due to obstructive hydrocephalus secondary to periaqueductal tumor who suffered multiple shunt failures and shunt revision. This patient presented with clinical syndromes, including akinetic mutism and diencephalic autonomic epilepsy. He had computed tomography(CT) and magnetic resonance(MR) findings of dilatation of ventricular system with periaqueductal tumor. The anatomical substrates of clinical findings are reviewed. A discussion of the probable mechanism proposes that dilatation of 3rd ventricle and rostral aqueduct sufficiently explains the entire syndrome.

La enfermedad de Creutzfeldt-Jakob: correlación clínica, electrofisiológica e histopatológica / Creutzfeldt-Jakob disease: clinical, electrophysiologic and histopathologic correlation

La enfermedad de Creutzfeldt-Jacob (ECJ) es una de las encefalopatías espongiformes subagudas caracterizada por deterioro neurológico progresivo e irreversible. Aunque se ha considerardo que el agente etiológico pueda ser un virus, los estudios experimentales han identificado "prion" como el agente infectante a una glicoproteína denominada. Pocos son los informes en donde se conjuguen las manifestaciones clínicas y los hallazgos electrofisiológicos. El presente artículo incluye tres casos de ECJ en los que las alteraciones en los potenciales evocados y el mapeo cerebral, brindan información útil sobre la enfermedad, especialmente en aquellos casos donde el cuadro clínico y los hallazgos electroencefalográficos son inespecíficos. Se concluye que las alteraciones observadas en los potenciales evocados (PE) sugieren que la afección histopatológica característica de encefalopatía espongiforme en la ECJ afecta estructuras corticales y subcorticales

Akinetic Mutism from Obstructive Hydrocephalus;Successful Treatment with Bromocriptine and Ephedrine

The authors report one case of akinetic mutism with obstructive hydrocephalus. The patient suffered from multiple shunt failures and shunt revision. After multiple shunt revision, the patient fell into an akinetic-mute state. She appeared awake but was no response to painful somatosensory, loud auditory or threatening visual stimuli and she required total nursing care. This behavioral syndrome was no response to shunt revision but we were able to successfully treat a case of akinetic mutism after combination theraphy of Bromocryptine and Ephedrine.