Acute Marchiafava‐Bignami Disease with Extensive Diffusion Restriction and Early Recovery: Case Report and Review of the Literature

Marchiafava‐Bignami disease (MBD) is a neurological disorder that has been found to be associated with chronic alcoholism and malnutrition. MBD classically results in acute edema and demyelination of the corpus callosum. Edema of the complete corpus callosum has been described to be an unfavorable prognostic factor. We present an acute onset of MBD with diffusion restriction of the complete corpus callosum and symmetric bilateral extension into the semioval center, that almost completely resolved clinically as well as in MRI only 3 days later. With early detection and treatment, the prognosis of MBD may be good even in cases with severe diffusion restriction of the complete corpus callosum.     

“Ears of the Lynx” Sign in a Marchiafava–Bignami patient: Structural Basis and Fiber‐Tracking DTI Contribution to the Understanding of this Imaging Abnormality

The “ears of the lynx” sign was previously reported as a neuroimaging finding observed in patients with autosomal recessive hereditary spastic paraplegia in association with a thin corpus callosum (ARHSP–TCC). We report a patient with a chronic form of Marchiafava–Bignami disease (MBD) that presented with this imaging feature. Diffusion tensor imaging (DTI) and fiber‐tracking data support that this finding is a consequence of the structural derangement, which enlarges a preexisting border zone of the bundles of fibers from the corpus callosum (CC) genu to the forceps minor and anterior corona radiata. Therefore, we assume that despite their pathological differences, damage to the anterior portion of the CC is responsible for the imaging similarities between MBD and ARHSP–TCC.     

Unilateral Apraxic Agraphia without Ideomotor Apraxia from a callosal lesion in a patient with Marchiafava-Bignami disease

Apraxic agraphia can be caused by left hemispheric cerebral lesions in the area that contains the spatial representations of the movements required to write, from a lesion in, or connections to, the frontal premotor cortex that converts these spatial representations to motor programs (Exner’s area). A right-handed woman with Marchiafava Bignami disease and lesions of the genu and splenium of her corpus callosum had apraxic agraphia without ideomotor apraxia of her left. A disconnection of Exner’s area in the left hemisphere from the right hemisphere’s premotor and motor areas may have led to her inability to write with her left hand.     

Cerebral microhemorrhage in Marchiafava–Bignami disease detected by susceptibility-weighted imaging

Marchiafava–Bignami disease (MBD) is a rare alcohol-associated disorder. Clinical features include not only disturbed consciousness, dysarthria, tetraparesis, astasia-abasia, and symptoms of interhemispheric disconnection as initial symptoms but also cognitive deficits as clinical outcomes. The clinical significance of cerebral microhemorrhage (CMH) has been recognized in patients with cognitive deficits; however, the presence of CMH in patients with MBD has not been emphasized. The aim of the present study was to clarify the relationship between CMH and MBD. For this purpose, we report four patients with MBD, who showed asymmetrical hypointense areas in multiple cortico-subcortical regions on susceptibility-weighted imaging (SWI). All cases had a history of chronic alcohol abuse and symmetrical lesions in the entire corpus callosum. These patients’ clinical symptoms included not only coma, dysarthria, and astasia-abasia as initial symptoms but also dementia as a clinical outcome. SWI showed asymmetrical hypointense areas in the multiple cortico-subcortical regions, indicating the presence of CMH. Compared with patients with normal cognitive function, demented patients showed higher severity of CMH. Our report would indicate that CMH is an important factor indicating the severity of dementia in patients with MBD.     

Fatal serotonin syndrome in a patient with Marchiafava–Bignami disease: Combined neurological and psychiatric emergency

Marchiafava–Bignami disease (MBD) is a rare fatal neurological disorder characterized by demyelination, primary degeneration, and necrosis of the corpus callosum. Although MBD is mostly associated with chronic alcohol consumption and malnutrition, it has been reported in non-alcoholic patients. Serotonin syndrome is a rare but potentially fatal side effect of antidepressants that results from overstimulation of both central and peripheral serotonergic receptors. In this report, we present a case with fatal serotonin syndrome happening in a non-alcoholic patient with the chronic form of MBD. To our knowledge, this case is the first report of fatal serotonin syndrome due to citalopram in an MBD patient. The present report may indicate that citalopram and other SSRIs should not be used in patients with MBD. Our case is also among few reported cases in the literature where no cause was identified in a patient with no previous history of alcohol intake.     

Delirium associated with concomitant use of duloxetine and bupropion in an elderly patient

Delirium is common in daily practice. Drug‐induced delirium constitutes approximately one‐third of all cases of delirium. In cases characterized by the limited efficacy of a single antidepressant, a combination of two antidepressants is required, which may induce a complex drug–drug interaction. We reviewed a case of duloxetine‐ and bupropion‐related delirium in an elderly male patient in our clinical practice. The patient was diagnosed with major depressive disorder and was treated with duloxetine. However, he developed delirium 10 days after bupropion was added to his treatment regimen. Three days after the cessation of bupropion, his delirious condition gradually improved. Duloxetine and bupropion are both cytochrome P450 2D6 inhibitors that may result in a higher level of hydroxybupropion. An increased level of hydroxybupropion may cause the elevation of dopamine and a risk of subsequent delirium. We should be aware of the risk of delirium induced by drug–drug interactions.     

胼胝体变性的临床特点与影像学特征研究

目的　研究分析了一组胼胝体变性 (MBD)患者的临床表现和影像学特征 ,探讨不同临床分型的特点与诊断方法。方法　 4例长期酗酒男性MBD患者 ,急性型 1例 ,慢性型 3例 ,均经CT检查 ,3例经头MR检查 ,1例行尸检病理检查。结果　急性型主要表现为意识障碍、精神症状与癫 ;慢性型以痴呆、精神症状为主要表现 ;CT与MR可见胼胝体膝部和压部病灶。CT病灶为低密度 ,MR表现为T1WI低信号 ;T2 WI高信号 ;2例伴发双侧大脑半球白质对称性病灶 ,1例出现脑干对称性病灶。尸检病理提示 :胼胝体呈多灶性脱髓鞘与坏死改变。结论　急性型MBD意识障碍突出 ,病程进展迅速 ;慢性型以痴呆、精神症状和锥体束损害为主要表现 ;CT与MR为其生前诊断的主要手段 ,以胼胝体膝部、压部对称性病灶为其主要异常表现。     

Delayed onset and prolonged interictal delirium following electroconvulsive therapy

Electroconvulsive therapy is safe and effective in the treatment of depression in older individuals. Minor cognitive side effects of electroconvulsive therapy include acute postictal confusion and reversible short‐term memory deficits. However, interictal delirium is uncommon in absence of risk factors. Herein, we report the case a depressed male patient without any known risk factors who developed interictal delirium 2 days after his sixth electroconvulsive therapy session. Interictal delirium improved with treatment within 1 week.     

Tuberculous meningitis masked by delirium in an alcohol-dependent patient: a case report

OBJECTIVE: Patients with alcohol dependence syndrome can present with delirium which will mask underlying organic causes for the delirium. However, other medical diseases can also present with similar symptoms and should not be missed. The issues related to differentiating the different causes of delirium are briefly discussed. We describe a case of tuberculous meningitis in a patient with history of alcohol dependence who presented with delirium. METHOD: A case report. RESULTS: A 38-year-old male was admitted with history of irrelevant talk and abnormal behaviour of 2-month duration. He was also disoriented and his short-term memory was impaired. He reported visual hallucinations. He had history of alcohol dependence of 5 years. A detailed mental status examination and neurological workup revealed an organic psychosis. CT scan showed a hypodense lesion suggestive of a tuberculoma. The cerebrospinal fluid findings were corroborative. He responded to antituberculous drugs which he took for one and a half years and recovered completely. He also underwent group therapy for his alcohol dependence and has since then refrained from alcohol intake. Currently he has gone back to his work as a car mechanic. CONCLUSION: We have highlighted the need for diagnosing and investigating carefully the cause of delirium in a patient with alcohol dependence syndrome. This shows that other curable causes of delirium must also be investigated in patients with alcohol dependence.     

Leptomeningeal carcinomatosis from urinary bladder adenocarcinoma: A clinicopathological case study

We report a 73‐year‐old male patient with leptomeningeal metastasis from urinary bladder adenocarcinoma. He was presented with prominent hyperactive delirium during the course of the disease. Meningeal carcinomatosis was detected 5 days before his death, but the primary site of the malignant tumor could not be determined. Necropsy revealed leptomeningeal infiltration of many adenocarcinoma cells that covered the cerebrum. The leptomeninges of the right middle frontal gyrus, superior temporal gyrus, precentral gyrus and inferior parietal lobe were most severely affected by tumor cell infiltration. Cerebral edema was found to extensively cover the basal part of the temporal lobe. In the cerebrum, tumor cells were clustered in the perivascular spaces and had invaded localized areas of the frontal lobe. Vascular cell adhesion molecule (VCAM)‐1 expression was detected in the small vessels of the cerebral upper cortical layers and of temporal subcortical u‐fibers. Numerous astrocytes positive for cytokeratin AE1/AE3 were found in the frontal and temporal lobes. Meningeal carcinomatosis from urinary bladder adenocarcinoma is extremely rare and up‐regulation of the adhesion molecules in the meningeal adenocarcinoma was confirmed.     

Delirium in inpatients with respiratory diseases

The features of delirium in patients being hospitalized due to respiratory diseases were investigated. From the inpatients in the respiratory medical ward of Tokyo Metropolitan Hiroo General Hospital over the course of 1 year, the patients who had delirium were diagnosed by a semistructured interview. The total number of subjects was 454, and patients with delirium were 43. Various clinical factors were compared between the delirium group and non-delirium group. In the delirium group, there were many elderly patients of 70 years or older. Moreover, there were many patients who had a chronic respiratory disease, patients in which the respiratory diseases were mutually complicated, and patients in whom other diseases combined with the respiratory disease in the delirium group. There were also many patients in the Intensive Care Unit (ICU), and patients with an endotracheal intubation or extubation. Based on the results of a multiple logistic regression analysis, for age, ICU accommodation, and endotracheal intubation, the value of the delirium group was more significant than that of the non-delirium group. In half of the patients from the delirium group, delirium developed within 1 week after hospitalization. In the patients who died in the hospital, however, delirium often developed days after they had been hospitalized. It was suggested that the later developed delirium had a relation to the prognosis.     

Alcohol withdrawal delirium manifested by manic symptoms in an elderly patient

Alcohol withdrawal syndrome is a commonly seen problem in psychiatric practice. Alcohol withdrawal delirium is associated with significant morbidity and mortality. Withdrawal symptoms usually include tremulousness, psychotic and perceptual symptoms, seizures, and consciousness disturbance. Herein, we report a case involving a 63‐year‐old man who had alcohol withdrawal delirium that was manifested mainly by manic symptoms.     

Successful treatment with quetiapine for delirium in terminally ill cancer patients

We describe six patients with terminal cancer whose delirium improved after they started quetiapine (QTP). Haloperidol (HPD) had failed to improve their delirium, and they had suffered adverse effects, including over‐sedation, extrapyramidal signs and dysphagia. We prescribed QTP to improve their delirium. We evaluated the severity of the delirium using the Japanese version of the Memorial Delirium Assessment Scale (jMDAS) before QTP treatment and 3, 7 and 14 days after QTP administration. The basal the Japanese version of the Memorial Delirium Assessment Scale score ranged from 18 to 24. Effective doses of QTP in the present cases were 12.5–50 mg/day. Delirium improved in all patients, and no significant adverse effects were observed. Several factors appear to contribute to symptoms of delirium in patients with terminal cancer. These causative factors are usually complicated and delirium often is refractory to usual pharmacotherapy, involving typical neuroleptics like HPD. QTP, however, appeared to be useful for delirium treatment in patients with advanced cancer.     

Delirium on re-starting clozapine after a short break in treatment

Extra care must be taken in treating patients with clozapine because of the serious side-effects. A 44-year-old man with schizophrenia developed delirium on two occasions immediately after restarting clozapine at the dosage he had previously tolerated well; the clozapine-free periods had lasted 2 and 10 days respectively. Because of this 're-challenge' it seems very likely that there is a causal relation between the direct resumption of treatment with clozapine and delirium. Even if clozapine treatment is interrupted for only a short time it is important that the 'new' course begins with a low dosage and is increased very cautiously until it reaches the former, tolerated level.     

Duration of Delirium Shortened by the Correction of Electrolyte Imbalance

Abstract: Among 53 delirious patients with an electrolyte imbalance in the serum, the duration of delirium in 18 cases was significantly shortened by the correction of electrolyte imbalance, compared with that of 35 cases of delirium without an electrolyte correction. The mean duration of delirium (mean value ± SE) corrected by electrolyte was 9.4 ± 1.9 days, whereas the mean duration of delirium without the electrolyte correction was 25.7 ± 4.6 days. The mean duration of delirium in 13 cases without the electrolyte imbalance was 25.0 ± 6.6 days. From these results, the electrolyte correction should be done systematically for the delirious patients as a treatment for the disorder.     

Duration of delirium shortened by the correction of electrolyte imbalance

Among 53 delirious patients with an electrolyte imbalance in the serum, the duration of delirium in 18 cases was significantly shortened by the correction of electrolyte imbalance, compared with that of 35 cases of delirium without an electrolyte correction. The mean duration of delirium (mean value +/- SE) corrected by electrolyte was 9.4 +/- 1.9 days, whereas the mean duration of delirium without the electrolyte correction was 25.7 +/- 4.6 days. The mean duration of delirium in 13 cases without the electrolyte imbalance was 25.0 +/- 6.6 days. From these results, the electrolyte correction should be done systematically for the delirious patients as a treatment for the disorder.     

A case of topical opioid‐induced delirium mistaken as behavioural and psychological symptoms of dementia in demented state

In Japan, indications for opioid analgesics, once exclusively used as pain killers for patients suffering from malignant cancer, have been expanded for a wide range of pain. Herein we report a patient with opioid‐induced delirium associated with the administration of buprenorphine patches that was well below the indicated therapeutic range limit. An 82‐year‐old woman was referred to us from an orthopaedic practitioner for uncontrollable behavioural problems apparently caused by the beginning of dementia; the patient had gradually developed disorientation, visual hallucinations, and delusions. Laboratory and imaging findings excluded common causes of delirium including Alzheimer's disease and diffuse Lewy body disease. Detailed questioning revealed that the patient's confused state appeared following a buprenorphine patch dose increase and subsequently disappeared after administration was stopped. Delirium has not been reported as a side‐effect in clinical trials of buprenorphine patches. However, our findings in this case show that even topical opioids can precipitate the development of a delirious state in elderly patients.     

Clinical experience of quetiapine in 24 elderly patients with delirium

Background: A recent study reported that patients with delirium responded well to the administration of atypical antipsychotic agents. In the present study we administered quetiapine to patients with delirium and obtained good results. Methods: This study included 24 patients (10 men, 14 women), referred to the psychiatry department during admission to other hospital departments, who were diagnosed as having delirium according to the diagnostic and statistical manual of mental disorders (4th edition) (DSM‐IV) between April 2001 and September 2002. The mean age of the patients was 76.5 years (men 71.0 years; women 80.5 years). An initial dose of quetiapine was established at 25–50 mg/day. Depending on the symptoms, the dose and frequency were increased as required. According to Trzepacz's delirium rating scale (DRS), the treatment response was evaluated prior to the administration of quetiapine and 1, 3, 5 and 7 days after administration began. Results: Prior to the administration of quetiapine, the mean DRS score was 18.1. The mean scores were 12.2, 10.8, 9.7 and 8.9 after 1, 3, 5 and 7 days of quetiapine administration, respectively. These values were significantly lower than the value before administration (P < 0.001). Seven days after the administration of quetiapine commenced, the total DRS score was lower than the cutoff point (12) in 20 patients (83.3%). In 18 patients (75.0%), delirium was clinically relieved. Doses ranged from 25 mg/day to 125 mg/day, with a mean dose of 54.7 mg/day. With respect to the administration method, the majority of patients (i.e. 13 patients) received quetiapine once per day (after dinner). Somnolence was observed in three patients as a side‐effect of quetiapine administration. However, this side‐effect improved after 1–2 days, without decreasing the dose. Conclusions: Quetiapine may be useful for controlling delirium and concerning side‐effects and extrapyramidal symptoms were not recorded in the present study. Thus, it is appropriate to trial quetiapine in the treatment of delirium.     

Spinal Subdural Hemorrhage as a Cause of Post-Traumatic Delirium

A 64-year-old man with TBI was admitted to our institute. In following days, he showed unusual behavior of agitation, restlessness, emotional instability and inattention. Post-traumatic delirium was tentatively diagnosed, and donepezil was given for his cognitive dysfunction. Although there was partial relief of agitation, he sustained back pain despite medication. Lumbar magnetic resonance image revealed SDH along the whole lumbar spine, and surgical drainage was followed. Postoperatively, his agitation disappeared and further medication was discontinued. We report a unique case of post-traumatic delirium in a patient with concomitant TBI and spinal subdural hemorrhage (SDH) that resolved with operative drainage of spinal hemorrhage.     

Le Plancher de Jeannot

This is the story of Jeannot based on a number of tragic events that predated his disease, a paranoid delirium shared by his older sister, their seclusion and the denial of their mother's death, after which the engraving of his room's wooden floor was realized and just before he died. This interview with the neuro-psychiatrist Guy Roux relates the discovery of this piece of work and the very first exhibitions in the psychiatric circles. The acquisition by the laboratory Bristol-Myers Squib triggered many cultural events with a broad media exposure so it was received with a great enthusiasm. The setting of the floorboard at hospital Sainte-Anne in Paris initiated by Pr Jean Pierre Olié was greeted by several comments of which some criticisms. Multiple novels and pieces of theatre were inspired by this story.