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[Clinical forms of acute herpetic infections of the central nervous system in adults].

The authors describe the course of herpetic encephalitis in 52 patients aged 16 to 64 years. Five types of the initial manifestations of herpetic infection of the CNS were revealed. In 44.3% of cases the disease started from the general cerebral symptomatology and consciousness disturbance; in 13.6% it started in a brain stroke-like manner followed by the development of the comatose status; in 13.6% of cases from memory disorder and unmotivated actions; in the same percentage of cases, the disease onset was marked by the dominance of dizziness, diplopia, ataxia and central hemiplegia ; in 15.9% the disease started from pains in the stomach, loin and lower limbs. Hemispheric and pseudotumorous stem encephalitides (48.1 and 13.6% respectively) were predominant; in 25% meningoencephalitides and in the remainder, encephalomyelitis running their course in the form of disseminated encephalomyelitis (5.7%), focal myelitis (5.7%) or opticomyelitis (1.9%). The data presented attest to the pleomorphism of the clinical picture of herpetic lesions of the CNS.

Acute Disease↗

[The profile of memory disturbance in early Lewy body dementia differs from that in Alzheimer's disease].

It may be difficult to distinguish Lewy body dementia (LBD) from Alzheimer's disease (AD) especially at an early stage. Clinical diagnostic criteria for LBD still lack sensitivity. We compared memory performance in early AD and LBD to identify features that may help to distinguish these two conditions. Patients with Mini Mental State scores equal or above 18 were consecutively selected retrospectively from the University Out-patient Memory Clinic database. Probable LBD and AD were respectively diagnosed according to the international consensus criteria and the NINCDS-ADRDA criteria. Short-term memory was assessed with the digit span subtest of the Wechsler Memory Scale and the Corsi block-tapping test. Long term verbal memory was assessed with a French version of the Grober and Buschke test. Long-term visual memory was tested with the recall of four geometric figures of the CERAD battery. There was no difference between the two groups in short-term or long-term visual memory. Except for the immediate and first free recalls, DCL patients performed significantly better than AD patients in all subtests of the Grober and Buschke test. The more striking differences were seen with the third total and delayed total recall. These results highlight the differences of memory impairment pattern in early LBD and AD. Episodic verbal memory is better in LBD patients who benefit more from cues, reflecting the subcortical component of the disease. Such neuropsychological data should be validated prospectively and further be part of the diagnostic criteria.

Aged↗

[A case of malignant glioma associated with verbal recent memory disturbance due to left hippocampal atrophy; case report].

Verbal recent memory disturbance was observed in a patient with a malignant glioma associated with left hippocampal atrophy. A 25 year-old male was admitted because of seizures. CT scan and MRI showed enhanced mass lesions in the left temporal lobe associated with ipsilateral hippocampal atrophy. Neurological examination disclosed right homonymous hemianopsia, word amnesia, alexia, agraphia and acalculia. Neuropsychological examination disclosed verbal recent memory disturbance, which consisted of impaired recall of the precisely memorized words after some interruption. Although hippocampal lesions are known to be often associated with cerebrovascular disease, hippocampal atrophy due to brain tumor is quite unusual. This case suggested that the left hippocampus is closely related to verbal recent memory. Hippocampal atrophy in this case conceivably derived from the decreased arterial flow due to perifocal edema or obstructive hydrocephalus.

Adult↗

Family observations of everyday cognitive impairment in dementia.

The observations made by family members of cognitive impairment and decline serve an important role in the description of dementia-related illnesses such as Alzheimer's disease. A common clinical practice is to interview the family and informally gather information about the severity and history of intellectual impairment such as memory disorder and aphasia. The present study introduces a method to formalize this information gathering using an inventory that allows a family member to rate the presence and severity of the everyday manifestations of dementia. Results of initial psychometric studies of the rating scale indicate that a family member can make reliable judgments of everyday cognitive impairment. Observér ratings such as these should significantly enhance diagnostic efforts when they are used in conjunction with neuropsychological tests and in cases in which the demented person cannot participate in a full neuropsychological evaluation.

Journal Article↗

Adult onset Niemann-Pick disease type C presenting with dementia and absent organomegaly.

A 39-year-old female presented to the Bryan Memory Disorders Clinic at Duke University with a 7-year history of an atypical progressive dementia, mildly impaired vertical gaze, dysarthria and mild ataxia. There was no evidence of organomegaly by clinical examination or by radionuclide liver/spleen scan. Brain biopsy disclosed a neuronal storage disorder characterized by ballooned neurons filled with oligo-lamellar cytosomes and lipid droplets. Cultured skin fibroblasts had diminished sphingomyelinase activity and impaired cholesterol esterification, although peripheral leukocyte sphingomyelinase activity was normal. Two years after biopsy, follow-up examination revealed marked progression of vertical gaze paralysis and ataxia. This case expands the clinical spectrum of Niemann-Pick disease type C by presenting in adulthood with subtle neurologic abnormalities; no visceromegaly and profound dementia.

Adult↗

[Alcohol dependence syndrome and before-discharge intervention method (BDIM)--Report 1. The process to develop the constructed BDIM].

Many alcoholics come to the hospital denying their own drinking problems. So, in the initial treatment stage for alcoholics, it is very important to bring patients' attention to their denial. In those days, there were many kinds of treatment method to help them aware of patients' denial. For example, psycho-educational therapy, Japanese Naikan therapy, attendance to self-help group meetings, and so on. But I don't think that these are effective enough to help them aware of their drinking problems, especially denial. The purpose of my study is to develop the therapeutic intervention method (Before discharge Intervention Method: BDIM). It is for being aware of patient's denial, stimulating his/her motivation for abstinence and attendance to medical follow-up sessions or self-help group meetings. To achieve these purposes, I apply Picard's initial intervention method that is a very useful therapy for alcohol dependence syndrome patients who reject consultation. The subjects of this study are 175 alcohol dependence syndrome inpatients and their family members. The period of BDIM practices is for about 1 week before discharge. BDIM's concrete programs are prepared by medical team under the therapist's guidance. Beforehand the therapist has to ask the patient whether he/she agree to practicing BDIM program or not. Then to obtain his/her family's approval of joining to BDIM, nurses talk by telephone or directly consult with the patient's family members. The therapists requires the patient's family members to write letters to him/her. In advance, the therapist has to take preliminary examination the letters not to be traumatic but spiritual. There are good memories about him/her and merits of the period without his/her drinking problems. Also, they write his/her drinking problems and their hope for abstinence and follow-up therapy after discharge and attendance of self-help group meetings. In BDIM session, the patients shall listen to his/her family members' messages by their letters with tears and receive their letters. Then the patient has an opportunity to talk his/her opinion about the letters and give his/her answer later. All the letters should be copied and kept with their medical file, because the therapists will talk about the letters with the patient and his/her family members in the follow-up session. Talking about letters after discharge helps him/her revive BDIM's memories and motivate abstinence, recovery and participation in self-help group. Even though alcohol dependence syndrome patient has some cognition and (or) memory disorders, I think that BDIM practices using some letters written by the patient and his/her family members are not only so useful for the patient to correct their cognition, but also helpful to be aware of denial and maintain motives to recovery. Owing to the deep emotion and warm heart from his/her family members, the patient can be aware of his/her family's sufferings and maintain abstinence for a long time.

Alcoholics Anonymous↗

Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Case report.

The authors report their encouraging experience using an endoscopic technique for transsphenoidal surgery in a patient with a large chordoma in the posterior fossa. The patient was a 40-year-old man with a 2-year history of progressive ataxia, a memory disorder, and emotional instability. A magnetic resonance (MR) image of the brain revealed a midline posterior fossa mass measuring 4 cm in diameter located between the clivus and the brainstem. The basilar artery and its bifurcation were encased by the tumor, which also distorted the brainstem. The patient had been treated at another hospital for obstructive hydrocephalus with a ventriculoperitoneal shunt and he received fractionated external-beam radiation treatment, although no histological diagnosis was ever made. The authors achieved a subtotal resection of the tumor through the patient's nostril using an endoscopic transsphenoidal technique. The portion of the tumor located behind the basilar artery was not resected to protect the brainstem perforating arteries. The patient showed dramatic improvement of his symptoms postoperatively. Residual tumor located behind the basilar artery was treated by stereotactic gamma knife surgery. This is the first reported case of a large posterior fossa chordoma treated by an endoscopic transsphenoidal technique.

Journal Article↗

[Rapidly progressing dementia as the presenting symptom of Waldenstrom's macroglobulinemia: findings from magnetic resonance imaging of the brain in Bing Neel syndrome].

CASE REPORT: A 68 year old male with no relevant clinical history was admitted to hospital because of symptoms of cognitive impairment (attentional deficit, short term memory disorders and behavioural disorders), accompanied by apraxia of gait and rectal and urinary incontinence. Results of a general clinical exploration were normal. In the MRI study of the brain numerous areas of hypersignal were observed in different arterial territories, which stood out in the T2, FLAIR and, above all, in diffusion weighted sequences. Analytical studies showed hypergammaglobulinemia with monoclonal IgM k and a bone marrow biopsy revealed infiltration by plasmatic cells. Bing Neel syndrome was diagnosed. CONCLUSIONS: We underline this exceptional form of presentation of Waldenstrom's macroglobulinemia and highlight the findings of diffusion weighted MRI, which suggested multiple infarcts for which a neoplastic vascular obstruction mechanism similar to that involved in malignant angioendotheliomatosis is posited.

Aged↗

Differential memory impairment in dementia with Lewy bodies and Alzheimer's disease.

This study was conducted in order to elucidate the functioning of the Central Executive System of Working Memory (WM) and to clarify the status of other cognitive functions in Dementia with Lewy bodies (DLB) and Alzheimer's disease (AD). Fourteen DLB, 22 AD, and 23 control subjects were assessed with the dual task paradigm and other cognitive tests. When compared with controls, DLB subjects performed more poorly in concurrent conditions on semantic WM tasks, and AD subjects performed more poorly on the spatial WM task. The DLB subjects had an inferior verbal span and AD subjects, an inferior recall on the CVLT. These data suggest relative impairments of verbal and semantic WM in DLB and relative impairments of spatial WM and verbal episodic memory in AD.

Aged↗

[Acute onset of somnolence and amnesia due to cerebral infarction of bilateral thalamus accompanied with tuberculous meningitis: a case report].

A 55-year-old man was admitted to our hospital because of somnolence and aspontaneity. He was hospitalized in the psychopathic ward under the initial diagnosis of depression. Chest X-ray showed infiltration in both upper lobe. Twelve days later, Mycobacterium tuberculosis was detected from his sputum and was confirmed by RT-PCR. Cerebrospinal fluid findings showed elevated ADA and mononuclear cells, suggesting the presence of tuberculous meningitis. However, the brain CT revealed no abnormal findings. By applying antituberculous treatment the pulmonary lesion improved but psychological symptoms remained. Three months later follow-up brain MRI was examined. Contrast enhanced granuloma was detected in the ambiens, suprasellar and quadrigeminal cisterns. A strong signal was seen in the left frontal thalamus and a weak enhanced lesion was detected in the right frontal thalamus on a T2 enhanced image. These lesions showed low intensity on a T1 enhanced image, suggesting cerebral infarction affecting the bilateral thalamus. Somnolence and memory disorder was due to cerebral infarction of the bilateral thalamus and tuberculous meningitis contributed to form the intracranial lesion. From the experience of this case, it is needed to consider cerebral infarction (especially the thalamus) due to tuberculous meningitis when we examine the patients with acute onset of psychological symptoms.

Amnesia↗

[European survey on current practice and disclosure of the diagnosis of Alzheimer's disease. A study based on caregiver's report].

UNLABELLED: The disclosure of a diagnosis of Alzheimer's disease is a complex and critical event. The aim of this study initiated by the Association Alzheimer-Europe was to explore 1) the context of the establishment of the diagnosis and the circumstances of its disclosure 2) the impact of the disclosure of the diagnosis on the patients and the caregivers 3) the amount and the adequacy of information provided by the physicians to the patients and the caregivers compared to their needs. Subjects. 323 caregivers were recruited through family associations in 11 European countries. METHODS: Data were collected using a structured questionnaire filled in by the caregivers. RESULTS: The caregivers' profile broadly was similar to that found in the published literature. The initial symptoms noted by the family members were memory disorders and behavioral manifestations, both in 50% of cases and isolated each in approximately 25%. Half of the carers reported a delay of one year or more between the initial symptoms and the first medical examination. The diagnosis was made mostly by neurologists, but also by psychiatrists, geriatricians, general practioners or neuropsychologists in various proportions according to the different countries. The diagnosis was disclosed in front of the patients only in approximately half of the cases. When the diagnosis was disclosed to the family alone, 56% of the carers did not want the diagnosis to be disclosed to the patients. Negative reactions of the carers to the disclosure of the diagnosis were clearly linked with the physician's attitude and time dedicated to information on the disease and its consequences. 43% of the carers were not satisfied by the information provided by the physicians and there was a discrepancy between the nature of the information provided by the physicians and the needs of the carers. Nearly one third of the patients had no regular follow-up after the diagnosis and 27% did not receive any treatment. CONCLUSION: This study shows important inadequacy in the management and counselling of patients with Alzheimer's disease and their relatives. Consideration should be given to developing a new philosophy of care based on a better understanding between health professionals, patients and families.

Aged↗

Complications of alcohol withdrawal: pathophysiological insights.

Disease processes or events that accompany acute alcohol withdrawal (AW) can cause significant illness and death. Some patients experience seizures, which may increase in severity with subsequent AW episodes. Another potential AW complication is delirium tremens, characterized by hallucinations, mental confusion, and disorientation. Cognitive impairment and delirium may lead to a chronic memory disorder (i.e., Wernicke-Korsakoff syndrome). Psychiatric problems associated with withdrawal include anxiety, depression, and sleep disturbance. In addition, alterations in physiology, mood, and behavior may persist after acute withdrawal has subsided, motivating relapse to heavy drinking. Recent advances in neurobiology may support the development of improved medications to decrease the risk of AW complications and support long-term sobriety.

Ethanol↗

[Efficacy of pronoran in age-related memory impairment].

Thirty patients aged 45-62 years (mean 54.8 +/- 3.7) have been studied. Pronoran (piribedil) was used for treatment of age-related memory impairment in dosage 50 mg per day during 30 and 90 days (2 groups of 15 patients). Psychophysiological examination included a study of sensomotor activity (time for visual-motor reactions--simple and complex) and functional lability of visual analyzer as well as evaluation of cognitive processes--memory and attention. Patients were examined before and after therapy of different duration. Pronoran exerted a positive effect on psychophysiological state of elderly people, improving memory and attention and increasing the velocity of psychomotor reactions and lability of nervous processes. The longer was the therapy, the more improved were above traits. Along with cogniotropic and mnemotoric (an influence on operative memory) effects of pronoran, its non-specific activating impact is described.

Age Factors↗

[A case of primary progressive multiple sclerosis with onset of memory impairment].

We report a 52-year-old woman with primary progressive multiple sclerosis (PPMS) presenting with chronic progressive memory impairment. From a couple of years prior to admission, she had developed impairment of her short-term memory. For example, she forgot her nephew's name, and spoke the same phrases again and again. She also sometimes forgot to turn off her gas stove and forgot things she bought in shops. Moreover, her mental activity gradually decreased and she became apathetic. However, she did not note her memory impairment, and had no hallucinations. She was admitted to our hospital on 20 May, 2003 because donepezil had been ineffective for treating her memory impairment. Neurologically, she showed bilateral horizontal gaze nystagmus, mild limb ataxia on the left and mildly ataxic gait. Neuropsychological examinations showed mildly impaired cognitive function, e.g., MMSE 25/30, WAIS-R full IQ 69 and especially in verbal short memory, which may represent temporal lobe dysfunction. Moreover, Benton's visual memory test revealed marked visual short-term memory impairment, while impaired performance on a Kana picking up test suggested mild to moderate attention impairment, which could have represented frontal lobe dysfunction. Brain MRI showed multiple T2-high plaque lesions close to the bilateral lateral ventricles, and bilateral optic nerve lesions enhanced by gadolinium. Also, spinal cord MRI showed a gadolinium enhanced lesion at Th5 on the left. Cerebral spinal fluid (CSF) examination showed normal cell count and protein level, and undetectable oligoclonal bands (OCB), but an elevated IgG index (1.1, normal < 0.85). Visual evoked potentials (VEPs) showed prolonged P100 latency bilaterally, indicating subclinical optic nerve lesions. She was thus diagnosed as having PPMS according to McDonald's diagnostic criteria for MS. 99mTc Single photon emission computed tomography (SPECT) showed a decreased cerebral blood flow (CBF) in the bilateral frontal and temporal lobes, which was consistent with her clinical features. PPMS patients generally present with chronic progressive spastic paraparesis and/or cerebellar ataxia. Cognitive impairments observed in PPMS are generally thought to be due to white matter lesions, i.e., subcortical dementia. However, some recent reports have shown MS patients with short-term memory impairment (antegrade amnesia) similar to cortical dementias such as Alzheimer's disease (AD). In such MS cases, visual short-term memory impairment seems characteristic of their cognitive impairment compared to AD cases. As well, the present case showed visual memory impairment as evaluated by Benton's memory test. Parietal and frontal lobes are reported to be important for verbal and visual working memory, respectively. Thus, in the present case, decreased CBF in the frontal and temporal lobes, which could have been due to a disconnection between cortices and subcortices caused by the white matter lesions, is consistent with the type of her cognitive dysfunction, i.e., notable visual memory impairment. PPMS may thus be an important disease as a differential diagnosis for chronic progressive dementia. Further neuropsychological and functional imaging studies will be necessary to achieve a better understanding of the mechanisms of cognitive impairment in PPMS.

Brain↗

[Memory changes in Parkinson's disease. Relation with clinical variables].

We studied verbal and visual short term memory, learning capabilities and long term retention in a sample of 96 patients with Parkinson's Disease (PD) and 42 controls matched by sex, age, years of education and verbal intelligence. We found significant differences between groups in visual short term memory and verbal learning, but not in verbal short term memory and long term retention. Performance in visual short term memory and learning correlated with the severity of disease and motor performance. Forty-one per cent of patients had impairment in visual short term memory, and this impairment is related with bradykinesia and correlated with age of onset. These results suggest that two forms of memory failure are closely related to motor symptoms and other clinical variables probably reflecting the same neuropathological substrate.

Aged↗

[Short term memory disturbances in paranoid schizophrenia--results of assessments with pictogram].

Short term memory (STM) was assessed in schizophrenic patients. 30 schizophrenic subjects and 30 healthy controls were examined with the pictogram test. The analysis of the results suggests that there is a disturbance in abstracting information in paranoid schizophrenia. Schizophrenic patients are not able to keep the limits of a concept of abstract information, while external information causes a fixation of the attention on stimuli, which are not important for the whole process. The process of decoding information does not proceed according to a certain recall strategy, but is determined by subjective importance of stimuli. The short term memory mechanisms are fixed by decreased control functions.

Adult↗