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PubMed2000

[Progressive limb-kinetic apraxia with myoclonus focal atrophy in the postcentral gyrus and the supplementary motor area].

We report a 70-year-old right handed man with a 5-year history of slowly progressive clumsiness of his left hand. A neurological examination disclosed mild rigidity and myoclonus in his left hand. He showed limb-kinetic apraxia, but neither ideational apraxia nor ideomotor apraxia was present. Aphasia and agnosia were also absent. He was thought to have the primary progressive limb-kinetic apraxia clinically. Brain CT and MRI revealed focal atrophy in the right postcentral gyrus and the supplementary motor area. A positron emission tomography (PET) study showed diffuse decrement of cerebral blood flow, predominantly in the right hemisphere. The decrease in the uptake of [18F]-Fluoro-deoxyglucose also revealed glucose hypo-metabolism, especially in the right frontal and parietal lobe. Striatal [11C]NMSP and [18F]FDOPA uptake were also reduced in an asymmetric pattern. These findings suggest that our patient is likely to have corticobasal degeneration. Transcranial magnetic stimulation using double pulse paradigm revealed a decrease in the level of cortico-cortical inhibition in the motor cortex on the affected side. Our results indicate increase in the excitability of motor cortical neurons in primary progressive limb-kinetic apraxia, likely due to a decreased excitability of cortico-cortical inhibitory mechanism as a result of focal degeneration of cortical neurons.

Aged↗
PubMed1999

[The significance of the study of aphasia, apraxia and agnosia at present time].

Neuropsychology initiated by the study of aphasia, apraxia and agnosia had been regarded as one division of psychiatry. This is one of the reasons why neuropsychology is minor territory in neurology. Some people say that neuropsychology seems difficult to understand, not scientific, not useful and is rather a man's taste than a medicine. I try to respond in this paper to these critics by way of analyzing our own cases while insisting on the today's importance of the study of aphasia, apraxia and agnosia. Our personal cases consist of the following three categories. 1) Primary progressive apraxia: proposal of the symptomatic concept suggested by the investigation of our own eight cases and those in the literature. 2) Musical alexia with agraphia in a trombone player: study of the cerebral localization in a person with special ability. 3) Disturbance of reaching proposal of intracerebral pathways in kinesthesia. Therefore it is highly possible that the significance of the study of aphasia, apraxia and agnosia should become enlarged now and after; and it seems urgent to answer its need that younger neurologists participate in this area.

Aged↗
PubMed2000

[Neuropsychological exploration in frontotemporal degeneration].

The present paper discusses the neuropsychological assessment in fronto-temporal lobe degeneration. Having established the neuroanatomical and functional basis for the discussion the major syndromes included in the concept of frontotemporal degeneration are reviewed from a neuropsychological standpoint. With reference to fronto-temporal dementia the different frontal or executive function tests and their limitations are discussed. With reference to progressive aphasia and semantic dementia we differentiate the distinct language profiles as observed in aphasia batteries and general neuropsychological tests. Reference is made to especially useful tests for the differentiation of the two syndromes from each other, as well as from other primary progressive disorders. Concluding remarks postulate a series of axis of cognitive function in fronto-temporal lobe degenerations, which exist at the functional as well as the anatomical level and along which the different syndromes evolve.

Aphasia↗
PubMed2005

Primary intracranial dural lymphoma of mucosa-associated lymphoid tissue (MALT) type: report of one case and review of the literature.

Primary indolent leptomeningeal lymphoma is a rare entity, and corresponds in most cases to mucosa-associated lymphoid tissue (MALT) type lymphoma. We are reporting a case of a 75 years old woman, who presented with a 2-year history of behavioral disorder, progressive memory loss and aphasia. Neuroimaging showed a mass infiltrating the frontal circumvolutions and the roof of the orbit. The biopsy revealed an infiltration of the dura by an indolent lymphoma, characteristic of a MALT-type lymphoma. Complete staging work-up did not show any evidence of systemic involvement. A treatment with systemic methotrexate, combined with intrathecal chemotherapy and followed by radiotherapy (30,6 Gy) of the primary site, was conducted. The 3-year follow-up confirms the persistent remission, the patient remaining well and free of symptoms. The review of the literature highlights the importance to recognize the indolent PLML as a distinct clinical entity, which exhibits a rather good prognosis following a relatively non-toxic therapy.

Aged↗
PubMed1994

[Prefrontal dementias: clinical features and SPECT in 6 cases].

Six patients with primary degenerative dementias and frontal or frontotemporal hypoperfusion are reported. Five were diagnosed as Frontal Progressive Dementia (FPD), characterized by striking changes in personality and social behavior. The last patient had a progressive aphasia and a moderate cognitive impairment. We discuss the heterogeneity of FPD that includes Pick's disease, frontal lobe type dementia (Neary) and progressive subcortical gliosis. The usefulness of SPECT and the need of a brain bank are remarked.

Aged↗
PubMed2000

Rapidly progressive aphasia and motor neuron disease: a clinical, radiological, and pathological study of an autopsy case with circumscribed lobar atrophy.

This report concerns an autopsy case of rapidly progressive aphasia and motor neuron disease. The patient was a Japanese woman who was 75 years old at the time of death. The family history did not reveal hereditary burden. She developed language disturbances and difficulty in swallowing at age 74. Neurological examination 1 month after the disease onset revealed motor aphasia without dementia and bulbar sign, followed by muscle weakness of the four extremities. Neuroradiological examination revealed progressive atrophy of the anterior part of the left temporal lobe. She died of respiratory difficulty 10 months after the disease onset. Macroscopically, neuropathological examination showed circumscribed atrophy of the left perisylvian region and, histologically, neuronal loss in the cerebral cortex, including the primary motor area, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to obvious degeneration of the pyramidal tracts and presence of Bunina bodies. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells and frontotemporal cortical layer II neurons. Based on these clinicopathological findings and a review of the literature, we concluded that our case is the first reported case of amyotrophic lateral sclerosis with dementia that clinically showed rapidly progressive aphasia.

Aged↗
PubMed2006

Herpes encephalitis presenting as mild aphasia: case report.

BACKGROUND: Encephalitis presenting as a change in mental status can be challenging to recognize in the primary care setting. However, early detection via a low threshold of suspicion can be useful, leading in turn to early treatment and improved survival. CASE PRESENTATION: We present a case which we consider relevant to primary care practitioners. The patient in question presented with relatively mild mental status changes, progressing to confusion, dysnomia and delirium over a period of three days. While infection did not appear to be the leading cause on her differential diagnosis, she was found on extensive workup to have encephalitis caused by Herpes Simplex Virus type 1. CONCLUSION: The case is instructive for general practitioners and other clinicians to maintain vigilance for central nervous system (CNS) infections which may present atypically.

Acyclovir↗
PubMedDate not supplied

[Diagnostic criteria for fronto-temporal lobe degeneration].

Circumscribed atrophy of the frontal and temporal lobes (frontotemporal lobar degeneration) accounts for about one fifth of cases of primary degenerative dementia occurring before the age of 65. It produces three prototypical clinical syndromes. The most common is frontotemporal dementia, characterized by personality change and profound alteration in social conduct and associated with bilateral atrophy of the frontal and anterior temporal lobes. Progressive non-fluent aphasia is characterized by difficulty in verbal expression, anomia and phonemic errors in the presence of relative preservation of comprehension and associated with atrophy predominantly of the left hemisphere. In semantic dementia there is fluent speech with semantic errors and severely impaired comprehension and naming, together with a visual associative agnosia, resulting from bilateral atrophy of the inferior and middle temporal gyri. The clinical syndromes occur with either of two main histological types: prominent microvacuolar change, without specific histological features (frontal lobe degeneration-type), severe astrocytic gliosis with or without ballooned cells and inclusion bodies (Pick-type). To improve clinical recognition and advance understanding of this relatively common form of cerebral degeneration, members of an international workshop on Frontotemporal Lobar Degeneration developed consensus criteria, building upon earlier published clinical diagnostic guidelines for frontotemporal dementia. The consensus criteria reported here specify core and supportive features for each of the prototypical clinical syndromes: frontotemporal dementia, progressive aphasia and semantic dementia, as well as providing broad inclusion and exclusion criteria for the generic entity of frontotemporal lobar degeneration.

Aged↗
PubMed1995

[Frontal lobe-type dementia associated with motor neuron disease].

A case of presenile dementia with dominant frontal disfunction, progressive aphasia and Motor Neuron Disease with prominent bulbar signs is reported. Considering the clinical examination, the measurements of the regional cerebral flow (SPECT) and the histological appearances, we suggest the diagnosis of Dementia of Frontal Lobe Type and Motor Neuron Disease. We reviewed other disorders labelled Primary Frontal or Fronto-temporal Dementias and we discuss this new dementia and the difficulty in its classification.

Biopsy↗
PubMed2001

The Landau-Kleffner Syndrome.

Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalograph (EEG) activity. LKS appears to share a common pathophysiologic mechanism with continuous spike-wave of sleep (CSWS), acquired epileptic opercular syndrome (AEOS), and even benign childhood epilepsy with centrotemporal spikes (BECTS), with differentiating factors including age of onset, area of primary epileptogenicity, and severity of clinical presentation. This article covers the clinical, diagnostic, therapeutic, and prognostic features of LKS. In a child with autistic spectrum disorder, the presence of a fluctuating clinical course or regression should raise suspicion for the presence of associated epilepsy.

Journal Article↗
PubMed2004

[Semantic dementia: clinical profile and cognitive-neuropsychological relevance].

Semantic Dementia (SD) represents a rare variant of presenile primary degenerative diseases of "frontotemporal lobar degenerations" (FTLD). SD is characterized by anterior-temporal atrophy and a selective semantic deficit. Neuropsychological impairment either bears on fluent aphasia with marked naming and comprehension difficulties or on a disorder of object recognition or on both. Behavioural alterations such as stereotyped behaviour and change in food preference become prominent as the disease progresses and are interpreted as a correlative for the pathological spreading to frontal structures. Since SD is often misclassified as Alzheimer's disease (AD), but calls for a different management, diagnostic consensus criteria by Neary and colleagues are depicted. The neuropsychological profile is described in detail and complemented by a case report. Implications from the dissociation of disturbed memory systems of SD (semantic) and AD (episodic) for models of long-term memory are discussed in terms of connectivity in the medial temporal lobe. SD can contribute to a better understanding of the structure and organisation of the semantic system.

Cognition↗
PubMed1998

Primary stenting of the extracranial internal carotid artery in a patient with multiple cervical dissections: technical case report.

OBJECTIVE AND IMPORTANCE: Spontaneous dissection of the extracranial internal carotid artery (ICA) and vertebral artery (VA) is a well-documented cause of stroke in young, previously healthy patients. The majority of patients with spontaneous dissection are successfully treated with antiplatelet or anticoagulation therapy, but a significant proportion of patients progress to suffer devastating morbidity and mortality. Surgical intervention has primarily consisted of proximal ligation, extracranial-intracranial bypass, or endarterectomy. Generally, these procedures are technically demanding and yield disappointing clinical results. CLINICAL PRESENTATION/INTERVENTION: A 36-year-old man without a significant medical history initially presented with a several-day history of episodic right upper extremity weakness and numbness and visual obscurations. Cerebral angiography revealed bilateral ICA long segment narrowing (95%), distal left VA high-grade (95%) stenosis compatible with dissections, and right VA proximal occlusion. While therapeutically anticoagulated on heparin, the patient continued to experience crescendo episodes of right upper extremity paresis and paresthesias as well as aphasia. The patient underwent primary stenting of the left ICA, using a series of six overlapping stents (three Gianturco-Roubin coronary stents and three Palmaz-Schatz coronary stents). The patient remained symptom-free without neurological complications, and subsequent angiography performed at the 9-month follow-up examination confirmed continued patency of the stented left ICA as well as recanalization of the right ICA and VA. CONCLUSION: Neurovascular stents offer a minimally invasive and potentially efficacious treatment for the prevention of cerebral ischemia in patients with spontaneous extracranial dissection who remain symptomatic despite therapeutic anticoagulation.

Adult↗
PubMed1992

[Dementia disclosing primary Gougerot-Sjögren syndrome].

Two cases of primary Sjögren's syndrome revealed by dementia are reported. The patients had progressive or subacute memory dysfunction and psychiatric disorders with depression and delirium. The diagnosis of Sjögren's syndrome was established by biopsy of the minor salivary glands. Both patients were treated with corticosteroids. The neuropsychiatric symptoms improved dramatically in one case and remained unchanged in the other case. Dementia in Sjögren's syndrome seems to be without aphasia, apraxia or agnosia, and associated with psychiatric features, particularly depressive symptoms, thus including some characteristics of subcortical dementia. Diagnosis may be difficult because, as shown in our cases, symptoms of ocular and buccal dryness can be absent. Salivary gland biopsy can be useful in the evaluation of patients with dementia of undetermined etiology.

Biopsy↗
PubMed2003

["Progressive acalculia": a variety of focal degenerative atrophy affecting number processing].

A 72-year-old man experienced increasing difficulties in calculating and processing numbers. He made many syntactic errors when he read and wrote numbers, both in arabic and number-word forms. Transcoding between arabic numerals and number-words was severely impaired. His calculation abilities were grossly impaired. He could not perform even simple multiplications, but additions and subtractions were preserved. He was unable to recite multiplication tables or the alphabet. In contrast, numerosity judgment, magnitude comparisons,and semantic evaluation of numbers were intact. The patient's deficit could be summarized as an Impairment in all tasks involving numbers in a verbal format, corresponding to the so-called "verbal anarithmetia'." It was noteworthy that verification of operations, including multiplications, was accurately performed. For example, the patient could not solve a multiplication like 8 x 9, but could estimate that 9 x 2=1 1 was closer to the exact result than 9 X 2=17, or could state that 8 x 2=16 was exact even though, at the same time, he expressed verbally the terms of the corresponding addition "eight plus two is ten." Other cognitive deficits were detected by neuropsychological tests which demonstrated mainly anomic aphasia, dysorthographic agraphia without alexia, impairment of short term and episodic memory, digital agnosia. However, the patient could not be considered to have demon-eti due to preserved self-sufficiency, except for tasks requiring calculation abilities. During the two years of follow-up, the impairment in processing numbers and calculation remained predominant over other deficits. Cerebral MRI showed an atrophy of the left temporal and parietal lobes, and SPECT study showed a reduction of regional cerebral blood flow of the left hemisphere. This original clinical presentation of a cerebral degenerative disease could be described as "primary progressive acalculia" whatever the underlying primary pathological process.

Aged↗
PubMed1977

Pure word deafness. (Auditory verbal agnosia).

The selective inability to comprehend the spoken word, in the absence of aphasia or defective or defective hearing, is defined as pure word deafness (auditory verbal agnosia). Reported cases of this rare disorder have suggested the site of involvement to be strategically placed, interrupting fibers from left and right primary auditory receptive areas which project to Wernicke's are in the dominant hemisphere. Our patient is a 44-year-old male who suffered from an uncertain illness complicated by fever, jaundice and generalized seizures seven years previously. Following an apparent convulsion, the patient was noted to be unable to understand spoken language without loss of ability to recognize and respond to sounds or marked impairment of speech or reading. The evidence suggested bilateral cerebral hemisphere disease more marked on the right. The abrupt onset without progression is consistent with a vascular or ischemic etiology. Conclusions about the nature of the lesion and areas involved must await further studies and ultimately tissue examination.

Adult↗
PubMed1993

Focal status epilepticus and epilepsia partialis continua in adults and children.

Focal status epilepticus and epilepsia partialis continua (FSE-EPC) are most frequently seen with chronic focal progressive encephalitis of Rasmussen and Russian spring-summer encephalitis. FSE-EPC may be the presenting feature of nonketotic hyperglycemic diabetes mellitus but is more often noted as a late complication especially if there is a coexistent cerebral lesion such as cerebral infarction. FSE-EPC may be related to multiple sclerosis, primary or metastatic brain tumors, the MERRF-MELAS syndrome, benign epilepsy of childhood with rolandic spikes, and in some adults with acquired aphasia. The physiological origin of the myoclonic jerks seen in EPC is cortical and may be either spontaneous or provoked by the joint position of the affected limb. The treatment of FSE-EPC is influenced by the underlying disorder.

Adult↗
PubMed1998

The initial recognition and diagnosis of dementia.

Dementia is characterized by a decline in cognition, behavioral disturbances, and interference with daily functioning and independence. Diagnosis is sometimes delayed as patients or family members often misattribute obvious manifestations of cognitive decline to normal aging rather than to the onset of a degenerative disease. Many physicians do not perform mental status examinations or do not use them effectively to detect early symptoms. Clinical markers are available to decrease the difficulty in distinguishing dementia from depression and confusional states such as delirium. Alzheimer's disease (AD) is the most common form of dementia; others include rapidly progressive dementias, dementias associated with strokes and Parkinson's disease, and frontotemporal dementias. Often, AD coexists with other forms of dementia. Sensitivity to early warning signs, interviews with family members, and mental status examinations are essential to early detection of AD, and will prove useful to primary-care physicians who care for older patients.

Alzheimer Disease↗