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Significant decreased insulin secretion in a diabetic patient with clinically probable multiple sclerosis.

A 39-year-old man with chief complaints of aphasia, disorientation and acalculia was admitted to our hospital. He was diagnosed as a clinically probable case of multiple sclerosis (MS) and his symptoms improved while on steroid pulse therapy. The patient had been diagnosed as having diabetes mellitus 16 years before the onset of MS and his insulin secretion further decreased with time. Slight insulin resistance was observed during a euglycemic hyperinsulinemic clamp study. These results suggested that this patient developed diabetes mellitus mainly due to the decrease of insulin secretion.

Adrenal Cortex Hormones↗

[A case report of valproate encephalopathy].

A 24-years-old woman with epilepsy treated with valproic acid developed disorientation, acalculia, perseveration, slow responsiveness and loss of memory. The drug withdrawal induced a quick improvement of mental function. In this case, hyperammonemia, hypocarnitinemia, increased protein in cerebrospinal fluid were found. When the drug was discontinued, she had a dramatic improvement. Valproic acid caused encephalopathy because of metabolic abnormality in mitochondria in this case. In the cases of encephalopathy caused by valproic acid, the drug administration should be withdrawn, and appropriate measures had to be taken. In addition, elevation of ammonia in the blood, lactic acid and pyruic acid in the serum and cerebrospinal fluid, and decreased carnitine level in the serum will be useful to make a diagnosis of valproate-induced encephalopathy.

Adult↗

[Lewy body dementia: some contributions to the clinical knowledge].

INTRODUCTION: Dementia with Lewy bodies is the second commonest type of dementia. The latest criteria for its diagnosis have recently been established (1996). PATIENTS AND METHODS: We report the clinical findings in 25 patients with probable dementia with Lewy bodies. RESULTS: No sex predominance was seen, onset was after the age of 65 years in 60% of the cases and there was a past history of dementia in 48%. Onset was with cognitive disturbances in 32%, Parkinsonism in 32% and both in 32%. When there was an interval between Parkinsonism and dementia, this was of 3-19 months. The dementia included attention disorder, impaired recall and praxis alterations. Disorders of executive function, behaviour or language, were seen in two thirds of the patients and acalculia in over half. Around 50% showed signs of subcortical involvement different from Parkinsonism. Parkinsonism was basically hypokinetic (96%), with tremor in two thirds of the cases, at rest (29%) postural (12%) or combined (59%), and symmetrical in 41%. Rigidity was only seen in 52% and was slight in most cases. In 16% there was weakness of vertical gaze. In 40% urine or double incontinence occurred, mainly when the dementia was at an intermediate or advanced state. CONCLUSIONS: Dementia with Lewy bodies is a dementia with fronto-temporo-parietal alterations, frequently associated with hypokinetic Parkinsonism (which always shows a lag period with respect to dementia of less than 24 months), hallucinations and a fluctuating course. Other findings supporting this diagnosis were seen in two out of every three patients.

Aged↗

[A case of crossed aphasia with echolalia after the resection of tumor in the right medial frontal lobe].

We report a right-handed woman, who developed a non-fluent aphasia after resection of astrocytoma (grade III) in the right medial frontal lobe. On admission to the rehabilitation department, neurological examination revealed mild left hemiparesis, hyperreflexia on the left side and grasp reflex on the left hand. Neuropsychologically she showed general inattention, non-fluent aphasia, acalculia, constructional disability, and mild buccofacial apraxia. No other apraxia, unilateral spatial neglect or extinction phenomena were observed. An MRI demonstrated resected areas in the right superior frontal gyrus, subcortical region in the right middle frontal gyrus, anterior part of the cingulate gyrus, a part of supplementary motor area. Surrounding area in the right frontal lobe showed diffuse signal change. She demonstrated non-fluent aprosodic speech with word finding difficulty. No phonemic paraphasia, or anarthria was observed. Auditory comprehension was fair with some difficulty in comprehending complex commands. Naming was good, but verbal fluency tests for a category or phonemic cuing was severely impaired. She could repeat words but not sentences. Reading comprehension was disturbed by semantic paralexia and writing words was poor for both Kana (syllabogram) and Kanji(logogram) characters. A significant feature of her speech was mitigated echolalia. In both free conversation and examination setting, she often repeated phrases spoken to her which she used to start her speech. In addition, she repeated words spoken to others which were totally irrelevant to her conversation. She was aware of her echoing, which always embarrassed her. She described her echolalic tendency as a great nuisance. However, once echoing being forbidden, she could not initiate her speech and made incorrect responses after long delay. Thus, her compulsive echolalia helped to start her speech. Only four patients with crossed aphasia demonstrated echolalia in the literature. They showed severe aphasia with markedly decreased speech and severe comprehension deficit. A patient with a similar lesion in the right medial frontal lobe had aspontaneity in general and language function per se could not be examined properly. Echolalia related to the medial frontal lesion in the language dominant hemisphere was described as a compulsive speech response, because some other 'echoing' phenomena or compulsive behavior were also observed in these patients. On the other hand, some patients with a large lesion in the right hemisphere tended to respond to stimuli directed to other patients, so called 'response-to-next-patient-stimulation'. This behavior was explained by disinhibited shift of attention or perseveration of the set. Both compulsive speech responses and 'response-to-next-patient-stimulation' like phenomena may have contributed to the echolalia phenomena of the present case.

Adult↗

[A case of Gogi aphasia with predominantly left temporal and parietal lobe atrophy].

We report a 68-year-old right-handed Japanese woman who had a history of progressive difficulty in understanding speech and naming. Neuropsychological examination presented Gogi (word meaning) aphasia and impairment of semantic memory for some common objects. She also presented acalculia and mild constructional impairment. There was no evidence of impairment in elementary perception and motor skills. Her memory performance of visual task was within normal range. She had neither personality change nor behavioral disorder. Magnetic resonance (MR) imaging showed atrophy in the left temporal lobe and the left parietal lobe. Single photon emission computed tomography (SPECT) scans demonstrated a decrease of regional cerebral blood flow in the atrophic sites and the left frontal lobe. We pointed out that the atrophy of the parietal lobe was atypical in the early stage of cortical degenerative disease presenting Gogi aphasia, in addition to in the light of classification of Fronto-Temporal Lobar Degeneration (FTLD).

Aged↗

[Non-persistent "doll phenomenon" in a patient with right thalamic infarction].

An 81-year-old right-handed woman was admitted because of acute dysarthria and left hemiparesis. She had lived herself without aids until the admission. On neurological examination she was confused and disoriented. She was ambulant, but had mild dysarthria and mild left hemiparesis. Neuropsychological tests showed severe impairment of memory, mild impairment of visual cognition, decreased fluency of word recall and mild paramnesia, but no acalculia, agraphia, aphasia or apraxia. MRI of the brain showed small infarction in the right anterior thalamus. 123I-IMP SPECT demonstrated a decrease in CBF of the thalamus, basal ganglia and frontal lobe on the right. During admission, she always played with a doll as if she took it as a real baby. This peculiar symptom. "doll phenomenon" continued for approximately three months later. The "doll phenomenon" usually appears in demented patients with diffuse mental deterioration or dysfunction of the frontal lobe. The present patient had not been demented until the onset of the thalamic infarction, and disturbance of cognition caused by the right thalamic infarction probably produced the "doll phenomenon".

Aged↗

[Clinical analysis of 7 patients with Gerstmann syndrome secondary to cerebral vascular disease].

OBJECTIVE: To analyze clinical features of patients with Gerstmann syndrome (GS). METHODS: We retrospectively analysed the clinical manifestations of 7 patients (6 men and 1 woman) with GS secondary to cerebral vascular diseases and reviewed the literatures. RESULTS: The age ranged from 51 to 70 years with a mean of 70 years. They all had sudden onset and the tetrad of GS-finger agnosia, left-right disorientation, agraphia and acalculia, 3 patients accompanied by incomplete aphasia, 3 by anomic aphasia, 2 by alexia and 1 by constructional apraxia. Cranial computed tomographic scan showed low-density focus of the left parietal lobe in 6 cases and high-density focus of the left parietal lobe in 1 case. CONCLUSION: GS has the high value in localization and the lesion is mainly localized to angular gyrus of the dominant hemisphere.

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↗

Progressive supranuclear palsy with widespread cerebral lesions.

A 51-year-old woman with no history of any familial neurological diseases initially presented with numbness in her extremities, slowing of movements, comprehension deficit, memory disturbance, dyscalculia, muscle rigidity, hyperreflexia, Parkinsonian gait, increasing disorientation, left-right disturbance, finger agnosia, alexia, acalculia, apraxia, aspontaneity, euphoria, gait disturbance, aphasia, echolalia, and in the terminal stage, mutism, contracture of lower extremities and cachexia. She died of bronchopneumonia at the age of 55. The brain showed widespread cerebral lesions, consisting of nerve cell loss and neurofibrillary tangles in the frontal, parietal and occipital cortex, demyelination and gliosis in the frontal, parietal and occipital subcortical white matter in addition to the typical pathological findings of progressive supranuclear palsy (PSP): severe neuronal loss with gliosis and neurofibrillary tangles (NFTs) in the subthalamic nucleus, globus pallidus and substantia nigra. In conclusion, we present a case of PSP with unusual clinical features (extrapyramidal signs, frontal and parietal lobe syndromes without ophthalmoplegia) and neuropathologically widespread cerebral lesions in addition to the typical pathological findings of PSP. The differential diagnosis of PSP and Alzheimer's disease and other degenerative disorders is discussed.

Alzheimer Disease↗

[Multiple brain infarctions in a young patient with Buerger's disease. A case report of cerebral thromboangiitis obliterans].

We report a 46-year-old woman with Buerger's disease who presented vascular dementia. In her early thirties, she began to feel cold sensation and pain in the lower extremities and later developed Raynaud's phenomenon in the upper extremities. The diagnosis of Buerger's disease was established on the basis of angiographic findings which showed the obstruction of peripheral vessels in both anterior tibial arteries. She was admitted to our hospital because of a transient attack of left brachial monoparesis and two episodes of epileptic seizure with progressive cognitive impairment for preceding five years. Neurological examination revealed acalculia, constructional apraxia, recent memory disturbance, and hyperreflexia of the left limbs without motor disturbance. Brain MRI revealed multiple infarctions mainly located in the border zone territories of the major cerebral arteries. Conventional angiography failed to detect abnormalities of cerebral vessels. She had no cardiovascular abnormality or coagulopathy. Cerebrovascular complications, so-called cerebral thoromboangiitis obliterans (CTAO), occur approximately in 2% of patients with Buerger's disease. CTAO has two types. Type 1 is associated with large artery changes and type 2 is associated with medium and small artery changes. Patients with type 2 CTAO usually have multiple infarcts in the cerebral arterial border zones and may develop progressive cognitive decline without motor deficit. Her clinical features are compatible with type 2 CTAO. While CTAO is a relatively rare manifestation of Buerger's disease, it should be suspected in cases of Buerger's disease in association with cognitive impairment of unknown onset.

Cerebral Infarction↗

[Hashimoto's encephalopathy associated with scleromyxedema: coincidental or the overlapping of two syndromes with a possible autoimmune origin?].

INTRODUCTION: The encephalopathies that may accompany Hashimoto's thyroiditis and scleromyxedema, both of which are diseases that probably have an autoimmune origin, are clinically similar. The presence of both Hashimoto's thyroiditis and scleromyxedema in a patient with encephalopathy led us to compare the clinical symptoms and the different possible mechanisms accounting for the pathology. CASE REPORT: We describe the case of a 54-year-old male who was admitted to hospital because of several occurrences of transient neurological focus that finally developed into a clinical picture of aphasia, acalculia and right homonymous hemianopsia. The patient had previously been diagnosed as suffering from scleromyxedema, which responded poorly to treatment, as well as IgG kappa paraproteinemia and, six months before admission to hospital, Hashimoto's thyroiditis. No abnormalities were found in the complementary tests, except for slightly high protein levels in the cerebrospinal fluid, a diffuse slowing of brain waves in the electroencephalogram and alterations due to thyroiditis and paraproteinemia. Neurological symptoms improved after a few days of corticoid therapy. CONCLUSIONS: The presentation of encephalopathy as a stroke and its excellent response to treatment with corticoids pointed to a case of Hashimoto's encephalopathy, although we cannot completely rule out the possibility of it being a dermato-neuro syndrome, which is associated to scleromyxedema. In this paper we review the different pathological mechanisms proposed for the two clinical entities, as well as the clinical features that are similar in both syndromes and which could be an indication of some common mechanism shared by them.

Adrenal Cortex Hormones↗

[Pure alexia secondary to progressive multifocal leukoencephalopathy as first manifestation of HIV infection].

INTRODUCTION: Pure alexia, or alexia without agraphia, is usually caused by disconnection between the gyrus angularis and visual areas of both hemispheres. Cerebrovascular disease is the most frequent cause of this syndrome. CLINICAL CASE: We report the first case of a patient with pure alexia secondary to progressive multifocal leukoencephalopathy (PML) as the first manifestation of Human Immunodeficiency Virus (HIV) infection. Polymerase chain reaction in cerebrospinal fluid was positive for JC virus. Despite favorable immunological and virological response to highly active antiretroviral therapy, neurological symptoms and demyelinating lesions progressed during the first months, and right homonyous hemianopsia, acalculia, and color anomia were noted. In magnetic resonance imaging (MRI) demyelinating lesions involved white matter of both occipital lobes, with left predominance, forceps major and splenium of the corpus callosum, together with white matter of the left temporal lobe and both hippocampal regions. During the following months, coinciding cidofovir treatment, the patient had partial clinical improvement, but neurological deficits persisted. The size of MRI lesions significantly improved. CONCLUSIONS: PML is frequently the first manifestation of AIDS (acquired immunodeficiency syndrome). It should be considered as a possible diagnosis of pure alexia.

Alexia, Pure↗

[Korsakoff's syndrome secondary to left thalamic bleeding].

Korsakoff's syndrome is characterised by an important impairment in memory in comparison to other cognitive functions. Presence of confabulations is frequent but they are not characteristic of this syndrome. Alcohol abuse is the main cause of this syndrome but it also has been associated to other causes, like malnutrition or structural lesions. Although the role of thalamus in memory processing is well established, the development of confabulations in the setting of a thalamic lesion is poorly described in the literature. We present a 61 year-old hypertensive man, with an important impairment in anterograde memory, confabulations and acalculia after a left thalamic haemorrhage. Other cognitive functions were intact except a partial temporal disorientation. Thalamic dementia is generally caused by bilateral thalamic damage. Usually, all cognitive functions are impaired. As thalamus takes part in Papez's circuit, either thalamic nuclei or their connections lesions may originate memory impairment. However, although a relation between the presence of confabulations and connections from thalamus to frontal lobe lesions has been described, there is almost no evidence in the literature.

Cerebral Hemorrhage↗

[Multiple sclerosis with higher cerebral dysfunction: a case report].

Higher cerebral dysfunctions such as aphasia, apraxia and agnosia have seldom been reported in multiple sclerosis (MS). 12 year-old right-handed boy felt unsteadiness of the body and headache for several days. Two months later, he had the same episode and complained of visual disturbance, and weakness and sensory disturbance on the face and the extremities. Additionally, he showed amnestic aphasia, acalculia, ideomotor apraxia, finger agnosia and right-left disorientation. Cerebrospinal fluid examinations revealed increases IgG, myelin basic protein and neuron specific enolase (11%, 25 ng/ml and 28.8 ng/ml, respectively). X-ray CT scan and MRI-CT examinations revealed sclerotic lesions on the left parietal white matter and the right mid-brain. The diagnosis was made as MS. He was treated with m-PSL (methyl-prednisolone) pulse therapy for three weeks and consecutively treated with PSL for four weeks. He recovered gradually, but visual disturbance and facial palsy remained. After seven months MRI-CT showed a high signal intensity on the left parietal white matter in spite of the disappearance of the lesion on X-ray CT scan. We suggest that these higher cerebral dysfunctions may result from the lesion of the left parietal white matter which produces a disconnection between each cortical area.

Agnosia↗

[Conduction aphasia. Neuropsychologic study of a patient].

We report a patient with clinical features consistent with conduction aphasia. The patient had dilated cardiomyopathy, and as a complication, had cerebral infarction one year before neuropsychiatric evaluation. His language was fluid, paraphasic, with a "phonetic approximation behavior" in an attempt to pronounce the adequate word. Comprenssion was normal and repetition difficult, with numerous paraphasias. Number repetition was particularly affected. Reading and writing had the same features as spontaneous language. In association with the language disorder, he had "aphasic" acalculia, but other signs of left parietal involvement were absent. Magnetic resonance images showed a low parietal small chronic ischemic area, which also involved the left infrasilvian region, both in the cortex and in the white matter, presumably in the arcuate bundle. The differential diagnostic difficulties of this condition are discussed from a behavioral standpoint.

Aphasia↗

Primary thalamic malignant fibrous histiocytoma of the dominant hemisphere causing severe neuropsychological symptoms.

A 45-year-old man with a malignant fibrous histiocytoma (MFH) of the left thalamus is reported. This tumor only rarely originates within the brain. Primarily intracerebral MFH usually has a superficial localization and rarely involves the deep cerebral structures. Our patient presented with complex neuropsychological symptoms, including aphasia, hemispatial neglect and acalculia. These findings are further supporting evidence for the role of the thalamus, particularly of its posterior parts, in language, speech and other neuropsychological functions.

Anomia↗

[Multi-infarct dementia clinically simulating dementia of Alzheimer type. A comparison with angular gyrus syndrome].

A 74-year-old right-handed man with multiple cerebral infarction who presented with dementia simulating dementia of Alzheimer type (DAT) is reported. He had been well until April 20, 1987 when he developed transient right hand palsy lasting overnight. Eleven days later, he became confused, disorientated, and amnestic. He was admitted to this hospital on June 8. Physical examination revealed hypertension (170/90mmHg). On neurological examination, his consciousness was clear but he was demented. He showed disorientation, amnesia, and urinary incontinence. His most prominent symptom was disturbance of speech, including fluent aphasia and alexia with agraphia. Additionally, he showed ideomotor apraxia, construction apraxia, right-left agnosia, finger agnosia, and acalculia. On July 9, he had a transient attack of right hemiplegia with confusion. The brain CT scan performed on admission was unremarkable except for cavum septi pellucidum and a small low density area in the right basal ganglia. However, single photon emission computed tomography (SPECT) by 123I-labeled N-isopropyl-p-iodoamphetamine disclosed hypoperfusion of the cerebral blood flow in the border zones of the temporoparietal and frontal lobes on the left. A follow-up brain CT scan taken one month later demonstrated low density in the new areas corresponding to hypoperfusion shown by SPECT. Although the clinical features of the present case resembled those of DAT, dementia in this case was regarded as the result of multiple cerebral infarction since it occurred acutely with mild motor deficits, and brain CT scans and SPECT showed lesions indicating focal cerebral ischemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Isolation of HIV in a seronegative demented patient without symptoms of immune deficiency.

A 60-year-old male patient, originating from West Africa, developed acute and regressive neurologic symptoms associated with aphasia, apraxia, acalculia, behavioral impairments, and an epileptic phase. Eighteen months after the onset of the disease, the patient was almost normal. All along the clinical course, biological abnormality patterns were minor. We noted only a mild neutropenia in the blood. We also observed a weak lymphocytosis and elevated protein content in the cerebrospinal fluid. Electroencephalogram examination revealed slow waves which disappeared after remission. A weak ventricular dilatation was detected on CT scan. Neither vascular, nor tumoral, nor a classical infectious origin could be identified. While the patient was seronegative to HIV, a HIV-like virus was isolated twice from his peripheral blood lymphocytes during the disease. Eighteen months later, the patient remained seronegative. He developed neither AIDS nor immunodeficiency. The subtype of HIV has been isolated and characterized, and its neurotropism is being investigated.

Antibodies, Monoclonal↗