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A serial study of changes in intrathecal immunoglobulin synthesis in a patient with central nervous system systemic lupus erythematosus.

A 50-year-old man with systemic lupus erythematosus developed organic brain syndrome. He responded to corticosteroid therapy and recovered completely from acalculia, apraxia and memory disturbance. Throughout his course, the cerebrospinal fluid (CSF) IgM, IgA and IgG indices were decreased in relation to the progression of normal alpha activity in the electroencephalogram. CSF Ig indices may be useful for monitoring central nervous system lupus disease activity.

Humans↗

[A case of multiple anomalies of cerebral vessels--fenestration of the middle cerebral artery aneurysm of the anterior communicating artery and arteriovenous malformation on the frontopolar region (author's transl)].

A case associated with multiple cerebral vascular anomalies, which consisted of fenestration of the middle cerebral artery, arteriovenous malformation and aneurysm of the anterior communicating artery, was reported. A 48 year-old male has been suffering from the left paralysis and mental disorder after the initial attack of subarachnoid hemorrhage, and the second attack resulted in the deterioration of the symptoms. He was admitted to our clinic on October 28, 1974. On neurological examination, mental disorders, such as disorientation, emotional incontinence, amnesia and acalculia, hemiplegia on the left and meningeal irritation signs were observed in admission period. Physical examination was negative. Cerebral angiographic findings were as follows: 1) Moderate vasospasm of the right internal carotid artery at the terminal segment, mild bowing of the anterior cerebral artery and stretching of the frontparietal opercular branches of the middle cerebral artery were observed. 2) Right frontpolar arteriovenous malformation fed by the frontobasal artery and the frontopolar artery, and drained via the aberrant cortical vein into the superior sagittal sinus. 3) Aneurysm of the anterior communicating artery was opacified by left carotid angiography. 4) An abnormal vessel derived from the terminal segment of the right internal carotid artery and terminated at the portion of the sphenoidal segment of the middle cerebral artery. Complete loop was formed between genuine middle cerebral artery and this abnormal artery. He was operated with dissecting microscope on November 11, 1974. The arteriovenous malformation at right frontopolar region was totally removed and aneurysm of the anterior communicating artery was clipped. According to the operative findings, the arachnoid membrane over the right frontopolar region was turbid and adhered to the adjacent tissues. On the contrary, no abnormal findings suggestive of previous subarachnoid hemorrhage were observed around the region of the anterior communicating artery aneurysm. These findings showed that subarachnoidal bleeding was caused by rupture of the arteriovenous malformation of right frontopolar region, but not by the aneurysm on the anterior communicating artery. The postoperative course was uneventful and during the hospitalization the patient starts on rehabilitation therapy. The authors discussed the genesis of fenestration of the middle cerebral artery and relation among these combined vascular anomalies. We inferred that fenestration of the middle cerebral artery arose from the in complete fusion of procursor vascular network in embryonic stage. Additionally, we emphasized that it was necessary to make a distinction between these two terms "fenestration" and "duplication".

Cerebral Angiography↗

[Agraphia and preservation of music writing in a bilingual piano teacher].

A bilingual virtuoso piano teacher developed aphasia and amusia, probably due to cerebral embolism. The perfectly demarcated and unique lesion was located in the left posterior temporoparietal region. Language examinations in French and Italian demonstrated entirely comparable difficulties in both languages. The linguistic course was favorable after a period of auditory agnosia and global aphasia. Language became fluent again 3 months after the onset, with a marked vocabulary loss and phonemic paraphasias with attempts at self-correction. Repetition was altered markedly with a deficit in auditory comprehension but no remaining elements of auditory agnosia. Reading was possible, but with some difficulty and total agraphia and acalculia persisted. Musical ability was better conserved, particularly with respect to repetition and above all to writing, the sparing of the latter constituting a fairly uncommon dissociation in relation to agraphia. Findings are discussed in relation to data in the literature concerning hemispheric participation in various musical tasks.

Agraphia↗

[Treatment of arterio-venous aneurysms (AVA) in language areas. About 18 cases (author's transl)].

This study, about 18 cases of AVA strictly in language areas (posterior end of the inferior frontal gyrus (Broca), posterior third of T1 and T2, supra-marginalis and angularis gyri), prepose to specify the possibilities and the legitimacy of surgical management as well as the technical parts of removal. Malformations may divided in to : 1 arterio-venous fistula, 3 medium AVAS, 8 important AVA, 6 cirsoïdum aneurysms. 15 patients presented one or several intracranial hemorrhage (ICH). 3 others were admitted before this complication : 2 epileptic seizures, 1 left hemicrania. 2 patients died because of important ICH before any surgical treatment. The 16 others were operated. Two died during post-operative course : one because of prior ICH with deep coma, the other third month after a good period. In one case upon 14, a disabling handicap concerning language is noted after an important ICH. Except an ethylic patient with post-operative acalculia, minor language handicaps were observed only in patients with hemorrhagic seizure, permissing always satisfactory life. The best results on vital and functional prognosis were obtained for patients with no prior important ICH. So we must indicate the radical removal of all known AVA or cirsoïd aneurysms. The opinion that these last malformations generally do not bleed, may produced mistakes with their weighty consequences.

Adolescent↗

[Progressive severity of left unilateral apraxia in 2 cases of Alzheimer disease].

Two patients presented with progressive left unilateral motor apraxia and progressive visuo-spatial difficulties, including constructional apraxia, dressing apraxia, spatial dysgraphia and dyslexia, spatial acalculia and neglect of the left side, without significant changes in the other cognitive functions. In both patients, radiological tests demonstrated cortical atrophy, more marked in the retrorolandic areas. A diagnosis of Alzheimer's disease was made in the first patient by cortical biopsy and in the second patient by post-mortem examination. The second patient died from an intercurrent neoplastic condition early in the course. Post-mortem examination of her brain showed the atrophy to be predominantly in the superior parietal gyri. In these areas, in addition to neuritic plaques and neurofibrillary tangles, these was also severe gliosis and superficial spongiosis. These two cases were clinically different from cortico-basal degeneration, where the motor difficulties are associated with occulo-motor problems, extrapyramidal signs and involuntary movements of the "alien hand" type. These data confirm the presence of focal forms of Alzheimer's disease. The motor apraxia seen in our patients resembles the kinesthesic apraxia described by Luria.

Alzheimer Disease↗

Eclamptogenic Gerstmann's syndrome in combination with cortical agnosia and cortical diplopia.

Cortical blindness is defined as a loss of vision due to bilateral retrogeniculate lesions (geniculocalcarine blindness). Gerstmann's syndrome is a combination of disorientation for left and right, finger agnosia, and profound agraphia, alexia, and acalculia. It is due to a lesion in the left angular gyrus, situated at the confluence of the temporal, parietal, and occipital lobes. We report on a patient who suffered from severe underdiagnosed eclampsia and who developed bilateral extensive medial temporal, parietal, and calcarine ischemic infarctions during an eclamptic fit. In addition, ischemia destroyed the left angular gyrus. The combination of these lesions led to Gerstmann's syndrome with additional cortical agnosia and cortical diplopia. For the first few months following the ischemic insult, the patient had been cortically blind. Thereafter, the patient slowly regained a visual acuity of 0.1 in both eyes. She then experienced monocular and binocular diplopia. Her ocular motility was normal; there was no phoria or tropia. Monocular and binocular diplopia slowly became less severe over the following year. Now, 2 years after the incident, the patient has a visual acuity of 0.2 in both eyes and no double vision. However, the handicapping symptoms of Gerstmann's syndrome, which make leading a normal life impossible, have persisted--the patient still cannot cope alone, mainly due to the severe disorientation for left and right. The picture of cortical agnosia, cortical diplopia, and Gerstmann's syndrome is a very rare combination. Visual recovery and rehabilitation in cortical blindness are severely affected and made difficult by the symptoms of Gerstmann's syndrome. In our case the reason for such a dramatic clinical picture was eclampsia, whose prodomes had not been diagnosed in time.

Adult↗

An unusual neurological feature of HIV-1 encephalopathy: Gerstmann's syndrome.

The authors describe the first case in literature of Gerstmann's syndrome (agraphia, acalculia, finger agnosia) occurred in HIV correlated encephalopathy developed as the first severe manifestation of HIV infection in a patient with prevalent white matter neuroradiologic alterations. The PDL rapidly extended from the left subcortical parietal-occipital regions to the pre-rolandic one, with subsequent involvement of the corpus calosum splenium and the bilateral temporal lobes white matter. The authors indicate the extent of the lesions and the involvement of the interhemispheric connection fibres as the pathogenetic mechanism of the "Gerstmann syndrome", that until today has not been reported in the literature of the wide variety of AIDS dementia complex. The administration of 1 g of zidovudine for about 9 months did not avoid the establishing of the neurologic damage, but the sudden suspension of the drug could have enhanced the exacerbation of inflammation and the involvement of areas whose lesion is classically believed responsible for cognitive impairment.

AIDS Dementia Complex↗

Paroxystic neuropsychological symptoms as the early expression of hepatic encephalopathy. A case report.

A 52-year-old patient presented with paroxystic episodes of generalized apraxia, anomia, agraphia and acalculia. The transient character of these attacks was supported by several neuropsychological examinations. Initially a tentative diagnosis of multiple TIA's was made. Treatment consisted of antiplatelet aggregation therapy. Three years later, however, paroxystic neuropsychological symptomatology occurred more frequently with an increase of severity. The patient was again seen and the differential diagnosis included epilepsy or a metabolic disturbance, in casu hepatic encephalopathy. A therapeutic trial with carbamazepine was started but the patient deteriorated further. He developed a flapping tremor and became stuporous. The blood ammonia was high and there were triphasic waves on the EEG. A probable diagnosis of hepatic encephalopathy was made and carbamazepine therapy was withdrawn. There was a good response on low protein diet and lactulose.

Hepatic Encephalopathy↗

[A case of intraventricular cystic meningioma].

We report a case of intraventricular cystic meningioma in the left lateral ventricle. A-44-year-old male patient was admitted with headache on November 25, 1991. Neurological examination revealed acalculia and left homonymous hemianopsia. CT and MRI showed a solid tumor which originated in the body of the left lateral ventricle, and which was associated with a cystic component postero-laterally. Tumor strains fed by the left anterior choroidal artery and the medial and lateral posterior choroidal artery by angiography. The tumor was totally removed via the lateral temporal parietal approach. Histological examination revealed meningotheliomatous meningioma in the solid tumor and only gliosis in the wall of the cyst.

Adult↗

Long-term outcome following head injury.

In a perspective study of follow-up of 141 head-injured patients, neurological, behavioural, neuropsychological and psychosocial parameters of outcome were used to measure the patient's functional status for 18 months. Neurophysical sequelae including seizure disorders were seen in 29 patients. Cortical functional disturbances observed were nominal difficulties in 5 patients, perseveration in 5 patients, disturbed kinetic melodies in 9 patients, frontal acalculia in 4 patients, constructional apraxia in one patient and left side neglect in one patient. These deficits were reversed except in 13 cases. Only 32 patients (22.7%) did not suffer from any behavioural changes. The role of compensation as an aetiologic factor was found in 5 patients. Out of 94 patients in whom scores in memory test was done, 11 patients performed better than their age and education-related norms. Scores in Raven's matrices for level of intellectual performance were done in 71 patients. The score was below 25th percentile in majority (58 cases). Among 130 patients with some jobs, 56 patients (43%) were fully restored. Out of 105 married patients, 45 patients (43%) had disturbed relations after head injury. Seven patients had separation of marriage. Compared to neurological deficits, behavioural and neuropsychological impairments were more prevalent and disabling. Psychosocial outcome, particularly vocational restoration was adversely affected by behavioural changes and cognitive deficits. Need for a multidisciplinary intervention to minimise the avoidable morbidity is emphasised.

Craniocerebral Trauma↗

[Early diagnosis of herpes simplex virus encephalitis by single photon emission computed tomography (SPECT) in patients with normal MRI].

Since treatment of herpes simplex virus encephalitis (HSVE) is most effective when started early, a sensitive and specific method for early diagnosis would be of great benefit. MRI and CT are commonly used for this purpose. In this study, we presented two patients who had serologically confirmed HSVE and had normal CT and MRI, but were diagnosed as having HSVE by means of SPECT in the early stage. Case 1 was a 56-year-old man who suddenly developed alexia. On admission, physical and neurological examination were unremarkable except for alexia, agraphia, acalculia, and left-right disorientation. Brain CT, MRI, and cerebral angiography were all normal. However, SPECT showed hyperaccumulation of 99m Tc-HM-PAO in the right temporal-occipital area. On the 5th hospital day, he became comatose. CSF study revealed marked pleocytosis. Even then, MRI including Gd-enhanced study was normal while SPECT continued to show hyperaccumulation. Detection of herpes simplex virus DNA in CSF by polymerase chain reaction was negative. Anti-HSV antibody titer in CSF and serum confirmed intrathecal production of the antibody on the 14th hospital day. Abnormal accumulation of tracer in SPECT returned to normal on the 31st day when he was alert but still had a mild Gerstman syndrome. Case 2 was a 61-year-old man with disturbance of consciousness, mental dysfunction, and generalized convulsion. He was diagnosed as having HSVE by means of CSF pleocytosis, detection of HSV DNA in CSF by polymerase chain reaction, and presence of anti-HSV antibody in the CSF. CT and MRI again revealed no abnormality while SPECT clearly showed hyperaccumulation in the left temporal lobe in an early stage. Hyperaccumulation of lipophilic tracer on SPECT study, especially in the temporal lobes, has been reported in the early stage of HSVE by previous investigators. Unlike MRI or enhanced CT, the increased tracer accumulation in SPECT does not reflect disruption of the blood-brain-barrier or inflammatory edema, but reflects hyperperfusion or some other HSVE related abnormality which is currently unknown. From these observations, we suggest that local hyperperfusion occurs before local inflammation, and that SPECT is the most useful scanning method for early diagnosis of HSVE when this disease is clinically suspected.

Encephalitis, Viral↗

[Symptomatology of dyscalculia in the presence of cortical lesions (author's transl)].

The authors examine the function of calculation in 26 patients presenting lateral local cortical lesions. They analyse the symptomatology of acalculia in relation to the location and lateral position of the lesion, and define the influence of concomitant constructive apraxia or aphasia before proposing a method for the classification of calculating disorders.

Aphasia↗