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1/127. neurofibrillary tangles in the dementia of "normal pressure" hydrocephalus.

    Routine neuropathological examination could not explain the dramatic improvement exhibited by one patient with "normal pressure" hydrocephalus after shunting. The improved patient contrasted remarkably with the unchanged condition of four others also shunted successfully. The five brains were analysed by quantitative morphometry to determine the degree of neurofibrillary tangle formation in mesial temporal neurons. The density of tangle-bearing nerve cells in the four unimproved cases was markedly greater than in age-matched control brains from nineteen normal subjects, and fell in the same range as that of eight dements with neuropathologically confirmed Alzheimer's disease. The density of the one who recovered was within normal limits. The duration of dementia before shunting, and the total duration of dementia in these five patients rank in the same order as their degree of neurofibrillary formation. Furthermore, a positive linear correlation exists between the Tangle Indices and the total duration of dementia. The data suggest that early diagnosis may improve the chances of reversing the dementia of normal pressure hydrocephalus before histological alterations prove too severe. ( info)

2/127. Reversible dementia in idiopathic normal pressure hydrocephalus. A case report.

    OBJECTIVE: To present a woman with idiopathic normal pressure hydrocephalus (NPH) showing marked improvement in mental function after shunt operation. DESIGN: Case report. SETTING: primary health care. PATIENT: The clinical symptoms of gait disturbance, occasional urine incontinence and severely impaired mental function typical of NPH were all present. Although dementia predominated in the clinical picture, the diagnosis was confirmed by computer tomography (CT) and cerebrospinal fluid infusion test and cisternography. MAIN OUTCOME MEASURES: A marked improvement of mental functions was observed after shunt operation. RESULTS: Mini Mental State Examination score before shunt operation was 9 compared with 20 one year after operation. Also ventricle size was reduced as evaluated by CT scan. CONCLUSION: This reported case contrasts previous reports concluding that shunt responsive patients have only slight mental disturbances without significant improvement of mental status after shunt operation. ( info)

3/127. syndrome of inappropriate secretion of antidiuretic hormone associated with idiopathic normal pressure hydrocephalus.

    A 79-year-old woman suffering from urinary incontinence and unsteady gait was diagnosed as having idiopathic normal pressure hydrocephalus (NPH) with hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). The concentration of antidiuretic hormone was high while the plasma osmolality was low in the presence of concentrated urine during the episodes of hyponatremia. magnetic resonance imaging (MRI) of the head showed enlargement of the third and lateral ventricles. After ventriculoperitoneal shunt surgery, the symptoms of NPH and hyponatremia improved. It may be possibly explained that mechanical pressure on the hypothalamus from the third ventricle is responsible for hyponatremia. ( info)

4/127. Third ventriculostomy in normal pressure hydrocephalus.

    Improvement in three out of four cases of normal pressure hydrocephalus after third ventriculostomy is reported. Pre- and postoperative ventricular pressure recording suggested that this improvement may be occurring in the absence of an improvement in ventricular pressures. Third ventriculostomy may relieve periventricular tissue stress and thus improve local blood flow, which is a likely mechanism in the syndrome of normal pressure hydrocephalus. ( info)

5/127. Normal pressure hydrocephalus in diabetic patients with recurrent episodes of hypoglycemic coma.

    The pathophysiology of brain damage induced by severe hypoglycemia is still unknown. We experienced a case with type 1 diabetes and recurrent severe hypoglycemic coma who showed a central brain atrophy and an abnormal cerebrospinal fluid flow, suggesting normal pressure hydrocephalus. Following this case, the CSF flow was studied using 111In-DTPA cisternography in six consecutive diabetic patients admitted for repeated episodes of hypoglycemic coma. All the patients showed the central brain atrophy on computed tomography and four of them (67%) had the ventricular reflux, with delayed clearance of 111In-DTPA. Two patients with abnormal CSF flow showed cognitive dysfunction by WAIS or WAIS-R. In contrast, none of five randomly selected diabetic patients, without hypoglycemic coma showed abnormal CSF flow. Our results suggest the presence of normal pressure hydrocephalus in diabetic patients with recurrent hypoglycemic coma. It may associate with the cognitive dysfunction. ( info)

6/127. Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure.

    A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus. ( info)

7/127. Anatomical and psychological mechanism of reduplicative misidentification syndromes.

    Reduplicative misidentifications syndromes (RMS) are rare memory disorders characterized by the subjective conviction that a place, person or event is duplicated. Even if RMS often follow a right frontal lesion, several studies have stressed the importance of bilateral hemispheric pathology. Moreover, from a psychological perspective, there is uncertainty if this symptom should be considered just as a kind of confabulation or if it should be associated with personal psychosocial and behavioral aspects. We report a patient who developed normal pressure hydrocephalus and RMS one year after a post-traumatic right frontal lesion. At the first neuropsychological evaluation, we found mild impairment of all functions, associated with the presence of reduplicative paramnesia. After the ventricle-peritoneal shunt intervention, we observed a progressive improvement of all functions but the frontal ones. The memory deficit became less specific and the RMS disappeared. We therefore postulate that a focal right frontal lesion is not sufficient to cause RMS per se. Our clinical report suggests that paramnesic events held on reasonable ground, not being just a kind of confabulation. ( info)

8/127. A case of myoedema and normal pressure hydrocephalus.

    A cachectic 55-year-old man presented with ataxia and metabolic instability. On physical examination, he had prominent myoedema in all muscle groups. In addition, a CT scan and lumbar puncture showed extensive nonobstructive hydrocephalus. Further clinical evaluation revealed elevated creatine phosphokinase and liver enzyme levels, although the patient was euthyroid. The patient improved neurologically and metabolically with supportive therapy but the myoedema persisted. Previous cases have emphasized that myoedema is a localized, electrically silent, benign myopathic disorder of unknown cause. As with a previous case with ventricular enlargement, myoedema may be part of systemic pathology. Finally, as in most other reports, myoedema is a rare condition; only 3 cases (of 44) with palpable (but not visible) myoedema were uncovered in this study. ( info)

9/127. Late recovery from head injury: case report and review.

    Some problems of prognosis in patients with head injury are reviewed, and a case of late and unexpected recovery from post-traumatic dementia presented. The possible mechanism of recovery is discussed with particular reference to normal pressure hydrocephalus. ( info)

10/127. Normal pressure hydrocephalus and basilar artery aneurysm. Case report and pathogenetic consideration.

    The authors describe the case of a 60-year-old patient with basilar artery aneurysm who in time developed normal pressure hydrocephalus (NPH). Clinical examinations and laboratory tests did not reveal other data from which the pathology could be attributed to causes other than the vascular malformation already considered. The mechanical obstruction exerted by the aneurysm lodged on the floor of the third ventricle represents, according to the authors, the physiopathogenetic mechanism by which a temporal delay is developed between the endoventricular pulsation and that of the cerebral veins so as to produce a precise pulsatile gradient in a centrifugal direction to allow the formation of an active hydrocephalus proportional to the entity of cerebrospinal fluid (CSF) pulse. ( info)
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