Reversible coma with raised intracranial pressure: an unusual clinical manifestation of CADASIL.

A 50-year-old woman presented with recurrent episodes of headache, nausea and disturbed consciousness that were fully reversible within a few days. Clinical and radiological findings suggested raised intracranial pressure, which on one occasion was confirmed by intracranial pressure monitoring. Magnetic resonance imaging performed in the asymptomatic interval disclosed a diffuse leukoencephalopathy. Brain biopsy surprisingly revealed the typical vascular changes of CADASIL and subtle endothelial alterations. The white matter showed edematous changes and reactive gliosis. Mutational analysis of the Notch3 gene revealed a previously unreported mutation. We suggest that a transient disturbance of the blood-brain barrier related to the underlying vascular pathology may have caused this unusual presentation of CADASIL.

Scintigraphic differentiation between two forms of primary dysautonomia early after onset of autonomic dysfunction: value of cardiac and pulmonary iodine-123 MIBG uptake.

Primary dysfunction of the autonomic nervous system can be observed in patients with Parkinson's disease and those with multiple system atrophy. However, the fate of the two diseases differs considerably and leads to different strategies for patient management. Differentiation of the two diseases currently requires a combination of several clinical and electrophysiological tests. First studies of myocardial innervation using iodine-123 metaiodobenzylguanidine (MIBG) indicated a possible role of scintigraphy for this purpose. An increase in the pulmonary uptake of 123I-MIBG has been reported in secondary dysautonomias. Whether sympathetic innervation of the lung is affected in primary dysautonomias is currently unknown. Therefore, cardiac and pulmonary uptake of 123I-MIBG was studied in 21 patients with Parkinson's disease, 7 patients with multiple system atrophy and 13 age- and sex-matched controls. Thoracic images were obtained in the anterior view 4 h after intravenous injection of 185 MBq 123I-MIBG, at which time the maximum neuronal uptake is reached. All patients with Parkinson's disease had significantly lower cardiac uptake of 123I-MIBG than patients with multiple system atrophy and controls. Sympathetic innervation of the lung was not affected in either disease. It is concluded that scintigraphy with 123I-MIBG appears to be a useful tool for differentiation between Parkinson's disease and multiple system atrophy early after onset of autonomic dysfunction.

White matter lesions in patients with idiopathic normal pressure hydrocephalus and in an age-matched control group: a comparative study.

OBJECTIVE: To compare the occurrence of periventricular lesions (PVLs) and deep white matter lesions (DWMLs) in elderly patients with idiopathic normal pressure hydrocephalus (NPH) and in an age-matched control group. METHODS: PVLs and DWMLs were evaluated using T2-weighted magnetic resonance scans of 37 patients with idiopathic NPH and 35 participants from an age-matched control group. All patients with idiopathic NPH included in this study improved after shunting. The control group consisted of 16 healthy elderly persons and 19 patients with depression. To allow quantitative assessment and comparison, scores for PVLs and DWMLs were calculated. Furthermore, possible correlations between white matter lesion scores, ventricular width, and age were investigated. RESULTS: There was a significantly higher incidence of PVLs and DWMLs in patients with idiopathic NPH. The mean total PVL was 12.1 (range, 2-24) in the NPH group and 3.9 (range, 0-10) in the control group (P < 0.001). The mean total DWML score was 12.9 (range, 3-24) in the NPH group and 4.5 (range, 0-16) in the control group (P < 0.001). There were significant correlations between the severity of PVL and DWML scores in both groups. Only a weak positive correlation between the severity of DWMLs and age was found in the NPH group, whereas this correlation was significant in the control group. There was a significant negative correlation between the width of the anterior horns and the severity of both PVLs and DWMLs in patients with NPH; however, positive correlations were found in the control group. CONCLUSION: Elderly patients with idiopathic NPH have more frequent and more severe PVLs and DWMLs than people in age-matched control groups. Our data suggest a frequent co-occurrence of idiopathic NPH and vascular subcortical encephalopathy; however, they do not support a direct causal relationship.

Flow void of cerebrospinal fluid in idiopathic normal pressure hydrocephalus of the elderly: can it predict outcome after shunting?

OBJECTIVE: We investigate the predictive value of cerebrospinal fluid (CSF) flow void on outcome after shunting in a prospective series of patients with idiopathic normal pressure hydrocephalus (NPH). METHODS: The degree and extension of CSF flow void were examined on T2-weighted magnetic resonance imaging scans of 37 elderly patients with idiopathic NPH who underwent subsequent shunting. The degree of flow void was assessed in comparison with the signal of large cerebral arteries. The extension was evaluated via the calculation of sum scores for the occurrence of flow void in different locations of the ventricular system. Those parameters were not considered in the decision to perform shunting. CSF flow void in the aqueduct and the adjacent third and fourth ventricles of the 37 patients with idiopathic NPH was compared with that of 37 age-matched control patients. CSF flow void scores in patients with idiopathic NPH were investigated for correlations between postoperative outcome scores and ventricular width indices. RESULTS: No difference was found between the occurrence of aqueductal CSF flow void in patients with idiopathic NPH and the control group. A significant difference, however, was noted for the extension of the CSF flow void, which was greater in the NPH group. Postoperative improvement was found in 33 of 37 patients with idiopathic NPH at a mean follow-up of 15.6 months. Only small, statistically not significant correlations were found between CSF flow void and postoperative outcome. Flow void sum scores, however, correlated significantly with ventricular width indices. CONCLUSION: The degree and extension of CSF flow void on T2-weighted magnetic resonance imaging scans have little predictive value for outcome after shunting in patients with idiopathic NPH. The greater extension of the CSF flow void in patients with NPH is most likely related to increased ventricular width. It is not useful to consider CSF flow void findings on conventional magnetic resonance imaging scans in making the decision to offer shunting in patients with idiopathic NPH.

In vivo proton spectroscopy of meningioma after preoperative embolization.

The time course of proton spectra of meningioma after embolization has been followed using a localized PRESS experiment with 135 ms of echo time. The most conspicuous findings were the observation of transient lactate signal within 24 h after blocking of the capillary bed. After that time large aliphatic signals from necrosis were observed, whereas other metabolite signals vanished. It was demonstrated, that tumor necrosis as shown by proton spectroscopy is complete within 4 days. This finding is significant for the timing of surgery, which can be performed after this period.