OBJECTIVE The presence of disproportionately enlarged subarachnoid space hydrocephalus (DESH) on brain imaging is a recognized finding of idiopathic normal pressure hydrocephalus (iNPH), but the features of DESH can vary across patients. The aim of this study was to evaluate the utility of MRI-based DESH scoring for predicting prognosis after surgery. METHODS In this single-center, retrospective cohort study, the DESH score was determined by consensus between a group of neurosurgeons, neurologists, and a neuroradiologist based on the preoperative MRI findings of the patients with suspected iNPH. The DESH score was composed of the following 5 items, each scored from 0 to 2 (maximum score 10 points): ventriculomegaly, dilated sylvian fissures, tight high convexity, acute callosal angle, and focal sulcal dilation. The association between the DESH score and improvement of the scores on the modified Rankin Scale (mRS), iNPH Grading Scale (iNPHGS), Mini-Mental State Examination (MMSE), Trail Making Test-A (TMT-A), and Timed 3-Meter Up and Go Test (TUG-t) was examined. The primary end point was improvement in the mRS score at 1 year after surgery, and the secondary outcome measures were the iNPHGS, MMSE, TMT-A, and TUG-t scores at 1 year after surgery. Improvement was determined as improvement of 1 or more levels on mRS, ≥ 1 point on iNPHGS, ≥ 3 points on MMSE, a decrease of > 30% on TMT-A, and a decrease of > 10% on TUG-t. RESULTS The mean DESH score for the 50 patients (mean age 77.6 ± 5.9 years) reviewed in this study was 5.58 ± 2.01. The mean rate of change in the mRS score was -0.50 ± 0.93, indicating an inverse correlation between the DESH score and rate of change in the mRS score (r = -0.749). Patients who showed no improvement in mRS score tended to have a low DESH score as well as low preoperative MMSE and TMT-A scores. There were no differences in the areas of deep white matter hyperintensity and periventricular hyperintensity on the images between patients with and without an improved mRS score (15.6% vs 16.7%, respectively; p = 1.000). The DESH score did differ significantly between patients with and without improved scores on the iNPHGS (6.39 ± 1.76 vs 4.26 ± 1.69, respectively; p < 0.001), MMSE (6.63 ± 1.82 vs 5.09 ± 1.93; p = 0.010), TMT-A (6.32 ± 1.97 seconds vs 5.13 ± 1.93 seconds; p = 0.042), and TUG-t (6.48 ± 1.81 seconds vs 4.33 ± 1.59 seconds; p < 0.001). CONCLUSIONS MRI-based DESH scoring is useful for the prediction of neurological improvement and prognosis after surgery for iNPH.

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Pineal parenchymal tumor of intermediate differentiation (PPTID) is rare. The WHO first classified PPTID in 2000 as a pineal parenchymal tumor (PPT) with an intermediate prognosis between pineocytoma (PC) and pineoblastoma (PB). It is considered an intermediate-grade tumor and divided into WHO grade II or III. The number of available reports about PPTID is presently limited, and the appropriate management for this tumor has not yet been determined. The authors report a rare case of PC in a 63-year-old woman who presented with lower-extremity weakness and gait disturbance. A pineal mass lesion was detected on MRI. A diagnosis of PC was established after microsurgical gross-total tumor resection, and the patient received no adjuvant therapy after surgery. Two years after surgery, a partial recurrence was recognized and Gamma Knife radiosurgery was performed. Fours years later, the patient developed diffuse leptomeningeal dissemination. She was successfully treated with craniospinal irradiation. Leptomeningeal dissemination may develop 6 years after the initial diagnosis of PC. A histopathological study of the recurrent tumor revealed a malignant change from PC to PPTID. The present case shows the importance of long-term follow-up of patients with PPTs following resection and the efficacy of craniospinal irradiation in the treatment of leptomeningeal dissemination.

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