Enlarged periventricular space and periventricular lesion extension on baseline brain MRI predicts poor neurological outcomes in cryptococcus meningoencephalitis

In Cryptococcus neoformans meningoencephalitis, brain MRI findings might reflect the phathomechanism of disease progression that is fungal accumulation in the peri-venular space and consequent invasion into the parenchyma. This study analyzed serial brain MRI findings of 76 patients with cryptococcus meningoencephalitis in association with the disease progression and outcomes. MRI parameters included the enlarged periventricular space (ePVS) score (range 0–8), periventricular lesion extension, cryptococcoma, and hydrocephalus. Clinical outcomes at 2-week, 10-week, and 6-month were evaluated using modified Rankin scale (mRS). At 6 months, 15 (19.7%) patients died and 34 (44.1%) had poor neurological outcomes (mRS scores > 2). At baseline, an ePVS score of ≥ 5 (Odds-ratio [OR]: 94.173, 95% confidence-interval [95%CI]: 7.507–1181.295, P < .001), periventricular lesion extension (OR: 51.965, 95%CI: 2.592–1041.673, P = .010), and presence of encephalitis feature (OR: 44.487, 95%CI: 1.689–1172.082, P = .023) were associated with 6-month poor outcomes. Presence of two or more risk factors among encephalitis feature, ePVS score ≥ 5, and periventricular lesion extension at baseline, was associated with 6-month poor outcomes (area under the curve [AUC]: 0.978, P < .001) and mortality (AUC: 0.836, P < .001). Disease progression was associated with interval development of cryptococcoma and hydrocephalus. Brain MRI findings might be useful in predicting outcomes and monitoring the progression of cryptococcus meningoencephalitis.

Outcomes of Cryptococcus meningoencephalitis and associated magnetic resonance imaging findings

In Cryptococcus Neoformans meningoencephalitis, brain MRI findings might reflect the phathomechanism of disease progression that is fungal accumulation in the peri-venular space and consequent invasion into the parenchyma. This study analyzed serial brain MRI findings of 76 patients with cryptococcus meningoencephalitis in association with the disease progression and outcomes. MRI parameters included the enlarged periventricular space (ePVS) score (range 0 − 8), periventricular lesion extension, cryptococcoma, and hydrocephalus. Clinical outcomes at 2-week, 10-week, and 6-month were evaluated using modified Rankin scale (mRS) scores. At 6 months, 15 (19.7%) patient died and 34 (44.1%) had poor neurological outcomes (mRS scores > 2). At baseline, an ePVS score of ≥ 5 (Odds-ratio [OR]: 94.173, 95% confidence-interval [95% CI]: 7.507 − 1181.295, P < 0.001), periventricular lesion extension (OR: 51.965, 95% CI: 2.592 − 1041.673, P = 0.010), and presence of encephalitis feature (OR: 44.487, 95% CI: 1.689 − 1172.082, P = 0.023) were associated with 6-month poor outcomes. Presence of two or more risk factors at baseline was highly associated with the 6-month poor outcomes (area under the curve [AUC]: 0.978, P < 0.001) and mortality (AUC: 0.836, P < 0.001). Disease progression was associated with interval development of cryptococcoma and hydrocephalus. In conclusion, brain MRI findings might be useful in predicting poor outcomes and monitoring the disease progression of cryptococcus meningoencephalitis.

GCT-55. INTRACRANIAL GERMINOMA ORIGINATING FROM ATYPICAL LOCATION WITH SUBCLINICAL ADH INSUFFICIENCY

INTRODUCTION Intracranial germinomas are rare tumors which usually develop in the midline structures and affect in 90% of cases the pineal gland and suprasellar regions. Sometimes they involve basal ganglia, septum pellucidum, and other regions. We report a very unusual presentation of an intracranial germinoma originating from the lateral ventricle. METHODS A 10-year-old boy presented with a 1-year history of polydipsia and polyuria. During the hypertonic saline test, a low ADH was detected and established the diagnosis of subclinical ADH insufficiency. MRI showed a heterogeneously enhancing periventricular lesion in the lateral ventricle, but no other abnormal findings, including hypophyseal stalk. Initially, the correlation of imaging findings and clinical symptoms were not clear. With suspected subependymoma, tumor removal was performed by small craniotomy. Since the intra-operative pathological diagnosis was germinoma, we performed only partial removal of the tumor. After establishing the histological diagnosis of germinoma, the patient received chemotherapy using carboplatin and etoposide, followed by radiation therapy. MRI showed no recurrence for five years after treatment. RESULTS/ CONCLUSION Our case presents two atypical features. First, intracranial germinoma originating from the lateral ventricle is quite rare. Though the cases with intracranial germinoma originating from septum pellucidum and corpus callosum have been reported, this case is even different. Second, imaging findings did not match clinical symptoms. The cause of subclinical ADH deficiency may be the occult hypophyseal germinoma. In conclusion, we report a 10-year-old case with a very unusual presentation of an intracranial germinoma originating from the lateral ventricle.

Impairments of Visuospatial Attention in Children with Unilateral Spastic Cerebral Palsy

Aim. This observational study aimed at assessing the prevalence of visuospatial attention deficits in children with unilateral spastic cerebral palsy (USCP), taking into consideration the affected hemibody and the localization of the brain lesion. Method. Seventy-five children with USCP were assessed with four visuospatial attention tests: star cancellation, Ogden figure copy, line bisection, and proprioceptive pointing. Results. A majority (64%) of children with USCP presented a deficit in at least one test compared to the reference values. The alterations observed in children with left or right USCP were related to egocentric or allocentric neglect, respectively. Children with cortico/subcortical lesion presented more often visuospatial attention deficits than children with periventricular lesion. Visuospatial attention deficits were not associated with brain lesion locations. Interpretation. Visuospatial attention deficits are prevalent in children with USCP and should be taken into account during their rehabilitation process. The present results shed new light on the interpretation of motor impairments in children with USCP as they may be influenced by the frequent presence of visuospatial deficits.

Lesion topographies in multiple sclerosis diagnosis

Objectives:To assess the contributions of cortico-juxtacortical and corpus callosum lesions to multiple sclerosis diagnosis and to compare the value of ≥1 vs ≥3 periventricular lesions in clinically isolated syndromes (CIS).Methods:Step 1: We evaluated lesion topography classifications in 657 patients with CIS with stepwise Cox proportional hazards regression models considering second attack as the outcome. Step 2: We established 2 dissemination in space (DIS) versions according to the periventricular lesion cutoffs of ≥1 and ≥3 and assessed their performance at 10 years with second attack as the outcome, first individually and then combined with dissemination in time (DIT) in all cases (n = 326), by age, and by CIS topography.Results:Step 1: The models (hazard ratios [95% confidence interval]) favored ≥1 over ≥3 periventricular lesions (2.5 [1.7–3.6]) and cortico-juxtacortical over juxtacortical lesions (1.4 [1.0–1.8]). Callosal lesions were not selected. Step 2: DIS specificity with ≥1 periventricular lesions was slightly lower than with ≥3 (59.1 vs 61.4) and the same after adding DIT (88.6). Regarding age, ≥3 periventricular lesions improved DIS specificity over ≥1 lesions in the 40–49 years of age bracket (66.7 vs 58.3). This difference disappeared when adding DIT (83.3). Optic neuritis had a similar pattern when evaluating CIS topographies.Conclusions:Our results comply with the Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS) consensus recommendation of combining cortical and juxtacortical lesions into a single term when possible. Concerning periventricular lesions, maintaining the current ≥1 cutoff in the McDonald criteria does not compromise specificity in typical CIS cases, but attention should be paid to older patients or optic neuritis cases.

Periventricular lesions and MS diagnostic criteria in young adults with typical clinically isolated syndromes

In patients who present with a clinically isolated syndrome (CIS), whose features are suggestive of multiple sclerosis (MS), fulfilling McDonald 2010 magnetic resonance imaging (MRI) criteria for dissemination in space (DIS) and dissemination in time (DIT) enables a diagnosis of MS. While ⩾1 periventricular lesion is included in the 2010 DIS criteria, earlier McDonald criteria required ⩾3 periventricular lesions to confirm DIS and recent Magnetic Resonance Imaging in Multiple Sclerosis (MAGNIMS)-recommended DIS criteria also require ⩾3 lesions. We investigated the effect of varying the required number of periventricular lesions and found that the best combination of specificity and sensitivity for clinically definite MS was seen for ⩾1 periventricular lesion using both the McDonald 2010 and MAGNIMS 2016 criteria.

Lesion probability mapping to explain clinical deficits and cognitive performance in multiple sclerosis

Background: Lesion dissemination in time and space represents a key feature and diagnostic marker of multiple sclerosis (MS). The correlation between magnetic resonance imaging (MRI) lesion load and disability is only modest, however. Strategic lesion location might at least partially account for this ‘clinico-radiologic paradox’. Objectives: Here we used a non-parametric permutation-based approach to map lesion location probability based on MS lesions identified on T2-weighted MRI. We studied 121 patients with clinically isolated syndrome, relapsing–remitting or secondary progressive MS and correlated these maps to assessments of neurologic and cognitive functions. Results: The Expanded Disability Status Scale correlated with bilateral periventricular lesion location (LL), and sensory and coordination functional system deficits correlated with lesion accumulation in distinct anatomically plausible regions, i.e. thalamus and middle cerebellar peduncule. Regarding cognitive performance, decreased verbal fluency correlated with left parietal LL comprising the putative superior longitudinal fascicle. Delayed spatial recall correlated with _amygdalar, _left frontal and parietal LL. Delayed selective reminding correlated with bilateral frontal and temporal LL. However, only part of the spectrum of cognitive and neurological problems encountered in our cohort could be explained by specific lesion location. Conclusions: Lesion probability mapping supports the association of specific lesion locations with symptom development in MS, but only to limited extent.

Heterogeneity in mild cognitive impairment: Differences in neuropsychological profile and associated white matter lesion pathology

This study examined whether distinct neuropsychological profiles could be delineated in a sample with Mild Cognitive Impairment (MCI) and whether white matter lesion (WML) burden contributed to MCI group differences. A heterogeneous, clinical sample of 70 older adults diagnosed with MCI was assessed using cognitive scores, and WML was quantified using a semi-automated, volumetric approach on T2-weighted fluid-attenuated inversion recovery (FLAIR) images. Using cluster and discriminant analyses, three distinct groups (Memory/Language, Executive/Processing Speed, and Pure Memory) were empirically derived based on cognitive scores. Results also showed a dose dependent relationship of WML burden to MCI subgroup, with the Executive/Processing Speed subgroup demonstrating significantly higher levels of WML pathology when compared to the other subgroups. In addition, there was a dissociation of lesion type by the two most impaired subgroups (Memory/Language and Executive/Processing Speed) such that the Memory/Language subgroup showed higher periventricular lesion (PVL) and lower deep white matter lesion (DWML) volumes, whereas the Executive/Processing Speed demonstrated higher DWML and lower PVL volumes. Results demonstrate that distinct MCI subgroups can be empirically derived and reliably differentiated from a heterogeneous MCI sample, and that these profiles differ according to WML burden. Overall, findings suggest different underlying pathologies within MCI and contribute to our understanding of MCI subtypes. (JINS, 2009, 15, 906–914.)

I foci iperintensi in corso di neurofibromatosi hanno sempre una evoluzione benigna?

More than 70% of patients with neurofibromatosis type 1 (NF-1), present hyperintense foci in MR DP-T2 weighted images, located in deep grey and white matter. The true nature of these hyperintense foci is still unknown: they could be hamartomas, that is composition and organisation alterated areas of cytologic normal tissue, gliotic areas, delayed or disorganised areas of myelinisation and, finally, low grade glial tumors (astrocytoma). We present the case of a 17 years old pazient, who underwent 8 MR brain studies in a six years period. We analyzed the MR characteristics of the hyperintense foci in seriated studies and, particularly, the evolution of a single, simple, hyperintense periventricular lesion in cistic tumor with intramural nodule.