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The most frequent type of Krabbe disease has an infantile onset. Unusual slowly progressive adult forms have also been described. We described a different involvement of white matter tracts where magnetic resonance signal alterations were evident in a case of a patient affected by late-onset form of disease.

Whether fractional anisotropy (FA), apparent diffusion coefficient (ADC), and fiber density index (FDi) values differ in the white matter close to glioblastomas of both symptomatic and asymptomatic patients was investigated. Twenty patients with glioblastomas underwent magnetic resonance imaging study. The FDi, FA and ADC values were calculated in areas of white matter in close proximity to the tumor (perWM) and encompassing fibers of cortico-spinal tract and in the contralateral normal-appearing white matter (nWM). The clinical compromise of the cortico-spinal tract was graded using Brunnstrom's criteria. FA and FDi were significantly decreased and ADC increased in perWM compared with the contralateral. Mean FDi, FA, and ADC values comparing perWM and nWM in symptomatic patients showed similar differences. Comparing the perWM of symptomatic and asymptomatic patients, mean FDi and ADC values were lower in symptomatic patients than in asymptomatic ones. A positive correlation was found between the clinical score (CS) and, separately, FDi, FA and ADC per WM values. In a multiple stepwise regression among the same factors, only the ADC of perWM values showed a positive correlation with the CS. An increased ADC plays a major role in reducing the number of fibers (reduced FDi) in symptomatic patients.

B.D. is a 48-year-old professional woman. She has been suffering for migraine since she was 28, but she did not have serious problems until last year, when headache episodes became more frequent and it was necessary an admission to emergency room. At the beginning, the events were about 6 per month, lasting from 2 to 4 days, beating and of high intensity together with nausea, vomit, photo and phonophobia and visual area. Looking at the anamnesis, we report a psychiatric treatment since about ten years, because of type II bipolar disorder. In spite of the psychopharmacological treatment, as the patient came in our Regional Headache Center, she talked about 7 events with aura (scintillating scotomas, emianopsia) per month, lasting 2-4 days with photo and phonophobia, nausea, crying crisis, anxiety. Although the neurological examination was normal, the sudden aggravation of pain symptomatology and the unresposiveness to usual painkillers, suggested a cerebral CT and CT-angiography. CT and CT-angiography discovered the presence of an aneurysm of the right middle cerebral artery (MCA) of 4 mm diameter, with parietal irregularities. The patient was operated to reduce the hemorrhagic risk, with a positive result. One year after the operation, the patient reports a decrease of headache events with a frequency of 2 per month, lasting only a few hours, which she can now solve with COXIB.

Diffusion-weighted imaging (DWI) has been shown to be highly sensitive in detecting acute cerebral infarction, but its use in detecting hypoxic-ischemic encephalopathy (HIE) in neonates is still controversial. Moreover, few reports concern pre-term infants with possible periventricular leukomalacia (PVL). We examined the ability of this technique to detect cerebral changes in the acute phase of PVL. Fifteen MR examinations were performed in 11 pre-term infants (mean age 3.4 days, range 2-6 days). Conventional DWI sequences, apparent diffusion coefficient (ADC) maps, and US obtained in the acute phase were compared. All the neonates underwent US follow-up up to 4 months after delivery; those with suspected PVL also underwent MRI follow-up for up to 2 months. Qualitative and quantitative evaluations were performed to assess the presence of DW changes compatible with PVL. Diffusion-weighted MRI showed signal hyperintensity associated with decreased ADC values in 3 subjects (27%). In these patients conventional MRI sequences were interpreted as normal and US (performed at the same time) as doubtful in 2 and compatible with PVL in 1 subject. The MRI and US follow-up confirmed severe damage in all these patients. In 1 neonate hemorrhages involving the germinative matrix were identified. In 8 neonates MRI was considered normal. In these subjects US follow-up (up to 4 months) confirmed no signs of PVL. Diffusion-weighted imaging may have a higher correlation with later evidence of PVL than does conventional MR imaging and US when performed in the acute phase of the disease.

We performed serial baseline and gadolinium (Gd)-DTPA-enhanced MRI in 4 patients with definite multiple sclerosis. Studies were performed every month for a total of 4 scans. We obtained short TR/short TE sequences at 10 and 60 minutes after Gd-DTPA injection. All patients had multiple hyperintense lesions seen on baseline MRI with long TR/short and long TE. There was Gd-DTPA enhancement in new, enlarging, and preexisting lesions that were unchanged in size. The enhancing lesions were always seen on T2-weighted images. There was no difference in enhancement between the 10- and 60-minute studies. Six of 85 preexisting lesions enhanced whereas all new or enlarging lesions enhanced. Enhancement persisted in only 1/3 of the new or enlarging lesions, suggesting that MR enhancement is a transient phenomenon due to local temporary blood-brain barrier breakdown. Our data indicate that Gd-DTPA enhancement monitoring is more sensitive than unenhanced MRI for detecting disease activity in MS.

A child with microcephaly, facial dysmorphisms, seizures, and congenital cardiopathy is presented. On the basis of skull x-rays, electroencephalogram, transillumination of the head, and computed tomography (CT) scan, the diagnosis of semilobar holoprosencephaly was made. The heterogeneous etiology of the disorder is discussed in order to evaluate the recurrence risk. The usefulness of CT scan for the classification in the different types of holoprosencephaly is stressed.