No existing medication has yet been shown to convincingly improve cerebellar ataxia. Therefore, the identification of new drugs for its symptomatic treatment is desirable. The objective of this case series was to evaluate the efficacy of treatment of cerebellar ataxia with the amino acid acetyl-dl-leucine (Tanganil). Thirteen patients (eight males, median age 51 years) with degenerative cerebellar ataxia of different etiologies (SCA1/2, ADCA, AOA, SAOA) were treated with acetyl-dl-leucine (5 g/day) without titration for 1 week. Motor function was evaluated by changes in the Scale for the Rating and Assessment of Ataxia (SARA) and in the Spinocerebellar Ataxia Functional Index (SCAFI) during treatment compared to a baseline examination. Quality of life (EuroQol-5D-3L) and side effects were also assessed. Mean total SARA decreased remarkably (p = 0.002) from a baseline of 16.1 ± 7.1 to 12.8 ± 6.8 (mean ± SD) on medication. There were also significant improvements in sub-scores for gait (p = 0.022), speech (p = 0.007), finger-chase (p = 0.042), nose-finger-test (p = 0.035), rapid-alternating-movements (p = 0.002) and heel-to-shin (p = 0.018). Furthermore, patients showed better performance in the SCAFI consisting of the 8-m-walking-time (8 MW, p = 0.003), 9-Hole-Peg-Test of the dominant hand (9HPTD, p = 0.011) and the PATA rate (p = 0.005). Quality of life increased during treatment (p = 0.003). No side effects were reported. In conclusion, acetyl-dl-leucine significantly improved ataxic symptoms without side effects and therefore showed a good risk–benefit profile. These findings need to be confirmed in placebo-controlled trials.

BackgroundPreviously proposed classifications for carotid plaque and cerebral parenchymal hemorrhages are used to estimate the age of hematoma according to its signal intensities on T1w and T2w MR images. Using these classifications, we systematically investigated the value of cardiovascular magnetic resonance (CMR) in determining the age of vessel wall hematoma (VWH) in patients with spontaneous cervical artery dissection (sCAD).Methods35 consecutive patients (mean age 43.6 ± 9.8 years) with sCAD received a cervical multi-sequence 3T CMR with fat-saturated black-blood T1w-, T2w- and TOF images. Age of sCAD was defined as time between onset of symptoms (stroke, TIA or Horner's syndrome) and the CMR scan. VWH were categorized into hyperacute, acute, early subacute, late subacute and chronic based on their signal intensities on T1w- and T2w images.ResultsThe mean age of sCAD was 2.0, 5.8, 15.7 and 58.7 days in patients with acute, early subacute, late subacute and chronic VWH as classified by CMR (p < 0.001 for trend). Agreement was moderate between VWH types in our study and the previously proposed time scheme of signal evolution for cerebral hemorrhage, Cohen's kappa 0.43 (p < 0.001). There was a strong agreement of CMR VWH classification compared to the time scheme which was proposed for carotid intraplaque hematomas with Cohen's kappa of 0.74 (p < 0.001).ConclusionsSignal intensities of VWH in sCAD vary over time and multi-sequence CMR can help to determine the age of an arterial dissection. Furthermore, findings of this study suggest that the time course of carotid hematomas differs from that of cerebral hematomas.

Our pilot study demonstrates that vessel wall CE is a frequent finding in PACNS and its mimics. Larger trials will be necessary to evaluate the utility of BB-MRI in the diagnostic workup of PACNS.

Background and Purpose— Vessel wall inflammation (VWI) may be a pathogenetic factor in cervical artery dissection (CAD). We used contrast-enhanced high-resolution MRI (hrMRI) and positron emission tomography CT (PET-CT) to systematically investigate VWI in spontaneous CAD. Methods— In this monocentric, prospective, observational study, all consecutive patients with acute, MRI-confirmed, spontaneous CAD admitted to our center between August 2007 and August 2009 were included. VWI was defined as perivascular contrast enhancement in hrMRI and increased perivascular [18F]-fluorodesoxyglucose uptake in PET-CT. VWI was further differentiated between local (restricted to the site of dissection) and generalized (exceeding the site of dissection). Results— A total of 37 patients were included. Multiple dissections were seen in 10 patients (27%). Twenty-five patients received both modalities as planned, 8 received only PET-CT, and 4 received only hrMRI. A subset of patients showed signs of a generalized VWI in hrMRI (4/29 patients, 14%) and PET-CT (8/33 patients, 24%). In patients who received both modalities, all with hrMRI signs of generalized VWI were PET-CT positive (3/3), whereas some PET-CT–positive patients were hrMRI-negative (4/7). If present, generalized VWI in hrMRI completely resolved within 6 months. The presence of >2 simultaneous dissections (seen in 2 patients) was significantly associated with generalized VWI in hrMRI ( P =0.015) but marginally not in PET-CT ( P =0.053). Conclusions— A subset of patients with spontaneous CAD showed signs of a generalized transient inflammatory arteriopathy in contrast-enhanced hrMRI and PET-CT. This subset of patients may be more prone to multiple dissections.

In a lifetime experiment benzo(a)pyrene (B(a)P) was administered to Sprague-Dawley rats either as an admixture to the diet or by gavage in an aqueous 1.5% caffeine solution. Dissolved benzo(a)pyrene induced more tumors of the forestomach than undissolved benzo(a)pyrene. The 1.5% caffeine solution (annual dose 27 g/kg) did not exert any carcinogenic activity under the conditions of this bioassay.