Cerebral involvement in celiac disease: A serial MRI study in a patient with brainstem and cerebellar symptoms

Neurology ◽  
1997 ◽  
Vol 49 (5) ◽  
pp. 1447-1450 ◽  
Author(s):  
Angelo Ghezzi ◽  
Massimo Filippi ◽  
Andrea Falini ◽  
Mauro Zaffaroni
Keyword(s):  
Celiac Disease ◽  
Neuronal Damage ◽  
Magnetization Transfer ◽  
Cerebellar Atrophy ◽  
Cerebral Involvement ◽  
Cerebellar Involvement ◽  
Mri Study ◽  
Serial Mri ◽  
Cerebellar Symptoms ◽  
Csf Findings

A patient with celiac disease and relapsing-progressive symptoms suggesting brainstem and cerebellar involvement underwent serial MRIs. The first examination revealed multiple enhancing and nonenhancing lesions. Thereafter, a large enhancing cerebellar lesion appeared, followed by severe cerebellar atrophy. The presence of structural neuronal damage was confirmed by proton MR spectroscopy and magnetization transfer imaging. MRI results and CSF findings suggested that neurologic complications were more likely due to an inflammatory process.

scholarly journals Infratentorial MRI Findings in Rasmussen Encephalitis Suggest Primary Cerebellar Involvement

2021 ◽  
Vol 8 (6) ◽  
pp. e1058
Author(s):  
Johannes T. Reiter ◽  
Bastian David ◽  
Selma Enders ◽  
Conrad C. Prillwitz ◽  
Tobias Bauer ◽  
...  
Keyword(s):  
Diffusion Tensor ◽  
Cerebellar Atrophy ◽  
Patient Specific ◽  
Strongly Correlated ◽  
Rasmussen Encephalitis ◽  
Voxel Based Morphometry ◽  
Mri Findings ◽  
Cerebral Involvement ◽  
Fluid Attenuated Inversion Recovery ◽  
Cerebellar Involvement

Background and ObjectiveRasmussen encephalitis (RE) is characterized by its unilateral cerebral involvement. However, both ipsi- and contralesional cerebellar atrophy have been anecdotally reported raising questions about the nature and extent of infratentorial findings. Using MRI, we morphometrically investigated the cerebellum and hypothesized abnormalities beyond the effects of secondary atrophy, implicating a primary involvement of the cerebellum by RE.MethodsVoxel-based morphometry of the cerebellum and brainstem was conducted in 57 patients with RE and in 57 matched controls. Furthermore, patient-specific asymmetry indices (AIs) of cerebellar morphometry and fluid-attenuated inversion recovery (FLAIR) intensity were calculated. Using diffusion tensor imaging, the integrity of the cortico-ponto-cerebellar (CPC) tract was assessed. Finally, a spatial independent component analysis (ICA) was used to compare atrophy patterns between groups.ResultsPatients with RE showed bilateral cerebellar and predominantly ipsilesional mesencephalic atrophy (p < 0.01). Morphometric AIs revealed ipsilesional < contralesional asymmetry in 27 and ipsilesional > contralesional asymmetry in 30 patients. In patients with predominant ipsilesional atrophy, morphometric AIs strongly correlated with FLAIR intensity AIs (r = 0.86, p < 0.0001). Fractional anisotropy was lower for ipsilesional-to-contralesional CPC tracts than opposite tracts (T = 2.30, p < 0.05). ICA revealed bilateral and strictly ipsi- and contralesional atrophy components in patients with RE (p < 0.05).DiscussionWe demonstrated atrophy of the ipsilesional-to-contralesional CPC pathway and, consequently, interpret the loss of contralesional gray matter as secondary crossed cerebellar atrophy. The ipsilesional cerebellar atrophy, however, defies this explanation. Based on FLAIR hyperintensities, we interpret ipsilesional atrophy to be due to inflammation in the scope of a primary involvement of the cerebellum by RE.

scholarly journals CACNA1A-p.Thr501Met mutation associated with familial hemiplegic migraine: a family report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Marina Romozzi ◽  
Guido Primiano ◽  
Eleonora Rollo ◽  
Lorena Travaglini ◽  
Paolo Calabresi ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Clinical Presentation ◽  
Cerebellar Atrophy ◽  
Familial Hemiplegic Migraine ◽  
Autosomal Dominant Disorder ◽  
Hemiplegic Migraine ◽  
Rare Form ◽  
Targeted Capture ◽  
Cerebellar Involvement ◽  
Cerebellar Symptoms

Abstract Background and aims Hemiplegic migraine (HM) is a rare form of migraine characterized by the presence of a motor and other types of aura. HM can be sporadic or familial. Familial hemiplegic migraine (FHM) is an autosomal dominant disorder, classified into 3 subtypes, based on the gene involved (CACNA1A in FHM1, ATP1A2 in FHM2 and SCN1A in FHM3). The clinical presentation is highly heterogeneous and some attacks may be severe. We report the clinical characteristics and genetic analysis of 12 patients belonging to a family with CACNA1A-p.Thr501Met gene mutation. Methods We screened for mutations in CACNA1A gene 15 patients belonging to the same family. The exonic sequences of CACNA1A were analyzed using a Tru-seq® Custom Amplicon (TSCA) (Illumina Inc., San Diego, CA) targeted capture and paired end library kit. Sanger sequencing was used to confirm CACNA1A variants and segregation analysis. Results CACNA1A-p.Thr501Met mutation was found in 12 of the 15 patients screened, which was compatible with the diagnosis of FHM1. Attacks of hemiplegic migraine were reported by 10 of the 12 subjects (83.33%). Only one subject developed persistent mild cerebellar symptoms and none of the subjects developed cerebellar atrophy. Discussion The variant p.Thr501Met was described previously in association with episodic ataxia and rarely with FHM related to cerebellar symptoms. FHM1 has a broad clinical spectrum and about half of the families have cerebellar involvement. In our study, only one patient developed persistent cerebellar deficits. These data suggest that CACNA1A-p.Thr501Met mutation can occur prevalently as hemiplegic migraine.

Focal hyperperfusion and elevated lactate in the cerebral lesions with anti-GABAaR encephalitis: A serial MRI study

2020 ◽  
Vol 47 (3) ◽  
pp. 243-246 ◽  
Author(s):  
Hiroki Ueno ◽  
Takahiro Iizuka ◽  
Yuichiro Tagane ◽  
Fumiyuki Yamasaki ◽  
Yuji Akiyama ◽  
...  
Keyword(s):  
Cerebral Lesions ◽  
Mri Study ◽  
Serial Mri ◽  
Elevated Lactate

scholarly journals Progressive cerebral atrophy in multiple sclerosis A serial MRI study

Brain ◽  
1996 ◽  
Vol 119 (6) ◽  
pp. 2009-2019 ◽  
Author(s):  
N. A. Losseff ◽  
L. Wang ◽  
H. M. Lai ◽  
D. S. Yoo ◽  
M. L. Gawne-Cain ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Cerebral Atrophy ◽  
Mri Study ◽  
Serial Mri

scholarly journals Neuromyelitis Optica Spectrum Disorder Coinciding with Spinocerebellar Ataxia Type 31

2017 ◽  
Vol 9 (2) ◽  
pp. 127-130
Author(s):  
Yoshiaki Takahashi ◽  
Yasuhiro Manabe ◽  
Ryuta Morihara ◽  
Hisashi Narai ◽  
Toru Yamashita ◽  
...  
Keyword(s):  
Spinocerebellar Ataxia ◽  
Neuromyelitis Optica ◽  
Cerebellar Atrophy ◽  
Spectrum Disorder ◽  
Spinocerebellar Ataxia Type ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Inflammatory Processes ◽  
Thoracic Cord ◽  
The Brain ◽  
Cerebellar Symptoms

We report the unusual case of a 63-year-old man with spinocerebellar ataxia (SCA) type 31 who developed neuromyelitis optica spectrum disorder (NMOSD) 14 years after the onset of cerebellar symptoms. In addition to cerebellar atrophy, magnetic resonance imaging showed multiple high-intensity areas in the brain and a long thoracic cord lesion from Th1/2 to Th11. The combination of NMOSD and SCA31 is accidental. However, our case suggests that inflammatory processes could be involved in the pathogenesis of NMOSD and SCA31.

A conventional and magnetization transfer MRI study of the cervical cord in patients with MS

Neurology ◽  
2000 ◽  
Vol 54 (1) ◽  
pp. 207-207 ◽  
Author(s):  
M. Filippi ◽  
M. Bozzali ◽  
M. A. Horsfield ◽  
M. A. Rocca ◽  
M. P. Sormani ◽  
...  
Keyword(s):  
Magnetization Transfer ◽  
Cervical Cord ◽  
Magnetization Transfer Mri ◽  
Mri Study

scholarly journals Quantitative T1ρ and Magnetization Transfer Magnetic Resonance Imaging of Acute Cerebral Ischemia in the Rat

2002 ◽  
Vol 22 (5) ◽  
pp. 547-558 ◽  
Author(s):  
Heidi I. Mäkelä ◽  
Mikko I. Kettunen ◽  
Olli H. J. Gröhn ◽  
Risto A. Kauppinen
Keyword(s):  
Magnetic Resonance Imaging ◽  
Cerebral Ischemia ◽  
Magnetic Resonance ◽  
Neuronal Damage ◽  
Magnetization Transfer ◽  
Dependent Manner ◽  
Resonance Imaging ◽  
Prognostic Information ◽  
Acute Cerebral Ischemia ◽  
Ischemic Brain Tissue

It has been previously shown that T1 in the rotating frame (T1ρ) is a very sensitive and early marker of cerebral ischemia and that, interestingly, it can provide prognostic information about the degree of subsequent neuronal damage. In the present study the authors have quantified T1ρ together with the rate and other variables of magnetization transfer (MT) associated with spin interactions between the bulk and semisolid macromolecular pools by means of Z spectroscopy, to examine the possible overlap of mechanisms affecting these magnetic resonance imaging contrasts. Substantial prolongation of cerebral T1ρ was observed minutes after induction of ischemia, this change progressing in a time-dependent manner. Difference Z spectra (contralateral nonischemic minus ischemic brain tissue) showed a significant positive reminder in the time points from 0.5 to 3 hours after induction of ischemia, the polarity of this change reversing by 24 hours. Detailed analysis of the MT variables showed that the initial Z spectral changes were due to concerted increase in the maximal MT (+3%) and amount of MT (+4%). Interestingly, the MT rates derived either from the entire frequency range of Z spectra or the time constant for the first-order forward exchange (ksat) were unchanged at this time, these variables reducing only one day after induction of ischemia. The authors conclude that T1ρ changes in the acute phase of ischemia coincide with both elevated maximal MT and amount of MT. These changes occur independent of the overall MT rate and in the absence of net water gain to the tissue, whereas in the consolidating infarction the decrease in the rate and amount of MT, as well as the extensive prolongation of T1ρ, are associated with water accumulation.

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