26 September 2018

CLIPPERS - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids


T1 GD+ sequences show multiple punctate and linear enhancing lesions in pons and medulla oblongata representing perivascular enhancing lymphatic tissue.


Enhancing punctate lesions are also present in corona radiata on both sides. FLAIR sequence showing corresponding white matter lesions that however present no significant oedema.

Findings represent CLIPPERS - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Rare entity caused by infiltration of the brain with inflammatory cells in perivascular areas, mostly found in brainstem.

More comprehensive description of CLIPPERS in Radiopaedia





04 March 2017

Huntington Disease


10 years old boy from parents being cousins to each other presents with progressive rigidity and dystonia. Initial MR showing significant bilateral atrophy in caudate nuclei and putamina with high signal on FLAIR and T2 consistent with gliosis.


Note striking symmetric atrophy of the putamina and caudate nuclei on T2 and IR.

Huntington disease (HD) is also known as Huntington chorea. HD is an autosomal dominant chronic hereditary neurodegenerative disorder with complete penetrance [Osborn]. 

Aggregates of huntingtin protein accumulate in axonal terminals, which eventually leads to the death of medium spiny neurons. Autopsy shows generalized cerebral atrophy with an average of 30% reduction in brain weight. Both the cortex and hemispheric WM are affected. The most characteristic gross abnormality is volume loss with rarefaction of the caudate nucleus, putamen, and globus pallidus [Osborn]. 

Microscopically, HD features neuronal loss with huntingtin nuclear inclusions, astrocytic gliosis, and iron accumulation. The changes are most severe in the basal ganglia but can also be seen in other regions of the brain, including the cerebellum [Osborn].

Juvenile-onset HD is initially characterized by rigidity and dystonia, much more than by chorea [Osborn].




26 February 2017

Intracranial Hypotension with Dural Sinus Thrombosis



Contrast enhanced T1 sequences showing large thrombi in the posterior parts of the superior sagittal sinus (SSS). However note that this young female patient in postpartum period is also presenting with classical signs of Intracranial Hypotension. There is general dura enhancement, swelling of hypophysis and slight sagging of the cerebellum. 



Transversal SWI and T2 show thrombus in SSS and swelling of the cortical veins. 



Sagittal and transversal FLAIR sequences show thrombus with higher signal that is obstructing (black ) flow-voids in SSS. 


It is believed that Intracranial Hypotension (that is most often caused by CSF leakage) is the reason for slowing down the venous blood flow in the dural sinus. However note that Dural Sinus Thrombosis (DST) is a very seldom complication of Intracranial Hypotension (2%). Most often does DST manifest as Intracranial Hypertension (20-40%).

Check my previous cases:
CSF Leakage - Intracranial Hypotension
Intracranial Hypotension
CSF Leakage Spine - MRI Protocol
Idiopathic Intracranial Hypertension


Capillary Telangiectasia


Classic case of an incidental finding of a small Capillary Telangiectasia in the pons. Note low signal on SWI and diffuse subtle contrast enhancement on T1. Location is very typical but other locations are common. 


On T2 this small Capillary Telangiectasia is hardly visible, without any signs of oedema. There is a blush of enhancement on T1C+ coronal image. 



Carbon Monoxide Poisoning - infarctions in globi pallidi


82 years old admitted with signs of intoxication, history of suicide attempt. NECT shows symmetric low density oedematous globi pallidi (GP) - compared to old CT.




Note low density in globi pallidi on the admission NECT compared to old CT. 



MR exam has shown diffusion restriction in globi pallidi (high signal in GP on this rather dark DWI and low signal on ADC) corresponding with acute infarctions.



FLAIR and T2 show oedema in globi pallidi corresponding with acute infarctions. 




Contrast enhanced T1 sequence (right) shows a very subtle enhancement in both globi pallidi consistent with blood-brain barrier damage.