Idiopathic Central Diabetes Insipidus Followed by Progressive Spastic Cerebral Ataxia

Long-Term MR Imaging Course of Neurodegenerative Langerhans Cell Histiocytosis

“…This association of atrophy with neuronal symptoms in patients with LCH has also been noted in other reports. 7,9,24,25 This observation is also in keeping with findings from multiple sclerosis where the progressive axonal loss remains clinically silent for many years until a threshold of axonal loss is reached, and the CNS is unable to compensate for the loss. 26 With the limited number of patients followed for an observation time of between 5 and 11 years, it remains an open question whether the radiologic progression of ND on MR imaging will ensue in neurologic symptoms at a later stage.…”

MRI features of neurodegenerative Langerhans cell histiocytosis

“…The dentate nuclei appeared as well-delineated curvilinear T2 hypointense and T1 hyperintense areas that were better depicted on coronal images displaying the evocative butterfly appearance. Calcifications in the dentate nucleus areas which have been previously described were absent in all of our patients who were also investigated by CT-scan [3,4,14,16]. Such calcifications may therefore not explain the T1 hyperintense signal observed in the dentate nuclei.…”

“…Tumour-like lesions correspond to an intracranial granulomatous infiltration leading to raised intracranial pressure, seizure and focal neurological deficits. They have been well-described in the imaging literature as single or multiple strongly enhancing lesions, appearing as white or grey matter supra or infratentorial masses, but also as extraparenchymal dural-, arachnoidal-, or choroid plexus-based lesions [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18]. ND-LCH is a less frequent and well-known complication than the classical histiocytic granuloma, characterized by a progressive cerebellar ataxia sometimes associated with a spastic tetraparesia, pseudobulbar palsy and cognitive decline, leading to severe disability and complete dependance [19].…”

18F-FDG PET in neurodegenerative Langerhans cell histiocytosis

“…Bilateral decreased 18 F-FDG uptake was observed in basal ganglia especially in the caudate nuclei (6/7 patients), in the cerebellum (especially in the vermis) (6/7 patients) and in the cerebral cortex (fronto temporal cortex) (5/7 patients). Areas of hypometabolism likely reflect neuronal loss and neurodegenerative lesions classically described in ND-LCH [10,[14][15][16]. While the functional changes detected with PET in the cerebellum were well correlated with the presence of lesions on MRI (all the patients had cerebellar white matter damage or cerebellar atrophy), only 3/6 patients with decreased uptake in the caudate nuclei and 0/4 in the frontal cortex had detectable abnormalities in these anatomical regions on MRI.…”

“…Interestingly, apart from hypothalamic hypermetabolism corresponding to the site of the granulomatous active process, the patient also displayed hypometabolism involving the caudate nuclei and small regions in the fronto-parietal cortex [17].Altogether, our results confirm to a larger extent the functional involvement in ND-LCH of the cerebellum,but also and this was less expected, the frequent involvement of the basal ganglia, especially the caudate nuclei, the frontal cortex, and the amygdalae. This may at least partly explain some cognitive deficits such as slow processing speed, shortterm memory, executive and attentional dysfunctions which have been displayed in ND-LCH (personal data) [14,18,19]. The diagnosis and extent of neuro-LCH are usually assessed by clinical examination and conventional neuroimaging.…”