diagnosis reported as 3 years in patients with grade II BLTG (2,10).We present the case of a BLTG manifesting with fast progressive cognitive dysfunction leading to dementia in an elderly patient. █ CASE REpORTA 72-year-old right handed male, treated only for essential arterial hypertension stage I, presented 3 months prior to admittance, with gradual deterioration of the cognitive functions and intermittently aggressive behavior towards close relatives. The symptoms progressed gradually, with the patient not being able to perform basic everyday tasks and presenting periods of extreme agitation. █ INTRODUCTIONPrimary thalamic gliomas are rare and have been presented in recent reports with an incidence of 0.84-5.2% among all intracranial tumors (2,4,15,18).Bilateral thalamic gliomas (BLTG) constitute a unique intracranial tumoral pathology typically manifested by varying degrees of personality disorder. The rate of BLTG is much lower than that of unilateral thalamic glioma and cannot be objectively expressed in any way at present (4-7,13).Imaging in BLTG is typical and reveals large tumors with symmetrical and strictly limited involvement of bilateral thalami, without any apparent connecting tumoral tissue (2).The prognosis of BLTG is poor and a rapid fatal evolution has been observed (14) with the longest period of survival after the Bilateral thalamic glioma is extremely rare and the incidence cannot be adequately expressed. We present the case of a 72 years old male suffering from the rapid deterioration of cognitive function to moderately severe dementia in a short period of time. Magnetic resonance studies demonstrated a bilateral thalamic glioma with a minimal focal gadolinium uptake in the left thalamus. Biopsy was performed and pathology report was of anaplastic astrocytoma, WHO grade III. Radiotherapy was proposed but was rejected by the patient's relatives. The patient deceased 57 days later. We performed an extensive review of the literature and by updating the previous described series we can state that to the best of our knowledge this is the 60th case described in the literature and the second eldest patient presented. Patients suffering from this disease present a poor prognosis, the longest survival described being of 3 years in patients diagnosed with grade II bilateral thalamic glioma. Adjuvant therapy in form of radiotherapy to the thalami is most commonly used but the benefits are unclear. The natural progression of WHO grade III bilateral thalamic glioma left untreated, as can be seen from our case, has an even poorer outcome.

Introduction. Creutzfeldt-Jakob Disease (CJD) is a very rare neurodegenerative disease with no cure that always leads to death. It is the most frequent prion disease and has multiple recognized forms, from which the sporadic form (sCJD) is the most common. The defining histological characteristic of CJD is the accumulation of the abnormal prion protein (PrPSc) along with neuronal loss and spongiform vacuolation. Typical neurological signs found in sCJD are rapidly progressive dementia, myoclonus, visual disturbances, pyramidal signs, extrapyramidal signs, behavioral changes and akinetic mutism. Material and method. We performed a retrospective study over the 2007-2017 period that included five cases of patients diagnosed with probable sCJD from our department. Results. The most common findings in the patients’ MRI were cerebral atrophy and ventricular enlargement, but we also found typical MRI changes such as increased T2 and FLAIR signal intensity in the head of the caudate nucleus, putamen and cerebral cortex. Repeated electroencephalograms showed typical sCJD patterns like periodic sharp wave complexes, mostly with biphasic or triphasic aspect. A complex differential diagnosis and numerous routine investigations (CBC, biochemical analysis, autoimmune panel, HIV antibodies, VDRL, anti-Borrelia antibodies, anti-TPO antibodies, anti-neuronal antibodies, B12 vitamin) were performed, without indicating an alternative diagnosis. All the patients died several months after the onset of symptoms. Conclusions. Given the rarity of the disease and the broad differential diagnosis, CJD proves to be a diagnostic challenge. In the absence of an alternative diagnosis, the association of rapidly progressive dementia, myoclonus, visual and cerebellar signs are highly suggestive for sCJD.

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Acute intermittent porphyria (AIP) is a metabolic disease with an autosomal dominant inheritance, with porphobilinogen (PBG) deaminase as the deficient enzyme in heme biosynthetic

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