ABSTRACT
INTRODUCTION: Fahr's syndrome is an infrequent disorder characterized by bilateral symmetrical calcification of basal ganglia and the cerebral cortex. It can be seen genetic, idiopathic, or secondary to endocrine diseases. This disease is related to different metabolic disorders particularly with diseases of the parathyroid gland. CASE 1: A 63-year-old female patient applied to our clinic due to having hypoparathyroidism with bilateral basal ganglia calcification in head computed tomography(CT). She had subtotal thyroidectomy 25 years ago. In the neurological examination, mild symmetrical parkinsonism was determined. In laboratory examination Ca:8 mg/dL (8.6-10.2), P:5.1 mg/dL (2.3-4.5), PTH:9.53 pg/mL (15-65) were detected. Calcitriol 0.25 µ/day was added to her treatment. Her parkinsonism disappeared after the treatment. CASE 2: A 49-year-old male patient was consulted when he was admitted to the department of neurology in our hospital. The physical examination demonstrated the characteristics of Albright's hereditary osteodystrophy. The neurological examination shows bilateral symmetrical bradykinesia, dysphagia, and moderate dysarthria. In the laboratory examination PTH: 46.5 ng/L(15-65), Ca:8.6 mg/dL (8.6-10.2), P:2.7 mg/dL (2.3-4.5) were detected and were all within the normal ranges. Consequently, pseudopseudohypoparathyroidism was decided as a diagnosis. G protein alpha subunit mutation (Gsα) was not detected due to technical limitations. CONCLUSION: When a patient is diagnosed as Fahr's syndrome, we should keep in mind parathyroid disorders. Fahr's syndrome must be evaluated in patients showing intracranial calcification accompanied by parathyroid diseases.
ABSTRACT
Our aim was to investigate whether neurological impairment in chronic Behçet's disease (BD) patients with normal appearing brain can be assessed by means of diffusion-weighted imaging (DWI). The averaged apparent diffusion coefficient (ADC) values were calculated in 22 different radiologically normal appearing brain regions in 32 patients with and without neurological findings and 20 control subjects. The ADC values in bilateral frontal, temporal and occipital normal appearing white matter were significantly higher in the patient groups compared with the control subjects (p < 0.05). In these brain regions, DWI revealed differences in the ADC values between patients with neurological findings (including symptomatic and neuro-Behçet patients) and the asymptomatic patient group. The similarity of the ADC values of patients without symptoms to those of the control group allowed clear discrimination between patients with and without neurological findings. DWI may serve to assess subclinical neurological involvement in BD, even when structural changes are absent.
Subject(s)
Behcet Syndrome/complications , Behcet Syndrome/metabolism , Brain/metabolism , Nervous System Diseases/metabolism , Adolescent , Adult , Behcet Syndrome/pathology , Brain/pathology , Case-Control Studies , Diffusion , Diffusion Magnetic Resonance Imaging , Female , Humans , Male , Middle Aged , Nervous System Diseases/etiology , Nervous System Diseases/pathologyABSTRACT
Our aim was to investigate whether neurological impairment in Behçet's disease (BD) can be assessed by means of proton MRS and whether it can assist in prognosis. We used single-voxel MRS to measure metabolites in regions of normal-appearing pons, basal ganglia and periventricular white matter (PWM) in 32 patients with chronic BD patients with and without neurological deficits and 29 control subjects. Patients had significantly higher N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the basal ganglia than the controls. The Cho/Cr ratio in the PWM was also significantly higher in the patients. MRS enabled clear discrimination of patients and controls and also revealed spectral differences between non-neuro-Behçet's disease and neuro-Behçet's disease in the basal ganglia. MRS can be used to assess brain involvement in BD even if structural changes are absent.