Prevalence and vascular risk factors of basal ganglia calcifications in patients at risk for cerebrovascular disease

Background and purposeRisk factors for and meaning of basal ganglia calcifications outside Fahr syndrome are poorly understood. We aimed to assess the prevalence of basal ganglia calcifications and the association with vascular risk factors.Materials and methods1133 patients suspected of acute ischemic stroke from the Dutch acute stroke (DUST) study who underwent thin-slice unenhanced brain CT were analyzed. Basal ganglia calcifications were scored bilaterally as absent, mild (dot), moderate (multiple dots or single artery) and severe (confluent). Uni- and multivariable logistic regression analysis was used to determine possible risk factors (age, gender, history of stroke, smoking, hypertension, diabetes mellitus, hyperlipidemia, body mass index (BMI), renal function and family history of cardiovascular disease under 60 years) for presence of basal ganglia calcifications and ordinal regression analysis for severity of basal ganglia calcifications.ResultsMean age was 67.4 years (SD: 13.8), 56.8% were male. 337 (29.7%) patients had basal ganglia calcifications, of which 196 (58%) were mild, 103 (31%) moderate, 38 (11%) severe. In multivariable logistic regression analysis, age (OR: 1.02, 95% CI 1.01–1.03, P < 0.01) and BMI (OR: 0.95, 95% CI 0.91–0.98, p 0.01) were significantly associated with the presence of basal ganglia calcifications. Ordinal regression analysis gave comparable results. Age (OR: 1.02, 95% CI 1.01–1.03, P < 0.01) and BMI (OR: 0.95, 95% CI 0.92–0.99, P 0.01) were significantly associated with severity of basal ganglia calcifications.ConclusionsIn this study with patients suspected of acute ischemic stroke, basal ganglia calcifications were common and significantly associated with older age and lower BMI.     

Parkinson's disease and basal ganglia calcifications: prevalence and clinico-radiological correlations.

We reviewed computerized tomograms (CT) for basal ganglia and dentate nucleus calcifications in 79 patients with Parkinson's disease (PD), 54 patients with Alzheimer's disease (AD) and 109 controls aged 50 or more. When it was determined, no patient had disturbances in calcium metabolism. We found: (1) 30 subjects out of 242 (12.3%) with calcification located within the lenticular nucleus in 28. (2) Calcifications were unilateral in 11 and asymmetric in 11. (3) The prevalence of calcifications was 21.5% in PD, 9.2% in AD and 7.3% in controls and were significantly more severe in PD than in C and AD (P less than 0.02). (4) PD patients with calcifications were clinically indistinguishable from those without calcification. (5) Calcifications within the basal ganglia were not associated with a levodopa-resistance. We suggest the basal ganglia calcifications are more frequent in PD, but we cannot explain why, since post-synaptic lesions have never been showed in PD.     

Clinical significance of basal ganglia calcifications detected by CT: A retrospective study of 33 cases

In a retrospective study 2015 CT scans were studied for the presence of basal ganglia calcifications. These were discovered in 33 cases. No abnormalities in serum calcium or phosphate levels or any other specific clinical symptoms related to these calcifications were found. It is concluded that basal ganglia calcifications casually detected by CT generally have no clinical significance.     

Bilateral basal ganglia calcifications visualised on CT scan.

Thirty-eight cases of basal ganglia calcification imaged on computed axial tomography were reviewed. Most cases were felt to represent senescent calcification. The possibility of a vascular aetiology in this group is discussed. A less common group of patients was identified with calcification secondary to abnormalities in calcium metabolism or radiation therapy. Three cases of basal ganglia calcifications were detected in juvenile epileptic patients receiving chronic anticonvulsants. These cases may be related to abnormalities in calcium metabolism and alkaline phosphatase activity. Clinical evidence of basal ganglia abnormality was generally absent demonstrating the preservation of neuronal pathways in most cases.     

Calcification of the basal ganglia: computerized tomography and clinical correlation

During a 1-year period, 4219 consecutive computerized tomograms (CT) were reviewed for basal ganglia calcification; 14 patients with such calcification were identified. Calcifications on CT scan were bilateral in 12 of these cases and unilateral in 2. All bilateral calcifications were symmetric. The globus pallidus was the site of calcification in 13 of the 14 patients. Bilateral dentate nucleus calcification was seen in one patient. Skull radiograms were normal in all but one. Patients had diverse symptoms that were often explained by other findings, suggesting that calcifications may be coincidental and that basal ganglia calcification may not be a nosologic entity. Disturbances of calcium metabolism were not found in these patients, minimizing the pathophysiologic significance of altered calcium metabolism and the need for extensive endocrinologic evaluation. The finding of basal ganglia calcification alone does not justify invasive diagnostic procedures. Extrapyramidal signs may be associated with basal ganglia calcification; parkinsonism associated with basal ganglia calcification differs from idiopathic parkinsonism in being resistant to levodopa therapy.     

Basal ganglia calcifications in a case of biotinidase deficiency

Biotinidase deficiency leads to a biotin-deficient state, with cardinal symptoms of ataxia, alopecia, and skin rash presenting in infancy. Previous reports of head CTs in patients with biotinidase deficiency did not note basal ganglia calcifications. We report the first case of biotinidase deficiency with basal ganglia calcifications. There were no symptoms referable to basal ganglia dysfunction.     

A case of pseudohypoparathyroidism with intracerebral calcification

Intracerebral calcifications, especially in the basal ganglia, are observed in many kinds of diseases. A 41-year-old man is reported, who suffered from an acute epidural hematoma and underwent surgery to remove the hematoma. We detected very extensive intracerebral calcification on CT. Laboratory findings revealed hypocalcemia and hyperphosphatemia. General physical examination revealed characteristics typical of pseudohypoparathyroidism. The patient was diagnosed as having pseudohypoparathyroidism type I by the Ellsworth-Howard test. Since the advent of CT, the incidence of basal ganglia calcification has increased. CT is 5 to 15 times more sensitive than skull radiography in the detection of intracerebral calcification. Although many pathological states can cause basal ganglia calcification, most of the calcifications which are recognized on CT scans are physiological. But in cases in which basal ganglia calcifications are recognized also on plain radiographs, various kinds of symptoms including ones of basal ganglia origin are often recognized, and calcifications often extend to regions other than basal ganglia, eg. cerebellum, thalamus, etc. Pseudohypoparathyroidism is a rare disease which presents hypocalcemia, some characteristic physical appearances, and dementia. It is important to decide whether further examinations are necessary or not, when basal ganglia calcification is recognized incidentally on CT scan.     

Clinical correlations of CT scan-detected calcifications of the basal ganglia

A review of CT scans of 7,081 patients demonstrated calcifications of the basal ganglia in 53. The calcifications were evident in the skull roentgenograms of only 4 patients out of 40 in whom both CT scans and plain roentgenograms were available, demonstrating the superior resolution of this new method. Seventy-five percent of the patients were older than 50 years of age. Of the younger patients, 5 had had prior cranial irradiation; 1 had received cranial irradiation and intrathecal methotrexate therapy for meningeal leukemia; and 2 others had deep-seated arteriovenous malformations. Serum concentrations of calcium and phosphorus were normal in all 46 patients in whom they were measured. We conclude that the detection of small calcifications of the basal ganglia in persons above 50 years of age is infrequently associated with either clinical signs of basal ganglia dysfunction or calcium and phosphorus abnormalities. Calcium deposition in these patients may be related to vascular changes associated with aging. In younger patients a specific pathogenetic factor or underlying process is infrequently found.     

Prevalence of CT-detected cerebral abnormalities in an elderly Swedish population sample

Objective – To measure the prevalence of computed tomography (CT)‐detected cerebral lesions in a population‐based sample of elderly persons living in Göteborg, Sweden. Methods – Cerebral CT‐scans were performed in the case of 466 women (mean age 74.3 ± 5.1 years) and 191 70‐year‐old men. A single rater assessed white matter lesions (WML) using four different scales, lacunar lesions, large infarcts, cortical atrophy, and basal ganglia calcifications. Results – White matter lesions frequency assessed by different scales ranged between 54.5% and 68.5%. Lacunar lesions were detected in 46.7% (30.1% had lacunes >5 mm) and cerebral infarcts in 3.0% of participants. Overall, 72.8% of participants evidenced cerebral vascular abnormalities. Severe cortical atrophy was more common in temporal (6.4%) and frontal (6.7%) lobes, than in parietal (1.7%) and occipital (1.1%) lobes. Basal ganglia calcifications were found in 38.7% of participants. WML, lacunar lesions, large infarcts, and degree of cortical atrophy correlated positively with age. More lacunes, basal ganglia calcifications, and occipital lobe atrophy were associated with male gender. Conclusions – Vascular and other brain lesions are very common on CT‐scan in an elderly population, but large vascular lesions are rare. This study provides the first reference for the prevalence of CT‐detected abnormalities in an elderly Swedish population.     

Psychopathological alterations in cases of symmetrical basal ganglia sclerosis

Psychopathological alterations caused by symmetrical basal ganglia sclerosis of different etiologies are described, involving cases with parathyroid gland/hormone dysfunction (some of them familial), patients after thyroidectomy, and patients with basal ganglia calcification of uncertain etiology. Initial symptomatology in a group of 62 patients is reported; chronic symptoms in another group of 35 patients were evaluated. Estimates of volume of the basal ganglia calcifications were made, in addition to precise topographical localizations by CT. In 40% the initial symptoms noted were psychiatric, compared with 50% who first presented neurological symptoms. In the group of chronic cases practically all showed intellectual impairment. There was a marked preponderance of organic affective syndromes (initially 21%, chronic 65%): the affective chronic patients can be subdivided into 37% depressive, 20% bipolar, 11% manic cases. We could find no direct relationships with regard to etiology, localization, volume or symptoms, except that extensive calcifications occur after parathyroid hormone deficiencies due to thyroidectomy and lead to more severe mental deterioration.     

Basal ganglia calcifications in postoperative hypoparathyroidism: a case with unusual characteristics

A patient is described with post-thyroidectomy hypoparathyroidism and basal ganglia calcifications. The patient presented with a tonic-clonic seizure. The calcifications shown on CT scan were extensive in the basal ganglia, the bifrontal periventricular white matter and even in the brainstem. The white matter with calcifications displayed a high signal intensity on MRI, probably due to a different stage of the calcifying process in the white matter than in the basal ganglia. A severe dystonic reaction was observed after phenothiazine therapy and is discussed.     

Fahr's disease presenting with paroxysmal non-kinesigenic dyskinesia: a case report

Fahr's disease is characterized by presence of abnormal calcifications in certain areas of the brain. We report on 23-year-old man admitted to us with the episodes of paroxysmal non-kinesigenic dyskinesia. He was detected to have symmetrical intracerebral calcifications in basal ganglia, thalamus and cerebellar hemispheres, and diagnosed as sporadic Fahr's disease. Paroxysmal dyskinesia was well responded to oxcarbazepine (600 mg/day) treatment.     

A prospective study of patients with CT detected pallidal calcifications.

In a prospective study pallidal calcification was detected in 30 of 1478 (2%) adult patients, on CT brain scans. In 8 cases (26%), the calcifications were detected either years after, or during the course of, conditions known to cause basal ganglia calcification, including AIDS in four cases. Eight patients (three with AIDS) had disturbances of calcium and phosphorus metabolism. It was concluded that: a) pallidal calcification is not uncommon and aetiological factors may be recognised more often than previously reported; b) AIDS is emerging as a significant cause of pallidal calcification in young adults, and c) in AIDS and other conditions, abnormal calcium and phosphate metabolism may act in conjunction with local vascular changes.     

Four cases of Cockayne syndrome

The evaluation of four patients with Cockayne syndrome (CS) by computed tomography (CT) and magnetic resonance imaging (MRI) is reported. All patients had characteristic clinical manifestations of CS. In a special respect, we found hyperopia in two patients and previous habitual abortions in two maternal histories. Extrapyramidal signs were seen in one patient. Three patients are type 1 CS (case 1, 3, 4) and one patient (case 2) is type 2 CS (congenital form). The cranial CT in two patients (case 1, 2) revealed prominent calcifications in basal ganglia, dentate nucleus and hemispheric white matter. While CT showed vagal calcifications in basal ganglia in other two patients (case 3, 4), T2-weighted MRI revealed obvious low intensity area in putamen and caudate nucleus, and high intensity area in the white matter. Sagittal section revealed atrophic changes of cerebellar vermis and brain stem. Thus it seems that MRI may be useful diagnostic adjunct in CS patients.     

Familial form of Fahr syndrome (report of two cases)

Two cases of familial form (in brothers) of extensive, symmetric, idiopathic calcifications of the basal ganglia and cerebellum (Fahr syndrome) are described. In discussion of factors predisposing to appearance of such idiopathic calcifications, particular attention was given to meningoencephalitis suffered in childhood and coexistence of a tumour of the sella of prolactinoma type, in case 1. Attention was also called to progressive, copious clinical and radiological symptology of the discussed cases. It was impossible to suggest a probable type of inheritance (parents of the patients were dead).     

Distinct basal ganglia hyperechogenicity in idiopathic basal ganglia calcification

We report a 67‐year‐old patient with idiopathic basal ganglia calcification (IBGC). He presented with progressive cognitive impairment, frontal lobe dysfunction, mild leg spasticity, and levodopa (L ‐dopa)‐responsive parkinsonism. Transcranial sonography (TCS) revealed marked hyperechogenicity of the basal ganglia and periventricular spaces bilaterally. The detected signal alterations showed a fairly symmetric distribution and corresponded to the hyperintense calcifications depicted on the computer tomography brain scan. The combination of symmetric hyperechogenic areas adjacent to the lateral ventricles and of the basal ganglia may serve as an imaging marker characteristic of IBGC. Hyperechogenicity due to extended basal ganglia calcification as presented here is distinct from the pattern of hyperechogenicity caused by heavy metal accumulation, which is described to be less striking. In addition to atypical parkinsonian syndromes such as progressive supranuclear palsy and multiple system atrophy, IBGC is thus another differential diagnosis of parkinsonism with basal ganglia hyperechogenicity. © 2010 Movement Disorder Society.     

High densities of tumor necrosis factor-α in the cerebral cortex and basal ganglia in human immunodeficiency virus-1-associated cognitive/motor complex: A quantitative regional analysis study

This study determined the regional distribution of tumor necrosis factor-alpha (TNF-α)-positive cells in HIV-1-infected brains and controls, and evaluated its correlation to cognitive impairment in HIV-1-associated cognitive/motor complex (HCMC) using TNF-α and glial fibrillary acidic protein (GFAP) double immunohistochemistry. The TNF-α-positive cell density in the cerebral cortex and basal ganglia was significantly increased in patients with HCMC compared with that in HIV-1-seropositive nondemented patients (non-HCMC), and the basal ganglia TNF-α-positive cell density was significantly correlated with Mini-Mental Status Examination (MMSE) score. Glial fibrillary acidic protein-positive astrocytes were significantly increased in density compared with normal controls, but no significant difference between HCMC and non-HCMC patients was obtained. The density of GFAP-positive astrocytes and TNF-α-positive cells was well correlated other than in the basal ganglia. The basal ganglia TNF-α-positive cell densities showed a significant correlation with MMSE scores. The result suggests that the significantly high content of TNF-α-positive cells in the cortex and basal ganglia is a neurochemical feature characteristic of HCMC. The significant correlation of the basal ganglia TNF-α-positive cell density with degree of cognitive impairment might be associated with subcortical dementia during HIV-1 infection.     

Psychosis revealing familial idiopathic basal ganglia calcification

We describe the case of a 39-year-old woman presenting with auditory hallucinations and delusions responsive to antipsychotic drugs. Computerized tomography scans revealed basal ganglia calcifications in the proband and in her two asymptomatic parents. Extensive etiological clinicobiological assessment allowed us to exclude known causes of brain calcifications and diagnose familial idiopathic basal ganglia calcification (IBGC).     

Neuropsychiatric and brain CT findings in polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

Seven patients with polycystic lipomembranous osteodysplasia and sclerosing leukoencephalopathy (PLO-SL or membranous lipodystrophy) were examined clinically and with CT scanning. The degree of dementia was severe in five cases, marked in one case, and mild in one case. All patients suffered from marked or severe prefrontal psychosyndrome, impairment of memory and signs of upper motor neuron involvement. Epileptic seizures and primitive reflexes were observed in six cases. Degree of agnostic-aphasic-apraxic symptoms varied. In all cases CT revealed general and diffuse brain atrophy, which was accentuated in frontal areas. In the basal ganglia bilateral calcifications (five cases) or a slight hyperdensity (two cases) were detected. CT finding in PLO-SL may lead to an early diagnosis of this fatal disease.     

Children with irreversible brain damage associated with hypothyroidism and multiple intracranial calcifications

Children who develop clinical hypothyroidism in early childhood have various degrees of irreversible brain damage, albeit less severe than cases detected by neonatal screening test for hypothyroidism in the first months of the life. We report three patients with hypothyroidism of childhood onset after a normal neonatal thyroid-stimulating hormone screening who showed deceleration in linear growth, spasticity in the lower limbs with deformity, mild intellectual impairment, and multiple calcifications in the basal ganglia and subcortical areas. The neurologic symptoms were not progressive but were irreversible in spite of thyroxine treatment. Motor disturbances commonly observed in postnatal-onset hypothyroidism are similar to those of cerebral palsy. Specific distribution of intracranial calcifications may result from metabolic derangement as a result of hypothyroidism, although the mechanism of calcification is not fully understood. We emphasize the need to re-evaluate thyroid function in diplegic patients with specific intracranial calcifications but normal neonatal thyroid-stimulating hormone screening.