Microvasculopathy May Precede Idiopathic Cerebral Calcifications — Case Report

Gomez, Camilo R.; Ĺuque, A.; Horenstein, Simon

doi:10.1177/000331978904000113

Cited by 20 publications

(14 citation statements)

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“…The clinical manifestations are presented in Table 1 and are compared with those reported in the literature. Sixty‐one patients diagnosed with BSPDC from 20 published studies included 43 patients from nine families with autosomal dominant inheritance,16–23 12 patients from five families were grouped under “familial” (where more than one person was affected without a clear pattern of inheritance),24–28 and six were sporadic cases 15, 31–34…”

Section: Resultsmentioning

confidence: 99%

“…All previously published case histories of diagnosed BSPDC obtained through literature search were included if they met the inclusion criteria. Case histories from 20 publications met the criteria 15–34. Inheritance patterns displayed in the published case histories were compared with the inheritance patterns displayed by the five families studied through the Registry.…”

Section: Methodsmentioning

confidence: 99%

“…It has been suggested that the hyperintense T2‐weighted images seen on magnetic resonance imaging (MRI) scan may reflect a slowly progressive metabolic or inflammatory process in the brain, which subsequently calcifies and is probably responsible for the neurologic deficits observed 14. It has also been suggested that accumulation of iron and calcium occurs in response to the extravascular deposition of an acid mucopolysaccharide‐alkalic protein complex, and MRI studies have suggested that vascular membrane abnormalities may be responsible for the leakage of plasma‐derived fluid, and this in turn may damage the neuropil and result in mineral accumulation 15…”

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confidence: 99%

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Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

2001

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Clinical features in bilateral striopallidodentate calcinosis (BSPDC), popularly referred to as Fahr's disease (five autosomal dominant families and eight sporadic cases, n = 38), recruited through a registry, are reported. Applying uniform criteria, cases reported in the literature (n = 61) were combined for detailed analysis. The mean (+/- S.D.) age of Registry patients was 43 +/- 21 and that of literature was 38 +/- 17. In combined data set (n = 99), 67 were symptomatic and 32 were asymptomatic. Of the symptomatic, the incidence among men was higher compared with women (45:22). Movement disorders accounted for 55% of the total symptomatic patients. Of the movement disorders, parkinsonism accounted for 57%, chorea 19%, tremor 8%, dystonia 8%, athetosis 5%, and orofacial dyskinesia 3%. Overlap of signs referable to different areas of central nervous system (CNS) was common. Other neurologic manifestations included: cognitive impairment, cerebellar signs, speech disorder, pyramidal signs, psychiatric features, gait disorders, sensory changes, and pain. We measured the total volume of calcification using an Electronic Planimeter and Coordinate Digitizer. Results suggest a significantly greater amount of calcification in symptomatic patients compared to asymptomatic patients. This study suggests that movement disorders are the most common manifestations of BSPDC, and among movement disorders, parkinsonism outnumber others.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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confidence: 99%

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Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

2001

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“…Estas enfermedades generan una alteración en el metabolismo de electrolitos que conllevan a su depósito progresivo, fundamentalmente, de fosfato de calcio en forma de hidroxiapatita, el mismo complejo de fosfato de calcio presente en los huesos (15) . El mecanismo aún es desconocido; se sugiere que la acumulación de calcio y hierro ocurre en respuesta al depósito extravascular de un complejo proteico mucopolisacárido; también, se postula que las anormalidades de la membrana vascular podrían ser responsables del escape de fluidos derivados del plasma que podrían dañar el tejido nervioso y causar acumulación de sales (16) , desencadenando con el paso del tiempo la presentación de síntomas y signos que obligan al paciente a solicitar ayuda médica. Esto ocurrió en nuestra paciente, 27 años después de la tiroidectomía, cuando al parecer de manera involuntaria ocurrió una resección bilateral de las paratiroides.…”

Section: Discussionunclassified

Calcificaciones cerebrales extensas y corea, en hipoparatiroidismo probablemente posquirúrgico

et al. 2012

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ResumenIntroducción: Las calcificaciones cerebrales pueden ser unilaterales o bilaterales; las unilaterales presentan como etiología las infecciones, infartos o traumatismos; las bilaterales pueden ser fisiológicas, metabólicas o idiopáticas. Caso clínico: Paciente mujer, de 48 años de edad, tiroidectomizada hace 27 años, tomaba levotiroxina; acudió por movimientos involuntarios coreicos y balísticos progresivos y dificultad para caminar. Se encontró hipotonía de hemicuerpo derecho y movimientos serpenteantes de miembro superior derecho, hipocalcemia, hiperfosfatemia y concentración baja de parathormona; la tomografia y resonancia magnética cerebrales evidenciaban calcificaciones bilaterales en ganglios basales y cerebelo. Recibió tratamiento con citrato de calcio, calcitriol y risperidona, con recuperación completa. Discusión: Los pacientes con calcificaciones de ganglios basales presentan síntomas extrapiramidales (20 a 30%), como parkinsonismo o coreoatetosis, convulsiones y trastornos neuropsiquiátricos. Las calcificaciones intracerebrales bilaterales se producen generalmente por depósito de calcio y otros minerales. El hipoparatiroidismo es una complicación frecuente en la tiroidectomía radical, con hipocalcemia, hiperfosfatemia y concentración baja de parathormona como el caso descrito. Algunos pacientes no desarrollan los síntomas de inmediato. El tratamiento mejora los síntomas sin alterar las calcificaciones, como en la paciente, en quien la mejoría sería por la normalización del calcio más que por la risperidona. Palabras clave: Patologías cerebrales; movimiento coreiforme; hipoparatiroidismo; risperidona. AbstractIntroduction: Cerebral calcifications may be unilateral or bilateral; unilateral etiology may include infections, trauma or stroke and bilateral may be physiologic, metabolic or idiopathic. Case report: Female 48 year-old patient who had thyroidectomy 27 years before, received levothyroxine, and who was attended for chorea, ballistic involuntary movements and progressive difficulty in walking. She presented right sided hypotonia, right upper limb winding movements, hypocalcemia, hyperphosphatemia and low parathormone levels; tomography and magnetic resonance showed bilateral calcifications in brain basal ganglia and cerebellum. She was treated with calcium citrate, calcitriol and risperidone with complete recovery. Discussion: Patients with basal ganglia calcifications have extrapyramidal symptoms (20-30%) such as parkinsonism or coreoatetosis, seizures, and neuropsychiatric disorders. Brain bilateral calcifications usually consist in calcium and other minerals deposit. Hypoparathyroidism is frequent complication of radical thyroidectomy accompanied with hypocalcemia, hyperphosphatemia and parathormone low concentration as the case described. Some patients do not develop symptoms immediately. Treatment improves symptoms but not calcifications; in our patient improvement would be due to calcium normalization more than risperidone treatment.

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“…Since hemorrhagic infarcts [13] and intracerebral hematomas [14] may calcify, embolic infarcts that are more likely to undergo hemorrhagic transformation may also be more likely to accumulate calcium. Microvascular ischemia with resultant alterations of the blood-brain barrier has been implicated in calcifications of the basal ganglia [15].…”

Section: Discussionmentioning

confidence: 99%

Partial Resolution of Calcifications in Cerebral Infarcts in a Patient with Lupus

Futrell¹

1993

Journal of Neuroimaging

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Microvasculopathy May Precede Idiopathic Cerebral Calcifications — Case Report

Cited by 20 publications

References 9 publications

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry

Calcificaciones cerebrales extensas y corea, en hipoparatiroidismo probablemente posquirúrgico

Partial Resolution of Calcifications in Cerebral Infarcts in a Patient with Lupus

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