2001-07-05-12 Bilateral striatal necrosis © Giuffrida www.thefetus.net/
Bilateral striatal necrosis
Alessandro Giuffrida, MD, Saveria Cantone, MD., Fabiola Galvani, MD, Claudio Giorlandino, MD.
Definition: Bilateral striatal necrosis is a symmetrical degeneration of the caudate nucleus and putamen sometimes the globus pallidus, substantia nigra and tegmental nuclei
Synonymous: Striatonigral degeneration, infantile; IBSN striatal degeneration, familial
Prevalence: In a nationwide survey of Japanese patients, it is estimated in 0.007:10,000 births.
Etiology: There are several possible etiologies:
Onset often occurs after a febrile event,, (possibly mediated by a hypoxic-ischemic) with nausea and vomiting. The initial even can be caused by Mycoplasma Pneumoniae infection,,, measles,, wasp sting, E. coli endotoxin, cardiac arrest, or Sydenham"s chorea
The infantile striatonigral degeneration that can be familial, autosomal recessive,
Possible mitochondrial DNA mutation of the mitochondrial ATPase 6 gene,
Pathogenesis: Although metabolic changes in the developing corpus striatum may be important in the pathogenesis of Infantile bilateral striatal necrosis, the origin of the pathogenetic mechanism is uncertain. In week old piglets subjected to hypoxia/ischemia, glutathione depletion and decrease in Na,K-ATPase activity occur in the affected region. Others have suggested that glutamate excitotoxicity mediated by glutamate receptors 1, 2/3, and 4 and excessive dopaminergic excitatory activity may play important roles in hypoxic-ischemic basal ganglia necrosis but that neuronal nitric oxide synthase does not contribute to that condition.
Ultrasound diagnosis: The video and the pictures offer a better and easier way to understand what is possible to see during a sonographic examination. In the frontal scanning (picture 1) it is possible to see some areas that represent necrosis of the nucleus arcuate; the pathology is easily recognizable in the sagittal scanning (picture 2) and most of all watching the video. The CT image (after birth) can confirm the prenatal diagnosis. Initial computed tomography scans can show mild atrophy of the caudate nuclei or basal ganglia, and the neuropathologic examination can reveal diffuse neuronal loss with some patchy preservation and marked astrogliosis in the striatum and globus pallidus.
This third trimester patient was scanned transvaginally. There is a 1 MB video-clip one parasagittal image
and a antero-posterior sequence of coronal views
And one view of the post-delivery CT
Clinical presentation after birth: Paterson and Carmichael first described the disorder in 1924. Depending on different pathologic and clinical features we can also distinguish: early, acute onset, early, gradual onset and late onset. The neurological symptoms includes symmetrical degeneration of caudate nucleus, putamen and sometimes the globus pallidus are the main neurologic finding, resulting in (mostly) extrapyramidal signs,,:
- Abnormal eye movements,
- Mental retardation,
- Regression of motor and verbal skills,
- Impaired consciousness,
- Cerebellar ataxia,
Associated pathologies: Similar neurological symptoms can also occur in Leber syndrome (Leber hereditary optic neuropathy), Leigh syndrome (subacute necrotizing encephalomyelopathy), familial dystonia and choreoathetosis and Huntington’s chorea; this could suggest a possible origin of the pathology in a mitochondrial DNA mutation. There is also a case of associated congenital adrenal hypoplasia and acute bilateral infantile striatal necrosis.
Differential diagnosis: De Morsier syndrome (septooptic dysplasia) or ventriculomegaly (just for beginners).
Prognosis: It depends on the severity of the neurological symptoms but an important rehabilitation is always needed.
Management: If detected early, pregnancy termination can be offered, otherwise an important rehabilitation is always needed. In children corticosteroid therapy can be attemptedand there is some research using monoamine oxidase inhibitors.
This case was originally posted at /www.ilfeto.it/ by Artemisia medical center
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