Search :     
Articles » Central nervous system
2001-07-05-12 Bilateral striatal necrosis © Giuffrida

Bilateral striatal necrosis

Alessandro Giuffrida, MD, Saveria Cantone, MD., Fabiola Galvani, MD, Claudio Giorlandino, MD.

Artemisia, Rome

Definition: Bilateral striatal necrosis is a symmetrical degeneration of the caudate nucleus and putamen sometimes the globus pallidus, substantia nigra and tegmental nuclei[1]

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[2].

Etiology: There are several possible etiologies[3]:

  • Onset often occurs after a febrile event[4],[5],[6] (possibly mediated by a hypoxic-ischemic) with nausea and vomiting. The initial even can be caused by Mycoplasma Pneumoniae infection[7],[8],[9], measles[10],[11], wasp sting[12], E. coli endotoxin[13], cardiac arrest[14],[15] or Sydenham"s chorea[16]

  • The infantile striatonigral degeneration that can be familial, autosomal recessive[17],[18]

  • Possible mitochondrial DNA mutation[19] of the mitochondrial ATPase 6 gene[20],[21]

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[22] and decrease in Na,K-ATPase activity occur in the affected region[23]. 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[24].

 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[25]. Depending on different pathologic and clinical features we can also distinguish: early, acute onset[26], early, gradual onset[27] 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[28],[29],[30]:

  • Choreoathetosis,
  • Dystonia
  • Rigidity
  • Abnormal eye movements,
  • Seizures[31],
  • Mental retardation,
  • Regression of motor and verbal skills,
  • Impaired consciousness,
  • Myoclonus,
  • Quadriparesis,
  • Cerebellar ataxia,
  • Nystagmus.

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[32].

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 attempted[33]and there is some research using monoamine oxidase inhibitors[34].


This case was originally posted at / by Artemisia medical center

[1] Roytta M, Olsson I, Sourander P, Svendsen P.Infantile bilateral striatal necrosis. Clinical and morphological report of a case and a review of the literature. Acta Neuropathol (Berl) 1981;55(2):97-103

[2] Hirayama, K.; Takayanagi, T.; Nakamura, R.; Yanagisawa, N.; Hattori, T.; Kita, K.; Yanagimoto, S.; Fujita, M.; Nagaoka, M.; Satomura, Y.; Sobue, I.; Iizuka, R.; Toyokura, Y.; Satoyoshi, E. : Spinocerebellar degenerations in Japan: a nationwide epidemiological and clinical study. Acta Neurol. Scand. 89 (suppl. 153): 1-22, 1994.

[3] Mito, T.; Tanaka, T.; Becker, L. E.; Takashima, S.; Tanaka, J. :  Infantile bilateral striatal necrosis: clinicopathological classification. Arch. Neurol. 43: 677-680, 1986.

[4] Bhaumik S, Behari M, Ahuja GK.Bilateral basal ganglia lucencies following acute febrile illness. Indian J Pediatr 1996 Jul-Aug;63(4):557-60

[5] Torre Md, Calado E, Macedo A, Ventura L, Cabral P.Acute infantile bilateral striatal necrosis . Acta Med Port 1997 Feb-Mar;10(2-3):205-7

[6] Yasukohchi S, Akabane T, Mori T, Tamagawa K, Morimatsu Y.A case report of infantile striatal necrosis with an acute onset. Brain Dev 1986;8(6):609-14

[7] Larsen PD, Crisp D Acute bilateral striatal necrosis associated with Mycoplasma pneumoniae infection. Pediatr Infect Dis J 1996 Dec;15(12):1124-6

[8] Noseda G, Harpey JP, Brandel JP, Roy C, Caille B, Prudent M, Maillard L, Renault F, Leveau J, Malafosse M.Acute basal ganglia necrosis with favorable course during Mycoplasma encepahlitis . Arch Pediatr 1996 Nov;3(11):1107-10

[9] Brandel JP, Vidailhet M, Noseda G, Harpey JP, Agid Y.Mycoplasma pneumoniae postinfectious encephalomyelitis with bilateral striatal necrosis. Mov Disord 1996 May;11(3):333-6

[10] Cambonie G: Infantile bilateral striatal necrosis following measles. - Brain Dev - 2000 Jun; 22(4): 221-3

[11] Zambrino CA, Zorzi G, Lanzi G, Uggetti C, Egitto MG. Bilateral striatal necrosis associated with Mycoplasma pneumoniae infection in an adolescent: clinical and neuroradiologic follow up. Mov Disord 2000 Sep;15(5):1023-6

[12] Franco E, Casado JL, Robledo A, Lopez-Dominguez JM, Blanco A, Diaz-Espejo C.   Bilateral striatal necrosis following a wasp sting . Rev Neurol 2000 Nov 16-30;31(10):997-8

[13] Hrynchak M, Ang LC, Munoz DG. Bilateral striatal necrosis in hemolytic-uremic syndrome. Clin Neuropathol 1992 Jan-Feb;11(1):45-8

[14] Boylan KB, Chin JH, DeArmond SJ.Progressive dystonia following resuscitation from cardiac arrest. Neurology 1990 Sep;40(9):1458-61

[15] Blomqvist P, Wieloch T.Ischemic brain damage in rats following cardiac arrest using a long-term recovery model. J Cereb Blood Flow Metab 1985 Sep;5(3):420-31

[16] Leveque C, Minvielle F, Sarrazin JL, Soulie D, Le Friant G, Cordoliani YS. What is your diagnosis? Striatal necrosis following an episode of Sydenham"s chorea. J Neuroradiol 1997 Aug;24(2):97, 174

[17] Holtzman, D.; Hedley-Whyte, E. T. :  CPC (infantile striatonigral degeneration, with cerebellar degeneration, familial). New Eng. J. Med. 327: 261-268, 1992.

[18] Leuzzi V, Bertini E, De Negri AM, Gallucci M, Garavaglia B. Bilateral striatal necrosis, dystonia and optic atrophy in two siblings. J Neurol Neurosurg Psychiatry 1992 Jan;55(1):16-9

[19] Campos Y, Martin MA, Rubio JC, Gutierrez del Olmo MC, Cabello A, Arenas J. Bilateral striatal necrosis and MELAS associated with a new T3308C mutation in the mitochondrial ND1 gene. Biochem Biophys Res Commun 1997 Sep 18;238(2):323-5

[20] De Meirleir L, Seneca S, Lissens W, Schoentjes E, Desprechins B.Bilateral striatal necrosis with a novel point mutation in the mitochondrial ATPase 6 gene. Pediatr Neurol 1995 Oct;13(3):242-6

[21] Thyagarajan D, Shanske S, Vazquez-Memije M, De Vivo D, DiMauro S. A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis. Ann Neurol 1995 Sep;38(3):468-72

[22] Martin LJ, Brambrink AM, Price AC, Kaiser A, Agnew DM, Ichord RN, Traystman RJ. Neuronal death in newborn striatum after hypoxia-ischemia is necrosis and evolves with oxidative stress. Neurobiol Dis 2000 Jun;7(3):169-91

[23] Golden WC, Brambrink AM, Traystman RJ, Martin LJ. Failure to sustain recovery of Na,K-ATPase function is a possible mechanism for striatal neurodegeneration in hypoxic-ischemic newborn piglets. Brain Res Mol Brain Res 2001 Mar 31;88(1-2):94-102

[24] Meng SZ, Ohyu J, Itoh M, Takashima S.Dopamine transporter and nitric oxide synthase in hypoxic-ischemic brain. Pediatr Neurol 2000 Feb;22(2):115-21

[25] Paterson, D.; Carmichael, E. A. : Form of familial cerebral degeneration chiefly affecting the lenticular nucleus. Brain 47: 207-231, 1924.

[26] Rosemberg S, Amaral LC, Kliemann SE, Arita FN.Acute encephalopathy with bilateral striatal necrosis. A distinctive clinicopathological condition. Neuropediatrics 1992 Dec;23(6):310-5

[27] Mizuguchi M, Usuda I, Yoneyama A, Kamoshita S.Infantile bilateral striatal necrosis: chronic and acute manifestations in a single case. Brain Dev 1994 Jan-Feb;16(1):61-4

[28] Roig M, Calopa M, Rovira A, Macaya A, Riudor E, Losada M.Bilateral striatal lesions in childhood. Pediatr Neurol 1993 Sep-Oct;9(5):349-58

[29] Shian WJ, Chi CS, Mak SC.Infantile bilateral necrosis of the striatum of corpus: report of four cases . Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi 1992 May-Jun;33(3):231-8

[30] Capa Garcia L, Herranz Fernandez JL, Arteaga Manjon-Cabeza R, Abadi A. Bilateral infantile striatal necrosis . An Esp Pediatr 1991 Jul;35(1):55-8

[31] Sasaki M, Matsuda H, Omura I, Sugai K, Hashimoto T.Transient seizure disappearance due to bilateral striatal necrosis in a patient with intractable epilepsy. Brain Dev 2000 Jan;22(1):50-5

[32] van der Ent CK, de Vroede MA, Augustijn PB, Wit JM.A special case of congenital adrenal hypoplasia and acute bilateral infantile striatal necrosis. Acta Paediatr 1995 Aug;84(8):957-60

[33] Yamamoto K, Chiba HO, Ishitobi M, Nakagawa H, Ogawa T, Ishii K. Acute encephalopathy with bilateral striatal necrosis: favourable response to corticosteroid therapy. Europ J Paediatr Neurol 1997;1(1):41-5

[34] Matsui Y, Kumagae Y.Monoamine oxidase inhibitors prevent striatal neuronal necrosis induced by transient forebrain ischemia. Neurosci Lett 1991 May 27;126(2):175-8

Help Support :