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Bilateral striatal necrosis in acute encephalopathy has been reported in a small number of adults with methanol or cyanide intoxication, hypoxic encephalopathy or haemolytic-uraemic syndrome. Acute encephalopathy with bilateral striatal necrosis has been reported in infants and children. However, the pathogenesis of the necrosis remains unclear. This is the first report of serial imaging from the very early to chronic stage in two acute encephalopathic adults with bilateral striatal necrosis. A clinicoradiological study is presented for clarification of the pathological process and pathogenesis. Striatal lesions were not detected in the very early stages, but only thereafter. Serial studies suggested that the lesions were caused by delayed neuronal death. These patients had severe lactic acidosis, near the limit for survival. There have been few reports of adults with acute encephalopathy and bilateral striatal necrosis in whom arterial pH was described; all these exhibited marked acidosis. The common pathophysiological condition among these encephalopathies with bilateral striatal necrosis could be lactic acidosis elicited by impairment of ATP generation through the Krebs cycle. The striatum might represent one of the target areas of Krebs-cycle blockade.
Bilateral striatal necrosis in acute encephalopathy has been reported in a small number of adults with methanol or cyanide intoxication, hypoxic encephalopathy or haemolytic-uraemic syndrome. Acute encephalopathy with bilateral striatal necrosis has been reported in infants and children. However, the pathogenesis of the necrosis remains unclear. This is the first report of serial imaging from the very early to chronic stage in two acute encephalopathic adults with bilateral striatal necrosis. A clinicoradiological study is presented for clarification of the pathological process and pathogenesis. Striatal lesions were not detected in the very early stages, but only thereafter. Serial studies suggested that the lesions were caused by delayed neuronal death. These patients had severe lactic acidosis, near the limit for survival. There have been few reports of adults with acute encephalopathy and bilateral striatal necrosis in whom arterial pH was described; all these exhibited marked acidosis. The common pathophysiological condition among these encephalopathies with bilateral striatal necrosis could be lactic acidosis elicited by impairment of ATP generation through the Krebs cycle. The striatum might represent one of the target areas of Krebs-cycle blockade.
Biotin-responsive basal ganglia disease (BBGD) is a recessive disorder with childhood onset that presents as a subacute encephalopathy, with confusion, dysarthria, and dysphagia, and that progresses to severe cogwheel rigidity, dystonia, quadriparesis, and eventual death, if left untreated. BBGD symptoms disappear within a few days with the administration of high doses of biotin (5-10 mg/kg/d). On brain magnetic resonance imaging examination, patients display central bilateral necrosis in the head of the caudate, with complete or partial involvement of the putamen. All patients diagnosed to date are of Saudi, Syrian, or Yemeni ancestry, and all have consanguineous parents. Using linkage analysis in four families, we mapped the genetic defect near marker D2S2158 in 2q36.3 (LOD=5.9; theta=0.0) to a minimum candidate region (approximately 2 Mb) between D2S2354 and D2S1256, on the basis of complete homozygosity. In this segment, each family displayed one of two different missense mutations that altered the coding sequence of SLC19A3, the gene for a transporter related to the reduced-folate (encoded by SLC19A1) and thiamin (encoded by SLC19A2) transporters.
A 4-year-old girl with bilateral striatal oedema in association with an echovirus type 21 infection is reported. In the course of a prolonged upper respiratory-tract infection, the patient developed muscular hypotonia, resting tremor, ataxia, sleepiness, hyperaesthesia, and indistinct speech. T,-weighted cranial MRI revealed bilateral oedema of the basal ganglia and the cerebellar peduncles. At follow-up after 3 months MRI changes and clinical symptoms had fully resolved.Bilatenl striatal lesions arc found in numerous diseases of childhood including trauma, hypoxemia-ischaemia, intoxication. and metabolic disorders. Some cases with sudden onset may be traced back to an infectious or paninfectious genesis such as tubcrculosis, mumps, and Mycoplastva pneuniombe infection (Hosenhcrg c't al. 1'992. Roig et al. 1993).We present the case o f a 4-year-old girl who developed bilateral striatal oedema in association with echovirus type 2 1 infection. 'lo our knowledge this is the tint description o f complete clinical and MRI-scan normalization. Case reportThc previously healthy 4-year-old girl presented with an acute upper respiratory-tnct infection. She had conjunctivitis, a cough, and continuous fever u p t o 39°C. On the third day she developed meningism. Examination of CSF in a local hospital showed mild pleocytosis (20 leucocytcs/mm5, mainly lymphocytes) with n o n a l protein and glucose conccntrarions. On day 9 the child was transferred t o o u r hospital with suspected viral meningitis.On admission she presented with general muscle hypotonia. an ataxic gait, a mild resting tremor, indistinct speech, and hyperacsthesia particularly of the arms. The girl was sleepy, but conscious, and tendon reflexes. motor nerve conduction velocity (tibial, peroneal, and ulnar nerve), SSEP (evoked potentials from mcdian and tibial nerve), and ophthalmologic examinations were normal; the EEG demonstrated non-specific slow waves. The CSF examination on day 15 again showed mild pletwosis (47 leucoq.tes/mni', 50% lymphocytes), an elevated protein content of 80.2 mgdL and a normal glucose concentration. Repcated electron-and light-microscopic cxarninations of the CSF well as viral and bacterial cultures were negative. Various blood cultures and repeated serologic examinations including those for influenza, mumps, herpes simplex, varicella zoster, and adeno-, echo-, polio-. and coxsackievirus, as well as Mj~coplrrsma ptieittnotiiae and Borrelia burgdorferi. were negative. The fever of u p to 39OC continued intermittently.A5 an intracranial process was suspected, a cranial CT was carried out o n day 24 and an MRI o n day 36. While the CTand l)euelopnirntnlhfedicitie& CbildNritmlugy 199H. 40. 4 2 1 4 2 3 421
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