Table 1.

Characteristics of CNS Disease in 16 Patients at Time of Their First Overt Clinical Neurological Manifestations

Age (mo)Primary (I) or Secondary Onset (II)Clinical SymptomsRadiological Symptoms
Coma MRI: White matter abnormalities 
  Seizures CT Scan: Calcifications 
  Brain stem symptoms Abnormal contrast enhancement 
1.5 Brain stem symptoms ND 
Coma CT Scan: Abnormal contrast enhancement 
   Subdural space dilatation 
Coma CT Scan: Severe necrotic areas 
  Seizures White matter abnormalities 
  Brain stem symptoms Subdural space dilatation 
Seizures CT Scan: Severe atrophy 
  Brain stem symptoms 
17 Coma CT Scan: Severe atrophy 
  Seizures 
19 Coma CT Scan: Necrotic areas 
  Seizures  
  Hemiplegia 
46 Ataxia CT Scan: White matter abnormalities 
  Somnolence  
  Meningism 
58 Ataxia CT Scan: White matter abnormalities 
II Coma 
  Tetraparesis 
  Brain stem symptoms ND 
  Developmental delay 
II Developmental delay CT Scan: Calcifications 
II Seizures CT Scan: Severe atrophy 
13 II Developmental delay MRI: White matter abnormalities 
   CT Scan: Calcifications 
   Abnormal contrast enhancement 
13 II Developmental delay MRI: White matter abnormalities 
  Coma CT Scan: Normal 
  Seizures 
  Tetraparesis 
14 II Coma MRI: White matter abnormalities 
57 II Ataxia MRI: Necrotic areas 
   White matter abnormalities 
Age (mo)Primary (I) or Secondary Onset (II)Clinical SymptomsRadiological Symptoms
Coma MRI: White matter abnormalities 
  Seizures CT Scan: Calcifications 
  Brain stem symptoms Abnormal contrast enhancement 
1.5 Brain stem symptoms ND 
Coma CT Scan: Abnormal contrast enhancement 
   Subdural space dilatation 
Coma CT Scan: Severe necrotic areas 
  Seizures White matter abnormalities 
  Brain stem symptoms Subdural space dilatation 
Seizures CT Scan: Severe atrophy 
  Brain stem symptoms 
17 Coma CT Scan: Severe atrophy 
  Seizures 
19 Coma CT Scan: Necrotic areas 
  Seizures  
  Hemiplegia 
46 Ataxia CT Scan: White matter abnormalities 
  Somnolence  
  Meningism 
58 Ataxia CT Scan: White matter abnormalities 
II Coma 
  Tetraparesis 
  Brain stem symptoms ND 
  Developmental delay 
II Developmental delay CT Scan: Calcifications 
II Seizures CT Scan: Severe atrophy 
13 II Developmental delay MRI: White matter abnormalities 
   CT Scan: Calcifications 
   Abnormal contrast enhancement 
13 II Developmental delay MRI: White matter abnormalities 
  Coma CT Scan: Normal 
  Seizures 
  Tetraparesis 
14 II Coma MRI: White matter abnormalities 
57 II Ataxia MRI: Necrotic areas 
   White matter abnormalities 

Abbreviations: MRI, magnetic resonance imaging; CT Scan, computed tomographic scans; ND, not determined.

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