Article Figures & Data
Figures
- Figure 1
- Pedigree of families of identified patients
- Figure 2
- CT brain showing A) Case 7: right hemispheric loss of gray-white matter interface and effacement of sulci affecting right anterior and middle cerebral artery territory, midline shift and left hemispheric cortical atrophy. B) Case 6: FLAIR MRI depicting multiple high signal intensity lesions in centrum semiovale and periventricular white matter. C) Case 4: FLAIR MRI revealed bilateral asymmetrical high signal intensities of the thalami and splenium of corpus callosum. D) Case 1: T2 MRI midline sagittal showing high signal intensity extending from medulla oblongata up to C5 of cervical cord.
- Figure 3
- Flow cytometric analysis of case 8 shows a PNH clone within RBC (89.7%), the findings suggestive of paroxysmal nocturnal hematuria. RBC - Red blood cells, PNH - Paroxysmal Nocturnal Hemoglobinuria, FITC - Fluorescein isothiocyanate
Tables
Patient number Age at presentation Gender Guillain Barre syndrome/CIDP Central nervous system lesions Hemolysis 1 Eleven months Male Axonal motor neuropathy Multiple strokes Acute transverse myelitis Hemolysis with hematuria occasional blood transfusion 2 Five months Female Demyelinating with secondary axonal sensory-motor neuropathy Normal No evidence of active hemolysis 3 Six months Female Demyelination neuropathy Acute necrotizing encephalitis Hemolysis with hematuria occasional blood transfusion 4 Eleven months Female Sensory-motor axonal neuropathy Acute necrotizing encephalitis Microscopic hematuria 5 Ten moths Female Sensory-motor axonal neuropathy Normal No evidence of active hemolysis 6 Two months Female Axonal motor neuropathy Acute disseminated encephalomyelitis Hemolysis with hematuria occasional blood transfusion 7 Four months Female Axonal motor neuropathy Arterial ischemic Stroke Hemolysis with hematuria occasional blood transfusion 8 Eleven months Male Axonal motor neuropathy Normal Hemolysis with hematuria occasional blood transfusion Patient number Hemoglobin CSF protein, control <400 mg/L CT/ MRI brain & spine Treatment Outcome 1 6-12 g/dl 1326 mg/L Encephalomalacia, cortical and cerebellar atrophy, cervical cord lesions from brainstem up to C6 IVIg, corticosteroids, plasma exchange Died at the age of 14 2 11 g/dl - Normal IVIg Walking with canes with high steppage gait 3 7-11 g/dl - Bilateral thalami, basal ganglia, occipital lobes IVIg, corticosteroids Died at the age of 10 4 11-13 g/dl 685 mg/L Bilateral thalami, internal capsule, corpus callosum lesions IVIg, corticosteroids Standing but not walking, aphasia, intellectual impairment 5 8-11 g/dl - Normal IVIg Flaccid paralysis of lower limbs, just sitting 6 7-11 g/dl 872 mg/L Extensive demyelination supra and infratentorial structures including brainstem IVIg, corticosteroids, eculizumab Persistent vegetative state 7 7.5-10 g/dl - Right middles artery stroke, left hemispheric atrophy IVIg Persistent vegetative state on ventilator support 8 7.5-9.9 g/dl 914 mg/L Normal IVIg, plasma exchange Flaccid lower limbs
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