1
|
[12]
|
1.25
|
F
|
PAH
|
x
| | | | |
Complete recovery
|
2
|
[13]
|
1.5
|
M
|
PAH, HUS
|
Untreated
|
Deceased without diagnosis
|
3
|
[7]
|
1.5
|
F
|
No information available
|
No information available
|
No information available
|
4
|
[7]
|
1.5
|
F
|
No information available
|
No information available
|
No information available
|
5
|
[7]
|
2
|
M
|
Lethargy, convulsions, hypotonia
|
No information available
|
No information available
|
6
|
[7]
|
2
|
M
|
No information available
|
No information available
|
No information available
|
7
|
[10]
|
2,5
|
M
|
Asymptomatic
|
Untreated
|
No information available
|
8
|
[13]
|
2,5
|
M
|
PAH, HUS
|
x
| | | | |
Deceased
|
9
|
[14]
|
3
|
M
|
PAH, HUS
|
Untreated
|
Deceased without diagnosis
|
10
|
[13]
|
3
|
M
|
PAH, HUS
|
Untreated
|
Deceased without diagnosis
|
11
|
[7]
|
3.5
|
M
|
Cognitive decline, lethargy, convulsions
|
No information available
|
No information available
|
12
|
[13]
|
4
|
F
|
PAH, HUS
|
x
| | | | |
Progressive PAH
|
13
|
[15]
|
4
|
F
|
HUS
|
x
|
x
|
x
| | |
Complete recovery
|
14
|
[16]
|
4
|
F
|
Cognitive decline, neuropathy, ataxia
|
x
|
x
|
x
| | |
Improvement, mild cognitive impairment, neurological sequelae
|
15
|
[17]
|
6
|
F
|
HUS
|
x
|
x
|
x
| | |
Chronic renal failure
|
16
|
[18]
|
7
|
F
|
Neuropathy, myelopathy, cognitive impairment, epilepsy
|
x
| |
x
| | |
Cognition improved, seizures resolved, neurological sequelae
|
17
|
[17]
|
8
|
F
|
HUS
|
x
|
x
|
x
| | |
Renal parameters improved
|
18
|
[19]
|
10
|
F
|
Acute cognitive decline, anorexia, catatonia, psychosis, seizures. Brain volume loss, thinned corpus callosum
|
x
|
x
| | | |
Seizures and psychiatric symptoms improved
|
19
|
[20]
|
11
|
F
|
Cognitive decline, behavioral changes, ataxia, myoclonic jerks
|
x
|
x
|
x
|
x
| |
Complete recovery
|
20
|
[15]
|
11
|
M
|
HUS, hypertensive encephalopathy, coma, convulsions
|
x
|
x
|
x
| | |
Complete recovery (but antihypertensive drugs necessary)
|
21
|
[9]
|
12
|
F
|
Ataxia, neuropathy, myelopathy, mild neuropsychiatric symptoms.
|
x
| | | | |
Improved; neurological sequelae
|
22
|
[21]
|
13
|
F
|
Cognitive decline, ataxia/dysarthria, EEG abnormal
|
x
| | | | |
Cognition improved, neurological sequelae
|
23
|
[13]
|
14
|
F
|
PAH, HUS
|
x
| | | | |
"Stable" disease
|
24
|
[19]
|
14
|
F
|
Cognitive decline, depression, ataxia, seizures, neuropathy/myelopathy
|
x
|
x
| |
x
| |
Improved; neurological sequelae
|
25
|
[10]
|
14
|
M
|
Acute psychosis, mental retardation
|
Untreated
|
No information available
|
26
|
[22]
|
16
|
F
|
Thromboembolism, neuropathy, myelopathy, psychiatric symptoms
|
x
|
x
|
x
|
x
| |
Disease progression
|
27
|
[23]
|
16
|
M
|
Atypical glomerulopathy
|
x
|
x
| | |
x
|
Coma, deceased
|
28
|
[24]
|
18
|
F
|
Glomerulonephritis; psychiatric symptoms, cognitive impairment, recurrent thromboses, pulmonary embolism, seizures, neuropathy, myelopathy, cortical atrophy, leukoencephalopathy, corpus callosum agenesis
|
x
|
x
|
x
| | |
Deceased after initial improvement
|
29
|
[25]
|
20
|
M
|
HUS, renal failure, malignant hypertension
|
x
|
x
|
x
| | |
Improved, renal function stable
|
30
|
[26]
|
20
|
M
|
Neuropathy, myelopathy, progressive encephalopathy, confusion, deep venous thrombosis, progressive respiratory failure
|
x
| | |
x
| |
Improved, neurological sequelae
|
31
|
[18]
|
22
|
F
|
Triggered by pregnancy/caesarian section: Sluggish response, neuropathy
|
x
| |
x
| | |
Complete recovery
|
32
|
[27]
|
23
|
M
|
Cognitive impairment, ataxia, neuropathy, spinal cord myelin lesions
|
x
|
x
|
x
|
x
| |
Improved, neurological sequelae
|
33
|
[22]
|
24
|
F
|
Myelopathy
|
x
|
x
|
x
|
x
| |
Moderate myelopathy
|
34
|
[9]
|
29
|
F
|
Asymptomatic
|
Untreated
|
Asymptomatic
|
35
|
[11]
|
29
|
F
|
Asymptomatic
|
x
| | | | |
Biochemical response
|
36
|
[28]
|
32
|
F
|
Progressive neuropathy, myelopathy, optic disk pallor, leukopenia
|
Untreated
|
Deceased without diagnosis
|
37
|
[24]
|
33
|
F
|
Glomerulonephritis, recurrent deep venous thrombosis.
|
x
|
x
|
x
| | |
No more thromboses
|
38
|
[29]
|
36
|
F
|
Neuropathy, psychiatric symptoms
|
x
|
x
|
x
| | |
Mental status improved, neurological sequelae
|
39
|
[30]
|
38
|
M
|
Hypertension, seizures, progressive confusion, progressive periventricular white matter lesions
|
x
|
x
|
x
| | |
Complete recovery
|
40
|
[18]
|
40
|
M
|
Cognitive decline, hallucinations, neuropathy, myelopathy, brain atrophy.
|
x
| |
x
| | |
Complete recovery
|
41
|
[24]
|
41
|
M
|
Depression, neuropathy, myelopathy, periventricular leucoencephalopathy, abnormal signal in spinal cord myelin
|
x
|
x
|
x
| | |
Overall improvement. Spinal cord myelin lesion disappeared
|
42
|
[28]
|
44
|
F
|
Cognitive decline, optic disk pallor, venous thrombosis, pulmonary embolism
|
Untreated
|
Deceased without diagnosis
|
43-48
|
[3]
|
4-14
|
n.a.
|
No individual information reported
|
No information available
|
N = 5 "very positive"; n = 1 "moderately impaired"
|
49-58
|
[4]
|
1-13.5
|
5 M
|
No individual information reported
|
No information available
|
Overall reduction of symptoms
|
5 F
|