AJR 2000; 175:922-923
© American Roentgen Ray Society
MR Imaging of Encephalopathy in Adult Henoch-Shönlein Purpura
Carlos Perez,
Elias Maravi,
Jorge Olier and
Rosa Guarch
Hospital Virgen del Camino 31008 Pamplona, Spain
Hospital Provincial de Navarra Pamplona, Spain
Henoch-Schönlein purpura (HSP) is a systemic
necrotizing vasculitis of unknown cause that affects small vessels and that is
characterized by palpable purpura, arthralgia, abdominal pain with bleeding,
and renal disease. Although well described in adults, HSP is usually seen in
children [1]. Involvement of
the central nervous system is rare. The most common manifestation is headache
followed by subtle encephalopathy with minimal changes in mental status, such
as labile mood, apathy, and hyperactivity
[2]. We report the MR imaging
findings of an adult with biopsy-proven HSP in whom mild encephalopathy
developed during the acute phase of the disease.
A 42-year-old man was admitted to the hospital in July 1998 with a 2-day
history of fever, malaise, headache, irritability, arthralgia, abdominal pain,
and a nonpruritic purpuric rash. Over the preceding 6 years he had experienced
all these features during nine similar episodes that persisted for 7-10 days
and then recurred every 6-9 months, with complete recovery between episodes
and no residual damage. A skin biopsy specimen was obtained and showed no
signs of vasculitis. During each episode, he was treated with oral
corticosteroids. At admission, his temperature was 36.8°C and his blood
pressure was 110 over 70 mm Hg. He had a purpuric rash on the buttocks and
lower extremities (Fig. 5A),
abdominal pain, and bloody stools. A symmetric synovitis of the knees and
ankles was present. The neurologic examination revealed normal findings.
Laboratory tests showed an increased serum IgA concentration. No circulating
immune complexes, cryoglobulins, antinuclear antibodies, rheumatic factor, or
antineutrophil cytoplasmic antibodies were detected. Bacterial serology
revealed negative findings. Colonic endoscopy showed petechiae and erosions of
the mucosa. A skin biopsy specimen showed leukocytoclastic vasculitis with
neutrophils within the vessel walls, necrosis of the vessel walls, edema of
the dermis, lekocytoclasia, and deposits of fibrin within and around the small
vessels (Fig. 5B). Immunofluorescence studies showed IgA and C3 along the small vessels of the
skin. A CT scan of the brain showed moderate atrophy. MR imaging of the head
showed moderate global atrophy and multiple small foci of increased T2 signal
intensity in the periventricular and deep white matter
(Fig. 5C). Cerebral angiography
was not performed because the small vessels involved in HSP are beyond its
limit of resolution. Because of the relapsing course of the HSP in this
patient and the patient's lack of response to corticosteroids, the patient was
treated with IV immunoglobulin (total dose, 2 g/kg). Rapid resolution of his
symptoms occurred, and the patient remains without relapse 18 months after
therapy.
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Fig. 5C. —42-year-old man with Henoch-Schönlein
purpura. Axial T2-weighted MR image shows multiple small foci of
hyperintensity in periventricular and deep white matter (arrowheads).
Note mild cortical atrophy.
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At presentation, this patient had palpable purpura, arthritis, diffuse
abdominal pain with bloody stools, elevated serum IgA level, and
leukocytoclastic vasculitis with IgA and C3 deposits, involving small vessels
of the dermis; his disease thereby met the criteria for classification as HSP
[3]. In addition, he had brain
atrophy and mild encephalopathy characterized by headache and irritability
with multiple subcortical small high-signal-intensity foci on T2-weighted MR
imaging. Although no histologic proof is available, the central nervous system
findings may represent multifocal leukocytoclastic vasculitis involvement.
This hypothesis is supported by the appearance of leukocytoclastic vasculitis
involvement of the small vessels of the skin in close temporal relationship
with encephalopathy. Encephalopathy caused by uremia or hypertension is
unlikely because blood pressure and renal function were normal.
Neurologic features of HSP are rare and have been recognized mainly in
children. Seizure, subdural hematoma, subarchnoid hemorrhage, intraparenchymal
bleeding, and infarction have been documented in children with HSP
[2]. Only one adult with HSP
and seizures, with normal findings on MR imaging of the head, has been
reported [4]. To our knowledge,
MR imaging findings of encephalopathy caused by HSP have not been reported
previously in the English literature.
References
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Belman AL, Leicher CR, Moshé SL,
Mezey AP. Neurologic manifestations of
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1985;75:687
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Mills JA, Michel BA, Bloch DA, et al. The American College of
Rheumatology 1990 criteria for the classification of
Henoch-Schönlein purpura. Arthritis
Rheum 1990;33:1114
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Fielding RE, Hawkins CP, Hand MF, Heath PD, Davies SJ. Seizures
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