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Foot-drop: An unusual complaint in Henoch-Schönlein purpura

Authors:
Manouchehr Amini at Tehran University of Medical Sciences
Manouchehr Amini
Iraj Najafi at Tehran University of Medical Sciences
Iraj Najafi
Reza Ganji at University of Tehran
Reza Ganji
Monir Hakemi at Shariati Hospital
Monir Hakemi
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LETTER TO THE EDITORS
Foot-drop: an unusual complaint in Henoch–Schönlein
purpura
Manouchehr Amini &Iraj Najafi &
Mohammad R. Ganji &Monir S. Hakemi &
Mohsen Nouri
Received: 29 May 2008 / Revised: 27 June 2008 / Accepted: 3 July 2008
#IPNA 2008
Sirs,
Involvement of the peripheral nervous system in Henoch–
Schönlein purpura (HSP) is rare [1]. Here, we introduce the
reader to an uncommon neural manifestation of HSP in the
lower limb.
A 15-year-old boy presented at the emergency room of a
hospital in Divan dare, a small city in the west of Iran, with
abdominal pain, fever, bloody diarrhea and melena,
arthralgia (both knees and elbows), gross hematuria, and
palpable purpura on the buttock and behind the thigh. He
was diagnosed with HSP and was treated with prednisolone
30 mg/day, which was reduced to 10 mg/day upon his
discharge. He was admitted for 6 days, and, when he was
discharged, the skin lesions had improved. Two weeks later,
the patient developed a right-side incomplete foot-drop,
which brought him to our center, Shariati Hospital. On his
admission, hematuria and facial edema were observed.
Physical examinations were not revealing, except for impaired
dorsal flexion of the right ankle. Para-clinical findings were
consistent with HSP: proteinuria (3,900 mg/24 h), and normal
levels of C3, C4, and CH50. Results of tests for viral markers
[hepatitis B virus (HBV), hepatitis C virus (HCV), and human
immunodeficiency virus (HIV)], antinuclear antibodies
(ANAs), cytoplasmic anti-neutrophilic cytoplasmic antibod-
ies (C-ANCAs,) perinuclear anti-neutrophilic cytoplasmic
antibodies (P-ANCAs) and cryoglobulin were negative.
Serum level of immunoglobulin A (IgA) was 218 mg/dl
(normal range 70–400 mg/dl). Ultrasonic evaluations of the
urinary tract showed normal findings, except for increased
cortical echogenicity of both kidneys. Histopathologic assess-
ment of the renal biopsy showed diffuse mesangio-proliferative
lesion with focal endocapillary proliferation and cellular
crescents in 15% of the glomeruli. Immuno-fluorescence
studies demonstrated IgA (2+) and C3 (1+) depositions in the
mesangium. Electromyography and nerve conduction velocity
(EMG/NCV) suggested mononeuropathy of the right deep
proneal nerve, but, because the patient was unwilling, neural
biopsy was avoided. Though not proven pathologically, the
cause of the dropped foot was assigned to the underlying HSP,
and an aggressive therapeutic strategy was adopted: intrave-
nous administration of methylprednisolone (500 mg; three
doses) and cyclophosphamide (750 mg), followed by oral
treatment with prednisolone 15 mg/day, which was increased to
40 mg/day (1 mg/kg) upon his discharge. Impaired dorsi-
flexion started to improve after 1 week from the beginning of
the treatment. Although the patient received cyclophosphamide
pulses monthly for three times, the hematuria relapsed when it
was discontinued. With prescription of azathiopurine
(100 mg/day) as an alternative, renal activity of HSP subsided
again.
Now, the patient has trace hematuria without proteinuria,
and the foot drop has improved completely.
The clinical feature of our patient was completely
compatible with HSP. Although other vasculitis, especially
microscopic polyarteritis nodosa (PAN), was demonstrated
as differential diagnosis, the result of the renal biopsy,
which showed mesangial deposition of IgA, confirmed the
diagnosis [1]. Headaches and changes in mental status are
the most frequent neurologic complications of HSP,
followed by seizures, focal neurologic deficits, mononeuro-
pathies, and polyradiculoneuropathies [2]. By reviewing the
literature we found three reports of an association of HSP
with manifestations of the peripheral nervous system
(PNS), including involvement of the posterior tibial nerve
Pediatr Nephrol
DOI 10.1007/s00467-008-0952-5
M. Amini (*):I. Najafi :M. R. Ganji :M. S. Hakemi :M. Nouri
Division of Nephrology, Shariati Hospital,
Tehran University of Medical Sciences,
Kargar Avenue,
Tehran, Iran
e-mail: aminimd@tums.ac.ir
... In two of the aforementioned 37 cases [12,41], a concurrent involvement of the peripheral nervous system was noted. In 15 further cases of Henoch-Scho¨nlein syndrome, which were reported between 1970 and 2009, a cranial or peripheral neuropathy without severe hypertension and without any CNS dysfunction was noted [9,[43][44][45][46][47][48][49][50][51][52][53][54][55][56]. ...
... A biopsy showing predominant IgA deposition was performed in 31 (57%) of the 54 patients (7, 9, 10, 12, 14-16, 19, 20, 22, 23, 25, 26, 28, 29, 31, 32, 35, 37-39, 43-47, 50, 51, 53, 61 and 62): skin biopsy (n ¼ 15), renal biopsy (n ¼ 11), both skin and renal biopsy (n ¼ 5). Testing for IgG ANCA was negative in 14 patients with this examination (6,8,12,19,20,25,29,32,35,43,45,51,53,54). ...
Nervous system dysfunction in Henoch-Schönlein syndrome: Systematic review of the literature
Article
Full-text available
  • Sep 2009
CNS or peripheral nervous system dysfunction sometimes occurs in Henoch-Schönlein patients. We review all Henoch-Schönlein cases published after 1969 with CNS dysfunction without severe hypertension and neuroimaging studies (n = 35), cranial or peripheral neuropathy (n = 15), both CNS and peripheral nervous system dysfunction without severe hypertension (n = 2) or nervous system dysfunction with severe hypertension (n = 2). Forty-four of the 54 patients were <20 years of age. In patients with CNS dysfunction without or with severe hypertension the following presentations were observed in decreasing order of frequency: altered level of consciousness, convulsions, focal neurological deficits, visual abnormalities and verbal disability. Imaging studies disclosed the following lesions: vascular lesions almost always involving two or more vessels, intracerebral haemorrhage, posterior subcortical oedema, diffuse brain oedema and thrombosis of the superior sagittal sinus. Following lesions were noted in the subjects with cranial or peripheral neuropathy without severe hypertension: peroneal neuropathy, peripheral facial palsy, Guillain-Barré syndrome, brachial plexopathy, posterior tibial nerve neuropathy, femoral neuropathy, ulnar neuropathy and mononeuritis multiplex. Persisting signs of either CNS (n = 9) or peripheral (n = 1) nervous system dysfunction were sometimes reported. In Henoch-Schönlein syndrome, signs of nervous system dysfunction are uncommon but clinically relevant. This review helps clinicians managing Henoch-Schönlein syndrome with nervous system dysfunction.
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... [9][10][11] Systemic diseases such as diabetes, vasculitis, connective tissue, and autoimmune disease may also be the etiological cause. [12][13][14] In these circumstances, foot drop is almost always unilateral. ...
Acute bilateral isolated foot drop: Report of two cases
Article
Full-text available
  • Apr 2015
Foot drop is defined as the weakness of the foot and ankle dorsiflexion. Acute unilateral foot drop is a well-documented entity, whereas bilateral foot drop is rarely documented. Slowly progressing bilateral foot drop may occur with various metabolic causes, parasagittal intracranial pathologies, and cauda equina syndrome. Acute onset of bilateral foot drop due to disc herniation is extremely rare. Here we present two cases of acute bilateral foot drop due to disc herniation. The first patient was a 45-year-old man presented with acute bilateral foot drop, without any sign of the cauda equina syndrome. Lumbar magnetic resonance imaging of the patient revealed L4-5 disc herniation. To our knowledge, this is the first presented case of acute bilateral foot drop without any signs of cauda equina syndrome caused by L4-5 disc herniation. The second patient was a 50-year-old man who was also presented with acute bilateral foot drop, and had T12-L1 disc herniation with intradural extension. Also this is the first presented case of T12-L1 disc herniation with intradural extension causing acute bilateral foot drop. We performed emergent decompressive laminectomy to both of the patients and extrude disc materials were excised. Both of the patients were recovered with favorable outcome.
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Bilateral Footdrop After Treatment of an Anterior Communicating Artery Aneurysm: A Case Report and Review of the Literature
Article
  • Mar 2011
  • NEUROSURGERY
Footdrop designates weakness of the ankle as well as toes dorsiflexion. Peripheral causes of unilateral footdrop are well established. Bilateral footdrop originating from pathologies in the central nervous system are rare and include a number of unexplored etiologies. A case of bilateral footdrop is presented. The patient presented with a grade IV subarachnoid hemorrhage with intraventricular extension. He was treated with coil embolization of an anterior communicating artery aneurysm. Postoperatively, he was found to have weakness of both ankle and toe dorsiflexion. Findings on magnetic resonance imaging of the cervical, thoracic, and lumbar spine were negative for abnormal cord signal, cord infarction, and compressive lesion. Magnetic resonance imaging of the brain revealed parasagittal bifrontal and right greater than left convexity foci of acute infarction. Central causes of acute footdrop are rare. However, they should be considered in the differential diagnosis, particularly in the presence of upper motor neuron signs on physical examination.
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Association of Guillain-Barr?? Syndrome and Henoch-Sch??nlein Purpura: Is Immunoglobulin A Responsible for the Neurologic Syndrome?
Article
  • Sep 1988
  • AM J MED SCI
Guillain-Barre syndrome and Henoch-Schonlein purpura (HSP) appeared simultaneously in a 35-year-old woman. During the course of the Guillain-Barre syndrome, the presence of IgA aggregates and a decrease in complement components were noted in the cerebrospinal fluid. These abnormalities disappeared when the neurologic syndrome remitted. This suggests the responsibility of IgA immune complexes, characteristic of HSP, in the occurrence of the associated Guillain-Barre syndrome.
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Neurologic Manifestations of Schoenlein-Henoch Purpura: Report of Three Cases and Review of the Literature
Article
  • May 1985
Three patients developed prominent neurologic symptoms and signs associated with Schoenlein-Henoch purpura. A 7 1/2-year-old boy was seen with status epilepticus after a 2-week history of generalized headaches, irritability, and intermittent colicky abdominal pain. A left hemiparesis and a left homonymous hemianopia with a right gaze preference that were present on initial examinations gradually resolved, but a mild left arm paresis persisted. Cutaneous, renal, and joint involvement followed initial CNS manifestations. The second patient, a 7-year-old girl, had a complex partial seizure with secondary generalization and a postictal hemiparesis seven days after presentation with classic signs of Schoenlein-Henoch purpura. Behavioral changes were noted during the acute phase of the illness. The third patient, a 13-year-old boy, developed signs of a left brachial plexopathy and transient weakness of his right leg during a complicated course of Schoenlein-Henoch purpura. Review of the world literature indicates that headaches and mental status changes are the most frequent neurologic complications of Schoenlein-Henoch purpura, followed by seizures, focal neurologic deficits, mononeuropathies, and polyradiculoneuropathies. The vasculitis of Schoenlein-Henoch purpura can involve the nervous system and may add significantly to the morbidity of the illness.
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Association of Guillain-Barré syndrome and Henoch-Schönlein purpura: is immunoglobulin a responsible for the neurologic syndrome?
Article
  • Oct 1988
  • AM J MED SCI
Guillain-Barré syndrome and Henoch-Schönlein purpura (HSP) appeared simultaneously in a 35-year-old woman. During the course of the Guillain-Barré syndrome, the presence of IgA aggregates and a decrease in complement components were noted in the cerebrospinal fluid. These abnormalities disappeared when the neurologic syndrome remitted. This suggests the responsibility of IgA immune complexes, characteristic of HSP, in the occurrence of the associated Guillain-Barré syndrome.
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Ataxia and peripheral neuropathy: Rare manifestations in Henoch-Schönlein purpura
Article
  • Jan 2002
Henoch-Schönlein purpura (HSP) is a multisystemic vasculitis. Nervous system involvement is usually underestimated. Headaches, mental status changes and seizures are the most frequent neurologic symptoms. Ataxia and mononeuropathy are both very rare. We present an 11-year-old boy with HSP who suffered from ataxia during the initial presentation and peripheral neuropathy at the time of a relapse. Brainstem vasculitic involvement was shown by magnetic resonance imaging, while cranial tomography was normal. All the neurologic symptoms and signs resolved following bolus methylprednisolone administration. Ten months later he had a second course of HSP with skin and renal involvement. A percutaneous renal biopsy, which was performed due to persistent hematuria, revealed mesangial proliferation with IgA deposition. During that period the patient experienced pain and numbness in the right foot and leg; electromyography showed signs of mononeuritis multiplex involving the right posterior tibial nerve. The patient responded to steroid therapy.
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