Cryoglobulinaemic neuropathy: a further cause of bilateral sciatic neuropathy
© Pérez et al. 2008
Received: 22 July 2008
Accepted: 04 October 2008
Published: 04 October 2008
Bilateral sciatic neuropathy is a rare condition and it has been described as a compression or entrapment neuropathy but it is an uncommon clinical manifestation due to necrotizing vasculitis. We report an unusual case of cryoglobulinaemic neuropathy in an elderly woman with no underlying infectious or neoplastic cause; acute bilateral sciatic mononeuropathy was the presenting clinical manifestation of the cryoglobulinaemia.
The term cryoglobulinaemia refers to the presence in the serum of immunoglobulins (Ig), which precipitate at temperatures below 37°C and redissolve on rewarming. Cryoglobulinemia is not considered a frequent cause of peripheral neuropathy, but it may be more common than previously thought in view of its relationship with the hepatitis C virus (HCV), a widespread infection . The incidence of the cryoglobulinaemic neuropathy (CN) has been described to vary from 6 to 60% [1, 2]. The most common form of CN is sensory neuropathy (76%) followed by sensorimotor polyneuropathy (15%) and mononeuritis multiplex (MM) (9%) . We describe an unusual case bilateral sciatic axonopathy in the context of cryoglobulinaemia.
Patient neurophysiological data.
> 4.6 ms
< 4 ms
< 4.5 ms
> 8 mV
> 4.5 mV
> 5 mV
> 50 m/s
> 43 m/s
> 43 m/s
> 43 m/s
> 36 μV
Unilateral sciatic nerve lesions are common. They are frequently the result of trauma and complications of hip replacement procedures. Conversely, bilateral sciatic neuropathy is a rare condition. It has been described as a compression or entrapment neuropathies, such as heterotopic ossification in a traumatic brain-injured patient , after surgery [5, 6], due to toilet seat entrapment , following sleeping in the lotus position , prolonged lying in the supine position with both legs hanging over the end of the bed  or being in a prolonged seated position in a floor-level cupboard . Combined distal tibial and peroneal neuropathies have been described following popliteal fossa compression related to legs hanging over a bed footboard . Although there was neither a sural nerve biopsy nor post mortem tissue to confirm, the vasculitic origin is the most plausible cause of the neuropathy. Bilateral sciatic neuropathy is an uncommon clinical manifestation resulting from necrotizing vasculitis. In one series of 94 cases of vasculitic neuropathy, the peroneal nerve or division was involved in 76%, the ulnar nerve in 28%, the tibial nerve in 11% and the median nerve in 9%, but sciatic nerve damage was not reported . In typical cases, the onset of the neuropathy is abrupt and the deficit severe, but in many cases only partial deficit in a nerve territory is observed .
The electrophysiological pattern in our case was typically axonal. MM may be the accurate diagnosis and not a length-dependent axonal polyneuropathy. This may be due to the abrupt clinical onset with no subsequent progression and the fact that the median nerve was neither clinically or electrophysiologic affected. It is possible that the initial form of the CN was MM which subsequently evolved into distal symmetrical sensorimotor neuropathy. Although an EMG examination of the biceps femoris was not performed, weakness in hamstring muscles supports the bilateral sciatic nerve damage diagnosis and not that of combined tibial and peroneal neuropathies.
Cryoglobulinaemia is usually classified into three subgroups: type I, single monoclonal Ig, usually a paraprotein, which is almost invariably associated with haematological disorders; types II and III, characterised by polyclonal IgG and monoclonal or polyclonal IgM RF, respectively. Circulating mixed cryoglobulins are often detected in many infectious and systemic disorders. Mixed cryoglobulinaemia (MC) shows a striking association with HCV infection (> 90%) . As a contrast, essential MC is a distinct condition characterised by leucocytoclastic vasculitis and frequent multiple organ involvement with no underlying disorder. The case we describe showed typical manifestation of Type II MC: presence of a monoclonal component and polyclonal Ig, RF activity, low C4, leucocytoclastic vasculitis in purpuric lesions and MM. It has been suggested that monoclonal cryoglobulins are more likely to be associated with demyelinating features, while vasa nervorum vasculitis and the subsequent axonopathy may be more commonly associated with mixed forms [2, 15]. On the other hand, the nerve fibre ischemia due to vasa nervorum changes is a consequence of either T-cell-mediated epineural vasculitis or humoral-mediated endoneural microangiopathy .
We present a case of bilateral sciatic neuropathy secondary to cryoglobulinaemia that did not present with an underlying infectious or neoplastic cause.
Written informed consent was obtained from the patient for publication of the Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Gemignani F, Melli G, Inglese C, Marbini A: Cryoglobulinemia is a frequent cause of peripheral neuropathy in undiagnosed referral patients. J Peripher Nerv Syst 2002, 7:59–64.View ArticlePubMedGoogle Scholar
- Cavaletti G, Petruccioli MG, Crespi V, Pioltelli P, Marmiroli P, Tredici G: A clinico-pathological and follow up study of 10 cases of essential type II cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry 1990, 53:886–889.View ArticlePubMedGoogle Scholar
- Gemignani F, Brindani F, Alfieri S, Giuberti T, Allegri I, Ferrari C, Marbini A: Clinical spectrum of cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry 2005, 76:1410–1414.View ArticlePubMedGoogle Scholar
- Safaz I, Alaca R, Bozlar U, Yaşar E: Bilateral sciatic nerve entrapment due to heterotopic ossification in a traumatic brain injured patient. Am J Phys Med Rehabil 2008, 87:65–67.View ArticlePubMedGoogle Scholar
- Yasin A, Patel AG: Bilateral sciatic nerve palsy following a bariatic operation. Obes Surg 2007, 17:983–985.View ArticlePubMedGoogle Scholar
- Roth JV: Bilateral sciatic and femoral neuropathies, rhabdomyolysis, and acute renal failure caused by positioning during radical retropubic prostatectomy. Anesth Analg 2007, 105:1747–1748.View ArticlePubMedGoogle Scholar
- Tyrrell PJ, Feher MD, Rossor MN: Sciatic nerve damage due to toilet seat entrapment: another Saturday night palsy. J Neurol Neurosurg Psychiatry 1989, 52:1113–1115.View ArticlePubMedGoogle Scholar
- Kornetzky L, Linden D, Berlit P: Bilateral sciatic nerve "Saturday night palsy". J Neurol 2001, 248:425.View ArticlePubMedGoogle Scholar
- Scherer K, Skeen MB, Strine SA, Juel VC: Hanging leg syndrome: combined bilateral femoral and sciatic neuropathies. Neurology 2006, 66:1124–1125.View ArticlePubMedGoogle Scholar
- O'Ferrall EK, Busche K, Dickhoff P, Zabad R, Toth C: A patient with bilateral sciatic neuropathies. Can J Neurol Sci 2007, 34:365–367.PubMedGoogle Scholar
- Tacconi P, Manca D, Tamburini G, Cannas A, Giagheddu M: Bed footboard peroneal and tibial neuropathy. A further unusual type of Saturday night palsy. J Peripher Nerv Syst 2004, 9:54–56.View ArticlePubMedGoogle Scholar
- Said G, Lacroix-Ciaudo C, Fujimura H, Blas C, Faux N: The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol 1988, 23:461–465.View ArticlePubMedGoogle Scholar
- Said G, Lacroix C: Primary and secondary vasculitic neuropathy. J Neurol 2005, 252:633–641.View ArticlePubMedGoogle Scholar
- Ferri C, Zignego AL, Pileri SA: Cryoglobulins. J Clin Pathol 2002, 55:4–13.PubMedGoogle Scholar
- Nemni R, Corbo M, Fazio R, Quattrini A, Comi G, Canal N: Cryoglobulinaemic neuropathy. A clinical, morphological and immunocytochemical study of 8 cases. Brain 1988, 111:541–552.View ArticlePubMedGoogle Scholar
- Gemignani F, Pavesi G, Fiocchi A, Manganelli P, Ferraccioli G, Marbini A: Peripheral neuropathy in essential mixed cryoglobulinaemia. J Neurol Neurosurg Psychiatry 1992, 55:116–120.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.