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Paraneoplastic cauda equina syndrome secondary to B-cell lymphoma
Clinical Neurology and Neurosurgery ELSEVIER
Clinical Neurology and Neurosurgery 99 (1997) 71-72
Letter to the Editor
Paraneoplastic cauda equina syndrome secondary to B-cell lymphoma G.C.Y. Fong*, K.Y. Fong Division ~ Neurology, University Department of Medicine, Queen MaO, Hospital, The UniversiO, o[ Hong Kong, 12 Polffulam Road. Hong Kong, Hong Kong
Received 25 June 1996: revised 25 July 1996; accepted 19 August 1996
I. Case report A 62-year-old male retired textile merchant presented with a 4 weeks' history of progressive bilateral lower limb weakness. It was associated with urinary retention and pain in the upper thigh and perineal region. Neurological examination showed flaccid paraplegia, depressed lower limb reflexes but normal plantar response. There was impairment of all sensory modalities below T12. The anal tone was mildly reduced. Examination did not review any lymphadenopathy or skir rash. No organomegaly was detected and cardiow~scular and respiratory system examination were norn:al. Investigations rew;aled normochromic normocytic anaemia (Hb = 9.3 g/dl) and elevated alkaline phosphatase (1084 U/I), alanine aminotransferase (309 U/l) and aspartate aminotransferase (94 U/l). Computerized tomography of abdomen and thoracic spine, MRI of brain, MRI of thoracic and lumbar spine with gadolinium enhancement and bone marrow examination were all normal Cerebrospinal fluid examination was acellular with high protein (0.88 gm/l), moderately depressed glucose level of 2.5 mmol/l (blood glucose = 6.8 mmol/l) but without evidence of malignant cells. Repeated examination of CSF for twice within two months of onset showed similar findings. Nerve conduction studies were normal but F-waves were clearly absent following stimulations of both tibial nerve and left peroneal nerve. Electromyogram demonstrated denervation changes over left tibialis anterior and vas-
* Corresponding author. 0303-8467:97/$17.00 ~) 1997 Elsevier Science B.V. All rights reserved. PII S0303-8467(96100052-2
tus lateralis. The electrophysiological studies were therefore consistent with a diffuse lumbosacral radiculopathy. Liver biopsy was performed because of elevated alkaline phosphatase which showed high grade B cell malignant lymphoma. Although bone marrow examination after 2 months showed dissemination of lymphoma, CSF examination and MRI lumbosacral root and plexus were repeatedly normal. There was no serological evidence to support a concurrent CMV infection.
2. Discussion In summary, this patient suffered from high grade B-cell lymphoma infiltrating both liver and bone marrow, but initially presented with a progressive cauda equina syndrome. Repeated CSF and imaging studies failed to show direct CNS involvement or lumbosacral root infiltration by lymphoma cell. The diagnosis of a paraneoplastic cauda equina syndrome was made. Peripherial nervous system and spinal cord involvement in malignant lymphoma included herpes zoster infection, toxic polyneuropathy, compressive neuropathy, radiation myeiopathy and Guillain-Barr6 Syndrome (associated with Hodgkin's lymphoma) [1]. Cauda equina syndrome has hitherto not been reported as a paraneoplastic syndrome. Direct involvement of the cauda equina by lymphoma, in contrast to leukaemia, is extremely rare. There were only 3 reported cases of cauda equina syndrome due to infiltration of angiotrophic lymphoma of B cell origin but with normal MRI findings [2,3]. Angiotrophic lymphoma (neoplastic an
72
G.C.Y. Fong, K.Y. Fong .. Clinical Neuroh)ey and :\"eurosurgery 99 (1997) 71 72
gioendotheliomatosis) characteristically causes multiple organ involvement with intravascular proliferation o f malignant cell. A p a r t from cutaneous and neurological manifestations, it may present initially in other sites including lung, liver, gastrointestinal tract, cervix, prostate, nasal cavity, bone, and skeletal muscle [4]. It usually runs a rapid progressive course resulting in death within 15 months and resistant to chemotherapy. The relatively static clinical course, lack o f skin rash and liver biopsy finding in our patient were against angiotrophic lymphoma.
References [1] Correale J, Monteverde DA, Bueri JA. Reich EG. Peripheral nervous system and spinal cord involvement in lymphoma; Acta Neurol Scand 1991: 83:45 51. [2] Laeomis D, Smith T W , Long RR. Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome. Clin Neurol Neurosurg 1992; 94:311 315. [3] Hamada K, Hamada T. Satoh M, Tashiro K, Katoh I. Naganuma M, Shima K, Ogata A. Nagashima K. Two cases of neoplastic angioendotheliomatosis presenting with myelopathy. Neurology 1991; 41: 1139-1140. [4] Rosai J. Skin:Tumors and Tumorlikc Conditions; Ackerman's Surgical Pathology; Chapter 4 pp 53 172.
Clinical Neurology and Neurosurgery 99 (1997) 71-72
Letter to the Editor
Paraneoplastic cauda equina syndrome secondary to B-cell lymphoma G.C.Y. Fong*, K.Y. Fong Division ~ Neurology, University Department of Medicine, Queen MaO, Hospital, The UniversiO, o[ Hong Kong, 12 Polffulam Road. Hong Kong, Hong Kong
Received 25 June 1996: revised 25 July 1996; accepted 19 August 1996
I. Case report A 62-year-old male retired textile merchant presented with a 4 weeks' history of progressive bilateral lower limb weakness. It was associated with urinary retention and pain in the upper thigh and perineal region. Neurological examination showed flaccid paraplegia, depressed lower limb reflexes but normal plantar response. There was impairment of all sensory modalities below T12. The anal tone was mildly reduced. Examination did not review any lymphadenopathy or skir rash. No organomegaly was detected and cardiow~scular and respiratory system examination were norn:al. Investigations rew;aled normochromic normocytic anaemia (Hb = 9.3 g/dl) and elevated alkaline phosphatase (1084 U/I), alanine aminotransferase (309 U/l) and aspartate aminotransferase (94 U/l). Computerized tomography of abdomen and thoracic spine, MRI of brain, MRI of thoracic and lumbar spine with gadolinium enhancement and bone marrow examination were all normal Cerebrospinal fluid examination was acellular with high protein (0.88 gm/l), moderately depressed glucose level of 2.5 mmol/l (blood glucose = 6.8 mmol/l) but without evidence of malignant cells. Repeated examination of CSF for twice within two months of onset showed similar findings. Nerve conduction studies were normal but F-waves were clearly absent following stimulations of both tibial nerve and left peroneal nerve. Electromyogram demonstrated denervation changes over left tibialis anterior and vas-
* Corresponding author. 0303-8467:97/$17.00 ~) 1997 Elsevier Science B.V. All rights reserved. PII S0303-8467(96100052-2
tus lateralis. The electrophysiological studies were therefore consistent with a diffuse lumbosacral radiculopathy. Liver biopsy was performed because of elevated alkaline phosphatase which showed high grade B cell malignant lymphoma. Although bone marrow examination after 2 months showed dissemination of lymphoma, CSF examination and MRI lumbosacral root and plexus were repeatedly normal. There was no serological evidence to support a concurrent CMV infection.
2. Discussion In summary, this patient suffered from high grade B-cell lymphoma infiltrating both liver and bone marrow, but initially presented with a progressive cauda equina syndrome. Repeated CSF and imaging studies failed to show direct CNS involvement or lumbosacral root infiltration by lymphoma cell. The diagnosis of a paraneoplastic cauda equina syndrome was made. Peripherial nervous system and spinal cord involvement in malignant lymphoma included herpes zoster infection, toxic polyneuropathy, compressive neuropathy, radiation myeiopathy and Guillain-Barr6 Syndrome (associated with Hodgkin's lymphoma) [1]. Cauda equina syndrome has hitherto not been reported as a paraneoplastic syndrome. Direct involvement of the cauda equina by lymphoma, in contrast to leukaemia, is extremely rare. There were only 3 reported cases of cauda equina syndrome due to infiltration of angiotrophic lymphoma of B cell origin but with normal MRI findings [2,3]. Angiotrophic lymphoma (neoplastic an
72
G.C.Y. Fong, K.Y. Fong .. Clinical Neuroh)ey and :\"eurosurgery 99 (1997) 71 72
gioendotheliomatosis) characteristically causes multiple organ involvement with intravascular proliferation o f malignant cell. A p a r t from cutaneous and neurological manifestations, it may present initially in other sites including lung, liver, gastrointestinal tract, cervix, prostate, nasal cavity, bone, and skeletal muscle [4]. It usually runs a rapid progressive course resulting in death within 15 months and resistant to chemotherapy. The relatively static clinical course, lack o f skin rash and liver biopsy finding in our patient were against angiotrophic lymphoma.
References [1] Correale J, Monteverde DA, Bueri JA. Reich EG. Peripheral nervous system and spinal cord involvement in lymphoma; Acta Neurol Scand 1991: 83:45 51. [2] Laeomis D, Smith T W , Long RR. Angiotropic lymphoma (intravascular large cell lymphoma) presenting with cauda equina syndrome. Clin Neurol Neurosurg 1992; 94:311 315. [3] Hamada K, Hamada T. Satoh M, Tashiro K, Katoh I. Naganuma M, Shima K, Ogata A. Nagashima K. Two cases of neoplastic angioendotheliomatosis presenting with myelopathy. Neurology 1991; 41: 1139-1140. [4] Rosai J. Skin:Tumors and Tumorlikc Conditions; Ackerman's Surgical Pathology; Chapter 4 pp 53 172.