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Subacute motor weakness and left renal mass

      To the Editor:
      A 65-year-old white woman presented with facial palsy and progressive weakness of both arms and legs. She had no previous history of fever, chills, recent infections, allergies, recent immunization, exposure to neurotoxic chemicals, and use of tobacco, illicit drugs, and alcohol. Her family history was unremarkable. Her temperature was 36.6°C, pulse was 105 beats per minute, and respiration rate was 25 breaths per minute. Blood pressure was 110/90 mm Hg. Hematologic laboratory values were normal. The physical examination showed no abnormalities. The patient was alert. Sensitivity to light, touch, and pinprick was normal, as was coordination. Neurologic examination revealed bilateral distal weakness on dorsal flexion of the feet, tingling paresthesias in the hands, and loss of tendon reflexes in both arms and legs. Muscle bulk, tone, and strength were normal. Neurophysiologic findings suggested a polyneuropathy of recent onset. Examination of the right median and tibial nerves showed reduced motor nerve conduction velocities in the upper and lower limbs. F waves and H reflex recorded after stimulation of tibial nerves were absent. Repetitive stimulation studies of the intrinsic hand muscles before and after exercise showed no decreased responses or postexercise facilitation. Needle electromyography showed no denervation potentials and a reduced pattern of motor unit discharge at maximum effort, which correlate with the degree of weakness. Analysis of the cerebrospinal fluid showed albuminocytologic dissociation with a marked increase in protein concentration (1.3 g/L) and mild pleocytosis (lymphocyte count, 10/mm3). In the following days, weakness of the legs worsened and right facial palsy became evident, requiring the patient to be confined to bed. The slow conduction velocities, presence of conduction block, and absence of F wave and H reflex indicated a demyelinating peripheral neuropathy. Biopsy specimens of the sural nerve were uninformative.
      The diagnosis of Guillain-Barré syndrome was made, using the criteria of Asbury and Cornblath (
      • Asbury A.K.
      • Cornblath D.R.
      Assessment of current diagnostic criteria for Guillain-Barre syndrome.
      ). Treatment with plasma exchange (total exchange, 3.5 L) and intravenous immunoglobulin (0.4 g/kg/d for 5 days) was started. Corticosteroids were not administered. The patient’s motor weakness improved. After 6 days, there was complete neurological recovery and she was able to walk. She was discharged after 2 weeks of hospitalization and instructed to take methylprednisolone (500 mg/d) and undergo rehabilitation.
      One month later, she presented again with weakness of arms and legs. She was unable to stand on her toes or heels, and deep tendon reflexes were absent. Despite corticosteroid use, her neurological symptoms had worsened and right facial palsy had returned with pain in the left flank. Abdominal ultrasonography revealed a 3.5-cm mass of heterogeneous configuration in the left kidney. Total body computed tomography (CT) confirmed the presence of the mass but ruled out metastatic disease; a bone scan was negative. A left nephroureterectomy was performed. A moderately differentiated clear cell carcinoma limited to the kidney was found, classified as stage I (T1N0M0). After nephrectomy, the patient’s condition improved. She remained in good health until about a year later when the neurological symptoms returned. She underwent a total body CT scan, which showed metastatic renal tumor dissemination to the liver, lung, and brain. However, she died before starting any first-line therapy.
      Several neoplasias may manifest initially as a paraneoplastic neurological syndrome, although the pathogenesis is not known. Some authors have described a cross-reaction between autoantibodies against intracellular or membrane neoplastic antigens and neuronal nuclear or membrane antigens (
      • Antoine J.C.
      • Mosnier J.F.
      • Absi L.
      • Convers P.
      • Honnorat J.
      • Michel D.
      Carcinoma associated paraneoplastic peripheral neuropathies in patients with and without anti-onconeural antibodies.
      ). These neuronal antibodies often cannot be identified. In patients without antibodies, neuropathies that occur about 3 years before cancer diagnosis are probably paraneoplastic; for neuropathies appearing many years before diagnosis, the association is most likely coincidental. Renal cell carcinomas are rarely associated with neurologic paraneoplastic syndromes (
      • Tveter K.J.
      Unusual manifestations of renal carcinoma. A review of the literature.
      ,
      • Phan T.G.
      • Hersch M.
      • Zagami A.S.
      Guillain-Barré syndrome and adenocarcinoma of the gall bladder a paraneoplastic phenomenon?.
      ), and Guillain-Barré symptoms rarely occur with solid tumors (
      • Oh S.J.
      • Slaughter R.
      • Harrell L.
      Paraneoplastic vasculitic neuropathy a treatable neuropathy.
      ,
      • Peterson K.
      • Forsyth P.A.
      • Posner J.B.
      Paraneoplastic sensorimotor neuropathy associated with breast cancer.
      ).
      We did not examine cerebrospinal fluid for malignant cells because leptomeningeal carcinomatosis is common in malignant lymphoproliferative diseases and is rarely the presenting feature of a solid tumor. The types of solid tumor most commonly associated with this condition are breast, lung, and gastrointestinal carcinomas and malignant melanoma. There has been one report of cancer of probable renal origin with meningeal carcinomatosis (
      • Yamada M.
      • Shintani S.
      • Mitani K.
      • et al.
      Peripheral neuropathy with predominantly motor manifestations in a patient with carcinoma of the uterus.
      ). Meningeal carcinomatosis usually presents with multiple cranial neuropathies, patchy radiculopathies, and changes in mental status (
      • Camps C.
      • Soler J.J.
      • Casado I.
      • Cervello M.
      • Chirivella M.
      • Sancho J.
      Leptomeningeal carcinomatosis as the primary manifestation of a carcinoma of probable renal origin.
      ,
      • Wong E.T.
      • Joseph J.T.
      Meningeal carcinomatosis in lung cancer. Case 1. Carcinomatous leptomeningeal metastases.
      ), which was not the case with this patient. Moreover, resolution of the neurological manifestations either after plasmapheresis or nephrectomy confirmed the autoimmune origin of the acute polyneuropathy.
      Our report suggests an association between renal cell carcinoma and paraneoplastic Guillain-Barré syndrome, and that nephrectomy can lead to the dramatic resolution of neurological symptoms. As observed in this patient, neurological symptoms may precede cancer diagnosis. Hence, prompt recognition of Guillain-Barré syndrome may anticipate the diagnosis of an early renal malignancy or its progression.

      References

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        Carcinoma associated paraneoplastic peripheral neuropathies in patients with and without anti-onconeural antibodies.
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        Unusual manifestations of renal carcinoma. A review of the literature.
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        Guillain-Barré syndrome and adenocarcinoma of the gall bladder.
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        Paraneoplastic sensorimotor neuropathy associated with breast cancer.
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        Peripheral neuropathy with predominantly motor manifestations in a patient with carcinoma of the uterus.
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