- Email: [email protected]
DRAINAGE OF MALIGNANT EFFUSIONS
43 a few. These side-effects confined almost exclusively to those patients with Paget’s disease or osteoporosis and were independent of dose. Bisphosphonates may release cytokiness and patients with malignancy may have an associated immunological defect that suppresses cytokine production. We postulate that this defect makes patients with malignancy less prone to these side-effects. Pamidronate is often given intravenously as an outpatient treatment and we therefore recommend that at the time of first dose, all infusions are administered on an inpatient basis and that patients should be observed for at least 24 h. Should an adverse reaction occur, patients should also be admitted for any subsequent infusion. None of our patients have had any long-term problems after therapy; however, patients should be warned about potential side-effects. STEPHEN J. GALLACHER STUART H. RALSTON University Department of Medicine, UDAY PATEL Glasgow Royal Infirmary, IAIN T. BOYLE Glasgow G31 2ER
was
striking although they affected only
were
1. Ralston
SH, Gardner MD, Dryburgh FJ, Jenkins AS, Cowan RA, Boyle IT. Comparison of aminohydroxypropylidene diphosphonate, mithramycin and corticosteroids/calcitonin in treatment of cancer-associated hypercalcaemia. Lancet 1985; ii: 907-10. HJ, Papapoulos SE, Blanksma, HJ, Moolengaar AJ, Vermeij P, Bijvoet
2. Harinck
OLM
Paget’s disease of bone: early and late responses to three different modes of aminohydroxypropylidene bisphosphonate (APD). Br Med J 1987,
treatment with
295: 1301-05. 3. Reid IR, King AR, Alexander CJ, Ibbertson HK. Prevention of steroid-induced osteoporosis with (3-amino-1-hydroxypropylidene)-1,1-bisphosphonate (APD). Lancet 1988; i: 143-46. 4. Cantrill JA, Buckler HM, Anderson DC Low dose intravenous 3-amino- 1-hydroxypropylidene-1,1-bisphosphonate (APD) for the treatment of Paget’s disease of bone. Ann Rheum Dis 1986; 45: 1012-18. 5. De Vnes E, Van Der Weij JP, Veen CJP, et al In vitro effect of (3-amino-1hydroxypropylidene)-1,1-bisphonic acid (APD) on the function of mononuclear phagocytes m lymphocytic proliferation. Immunology 1982; 47: 157-63. 6. O’Garra A Interleukins and the immune system 1. Lancet 1989; i: 943-47.
POTENTIAL CONFUSION OF NEUROLEPTIC MALIGNANT SYNDROME AND WILSON’S DISEASE
SIR,-At least one report has described alleged neuroleptic malignant syndrome (NMS) in Wilson’s disease. Before further cases are reported as evidence of an association, we argue that the diagnosis of NMS is difficult in Wilson’s disease since the features of each condition may overlap. Some cases of neurological Wilson’s disease present with acute symptoms, and rigidity and fever are characteristic, often occurring preterminally. Such patients are young with predominantly dystonic symptoms (older patients are more likely to have tremor). Several such individuals were reported before the neuroleptic era. A 10-year-old boy2 died after 6 months of dystonic symptoms. In the final 3 months he had fluctuating fever but no evidence of focal sepsis. For 3 weeks before death, he was disorientated. Another 10-year-old boy3 with a 6 month history had fever during the last 10 weeks of illness. He also became drowsy and doubly incontinent. The sister3 of this case died aged 15 after 14 months of illness, with raised temperature and incontinence during the last 4 weeks. A fourth case presented at age 18 with acute psychosis and had dystonic features a year later. This patient died aged 22, with fever of unknown cause during the final 3 weeks. A 22-year-old man5 died 6 weeks after onset of painful muscular spasms and myotonia. He had fever for 3 days before death. Blood cultures were negative. A 23-year-old man6 had rigidity. After treatment with British anti-lewisite and EDTA, his condition worsened. He had a fever but no signs of infection, with negative blood cultures. Wilson7 recognised two types of neurological disease, acute and chronic. In the acute form, high irregular temperature and rapid emaciation were distinctive features. Symptoms were severe from the outset and patients looked "toxic". It is unclear why fewer such cases have been reported in recent years. Possibly, early cases are described in more detail, the early (Wilsonian progressive lenticular degeneration) concept of Wilson’s disease was narrow and biased towards acute dystonic cases, and/or the modem availability of treatment and premorbid diagnosis may have rendered this picture less common. Among the Cambridge series,8,9 it is difficult to find
any definite
cases
that mimic NMS. Muscle enzymes
were not
routinely measured, but, interestingly, one 20-year-old man with dystonic features did have a raised creatine phosphokinase level, the significance of which was unclear. Later he became acutely ill, but whether he was pyrexial is uncertain. We therefore suggest that the combination of muscular rigidity and fever, often with confusion, is an integral feature of acute dystonic Wilson’s disease. This picture is readily confused with NMS (with which it may share a common lesion) and may be inappropriately treated as such. In Wilson’s disease, the appearance of fever is a grave prognostic sign. Possibly such patients require liver transplantation," since aggressive chelation may worsen symptoms. Wilson’s disease should be excluded in any young patient in whom NMS develops. Warneford Hospital, Oxford OX3 7JX
T. R. DENING
University of Cambridge Clinical School, Addenbrooke’s Hospital, Cambridge
G. E. BERRIOS
1. Kontaxakis V, Stefanis C, Markidis M, Tserpe V. Neuroleptic malignant syndrome m a patient with Wilson’s disease. J Neurol Neurosurg Psychiatry 1988; 51: 1001-02. 2. Ormerod JA. Cirrhosis of the liver in a boy, with obscure and fatal nervous symptoms. St Bartholomew’s Hospital Rep 1890; 26: 57-68. 3. Gowers WR On tetanoid chorea and its association with cirrhosis of the liver. Rev Neurol Psychiatry 1906; 4: 249-58. 4. Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34: 295-509. 5 Howard CP, Royce CE. Progressive lenticular degeneration associated with cirrhosis of the liver (Wilson’s disease). Arch Intern Med 1919; 24: 497-508. 6. Hollister LE, Cull VL, Gonda VA, Kolb FO. Hepatolenticular degeneration: clinical, biochemical and pathologic study of a patient with fulminant course aggravated by treatment with BAL and versenate. Am J Med 1960; 28: 623-30. 7. Wilson SAK Neurology. London: Edward Arnold, 1940: 811. 8. Walshe JM. Wilson’s disease (hepatolenticular degeneration). In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of clinical neurology. Vol 27. Amsterdam: North Holland, 1976: 379-414. 9. Dening TR, Berrios GE. Wilson’s disease: psychiatric symptomatology in 195 cases. Arch Gen Psychiatry (in press). 10. Polson RJ, Rolles K, Calne RY, Williams R, Marsden CD. Reversal of severe neurological manifestations of Wilson’s disease following orthotopic liver transplantation. Q JMed 1987; 64: 685-91.
DRAINAGE OF MALIGNANT EFFUSIONS or gross ascites relief. The use of a for initial require paracentesis symptom large-bore tube or Abrahms pleural biopsy needlel,2 is usually recommended for this procedure on the erroneous assumption that the fluid is viscous and will obstruct smaller needles or cannulae. The task is usually delegated to a junior doctor who may be
SIR,-Patients with malignant pleural effusions
often
inexperienced in paracentesis. For all these reasons the procedure is often painful and distressing. Small-bore cannulae (usually used for suprapubic catheterisation) can make the procedure simpler and less traumatic for both patient and doctor. Since July, 1987, we have used Bonanno catheters in over 200 patients with pleural effusions or ascites. Most patients had breast or ovarian cancer and many patients had repeated drainage. Small-bore catheters have several advantages. The introducing needle is sharp and just slightly larger than 19 G. No initial incision is required and much less force is necessary to introduce the catheter into the pleural or abdominal cavity. The cannula may be sewn directly onto the skin and should never fall out. Splinting of the ribs, which can occur when large-bore tubes are in-situ, is not a problem. On removal the entry site is so small that a simple dressing is all that is required, rather than the usual purse-string suture. In our experience blockage of the cannula by viscous fluid is rare, even in patients who have heavily blood-stained effusions with a high protein content. Furthermore, agents such as bleomycin or tetracycline for chemical pleurodesis are easily inserted. We advocate more widespread use of such small-bore cannulae for drainage of malignant pleural or peritoneal effusions. Royal Marsden Hospital, London SW3 6JJ
J. L. MANSI G. W. HANKS
1. Ford MJ, Munro JF. Intercostal drainage. In: Practical procedures in clinical medicine. London. Churchill Livingstone, 1980: 48-57 2 lles PB, Ogilvie C. Pleural aspiration and biopsy. In: Procedures in practice. London: British Medical Journal, 1988: 92-98.
was
striking although they affected only
were
1. Ralston
SH, Gardner MD, Dryburgh FJ, Jenkins AS, Cowan RA, Boyle IT. Comparison of aminohydroxypropylidene diphosphonate, mithramycin and corticosteroids/calcitonin in treatment of cancer-associated hypercalcaemia. Lancet 1985; ii: 907-10. HJ, Papapoulos SE, Blanksma, HJ, Moolengaar AJ, Vermeij P, Bijvoet
2. Harinck
OLM
Paget’s disease of bone: early and late responses to three different modes of aminohydroxypropylidene bisphosphonate (APD). Br Med J 1987,
treatment with
295: 1301-05. 3. Reid IR, King AR, Alexander CJ, Ibbertson HK. Prevention of steroid-induced osteoporosis with (3-amino-1-hydroxypropylidene)-1,1-bisphosphonate (APD). Lancet 1988; i: 143-46. 4. Cantrill JA, Buckler HM, Anderson DC Low dose intravenous 3-amino- 1-hydroxypropylidene-1,1-bisphosphonate (APD) for the treatment of Paget’s disease of bone. Ann Rheum Dis 1986; 45: 1012-18. 5. De Vnes E, Van Der Weij JP, Veen CJP, et al In vitro effect of (3-amino-1hydroxypropylidene)-1,1-bisphonic acid (APD) on the function of mononuclear phagocytes m lymphocytic proliferation. Immunology 1982; 47: 157-63. 6. O’Garra A Interleukins and the immune system 1. Lancet 1989; i: 943-47.
POTENTIAL CONFUSION OF NEUROLEPTIC MALIGNANT SYNDROME AND WILSON’S DISEASE
SIR,-At least one report has described alleged neuroleptic malignant syndrome (NMS) in Wilson’s disease. Before further cases are reported as evidence of an association, we argue that the diagnosis of NMS is difficult in Wilson’s disease since the features of each condition may overlap. Some cases of neurological Wilson’s disease present with acute symptoms, and rigidity and fever are characteristic, often occurring preterminally. Such patients are young with predominantly dystonic symptoms (older patients are more likely to have tremor). Several such individuals were reported before the neuroleptic era. A 10-year-old boy2 died after 6 months of dystonic symptoms. In the final 3 months he had fluctuating fever but no evidence of focal sepsis. For 3 weeks before death, he was disorientated. Another 10-year-old boy3 with a 6 month history had fever during the last 10 weeks of illness. He also became drowsy and doubly incontinent. The sister3 of this case died aged 15 after 14 months of illness, with raised temperature and incontinence during the last 4 weeks. A fourth case presented at age 18 with acute psychosis and had dystonic features a year later. This patient died aged 22, with fever of unknown cause during the final 3 weeks. A 22-year-old man5 died 6 weeks after onset of painful muscular spasms and myotonia. He had fever for 3 days before death. Blood cultures were negative. A 23-year-old man6 had rigidity. After treatment with British anti-lewisite and EDTA, his condition worsened. He had a fever but no signs of infection, with negative blood cultures. Wilson7 recognised two types of neurological disease, acute and chronic. In the acute form, high irregular temperature and rapid emaciation were distinctive features. Symptoms were severe from the outset and patients looked "toxic". It is unclear why fewer such cases have been reported in recent years. Possibly, early cases are described in more detail, the early (Wilsonian progressive lenticular degeneration) concept of Wilson’s disease was narrow and biased towards acute dystonic cases, and/or the modem availability of treatment and premorbid diagnosis may have rendered this picture less common. Among the Cambridge series,8,9 it is difficult to find
any definite
cases
that mimic NMS. Muscle enzymes
were not
routinely measured, but, interestingly, one 20-year-old man with dystonic features did have a raised creatine phosphokinase level, the significance of which was unclear. Later he became acutely ill, but whether he was pyrexial is uncertain. We therefore suggest that the combination of muscular rigidity and fever, often with confusion, is an integral feature of acute dystonic Wilson’s disease. This picture is readily confused with NMS (with which it may share a common lesion) and may be inappropriately treated as such. In Wilson’s disease, the appearance of fever is a grave prognostic sign. Possibly such patients require liver transplantation," since aggressive chelation may worsen symptoms. Wilson’s disease should be excluded in any young patient in whom NMS develops. Warneford Hospital, Oxford OX3 7JX
T. R. DENING
University of Cambridge Clinical School, Addenbrooke’s Hospital, Cambridge
G. E. BERRIOS
1. Kontaxakis V, Stefanis C, Markidis M, Tserpe V. Neuroleptic malignant syndrome m a patient with Wilson’s disease. J Neurol Neurosurg Psychiatry 1988; 51: 1001-02. 2. Ormerod JA. Cirrhosis of the liver in a boy, with obscure and fatal nervous symptoms. St Bartholomew’s Hospital Rep 1890; 26: 57-68. 3. Gowers WR On tetanoid chorea and its association with cirrhosis of the liver. Rev Neurol Psychiatry 1906; 4: 249-58. 4. Wilson SAK. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain 1912; 34: 295-509. 5 Howard CP, Royce CE. Progressive lenticular degeneration associated with cirrhosis of the liver (Wilson’s disease). Arch Intern Med 1919; 24: 497-508. 6. Hollister LE, Cull VL, Gonda VA, Kolb FO. Hepatolenticular degeneration: clinical, biochemical and pathologic study of a patient with fulminant course aggravated by treatment with BAL and versenate. Am J Med 1960; 28: 623-30. 7. Wilson SAK Neurology. London: Edward Arnold, 1940: 811. 8. Walshe JM. Wilson’s disease (hepatolenticular degeneration). In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of clinical neurology. Vol 27. Amsterdam: North Holland, 1976: 379-414. 9. Dening TR, Berrios GE. Wilson’s disease: psychiatric symptomatology in 195 cases. Arch Gen Psychiatry (in press). 10. Polson RJ, Rolles K, Calne RY, Williams R, Marsden CD. Reversal of severe neurological manifestations of Wilson’s disease following orthotopic liver transplantation. Q JMed 1987; 64: 685-91.
DRAINAGE OF MALIGNANT EFFUSIONS or gross ascites relief. The use of a for initial require paracentesis symptom large-bore tube or Abrahms pleural biopsy needlel,2 is usually recommended for this procedure on the erroneous assumption that the fluid is viscous and will obstruct smaller needles or cannulae. The task is usually delegated to a junior doctor who may be
SIR,-Patients with malignant pleural effusions
often
inexperienced in paracentesis. For all these reasons the procedure is often painful and distressing. Small-bore cannulae (usually used for suprapubic catheterisation) can make the procedure simpler and less traumatic for both patient and doctor. Since July, 1987, we have used Bonanno catheters in over 200 patients with pleural effusions or ascites. Most patients had breast or ovarian cancer and many patients had repeated drainage. Small-bore catheters have several advantages. The introducing needle is sharp and just slightly larger than 19 G. No initial incision is required and much less force is necessary to introduce the catheter into the pleural or abdominal cavity. The cannula may be sewn directly onto the skin and should never fall out. Splinting of the ribs, which can occur when large-bore tubes are in-situ, is not a problem. On removal the entry site is so small that a simple dressing is all that is required, rather than the usual purse-string suture. In our experience blockage of the cannula by viscous fluid is rare, even in patients who have heavily blood-stained effusions with a high protein content. Furthermore, agents such as bleomycin or tetracycline for chemical pleurodesis are easily inserted. We advocate more widespread use of such small-bore cannulae for drainage of malignant pleural or peritoneal effusions. Royal Marsden Hospital, London SW3 6JJ
J. L. MANSI G. W. HANKS
1. Ford MJ, Munro JF. Intercostal drainage. In: Practical procedures in clinical medicine. London. Churchill Livingstone, 1980: 48-57 2 lles PB, Ogilvie C. Pleural aspiration and biopsy. In: Procedures in practice. London: British Medical Journal, 1988: 92-98.