JAGS
FEBRUARY 2014–VOL. 62, NO. 2
temporal lobe memory system.1 This type of memory makes evolutionary sense. For example, one does not need to be stung repeatedly by wasps before one finally remembers to avoid them; a single exposure is forever sufficient. Although emotional and declarative memories appear to follow different paths, it is not clear whether decrements in memory function also follow separate paths in memory disease. Herein is presented a case of a woman with vascular dementia in whom emotional memory was relatively spared.
CASE PRESENTATION An 80-year-old woman had a history of progressive vascular dementia, type II diabetes mellitus, and congestive heart failure (CHF). Her dementia limited her overall function, and she was unable to perform instrumental activities of daily living. She received donepezil 10 mg/d, memantine 10 mg twice daily, isosorbide dinitrate extended release 40 mg twice daily, glipizide extended release 20 mg/d, nifedipine extended release 10 mg twice daily, and nitroglycerine 0.4 mg sublingual, the last as needed for angina pectoris. One night, she experienced a hypnopompic hallucination of a man standing at the foot of her bed. The event was frightening to her, and she reported it with the belief that it had happened. There was no other evidence of psychosis, and she was not treated with any antipsychotic drugs. She continued to recall the event spontaneously over the subsequent 14 months until her death of CHF, although she was unable to recall other important events that occurred in her life at approximately the same time (e.g., the death of a dear friend), and she continued to have difficulty with all short-term memories (e.g., losing items, forgetting appointments, inability to manage funds).
DISCUSSION This woman with vascular dementia, demonstrated by decline in function and inability to form new declarative memories, was able to recall a single frightful event for longer than a year, as demonstrated by her spontaneous verbalization of the memory. Although the event she recalled was not real, but the result of a hypnopompic hallucination, it was experienced and recalled as real. Hypnopompic hallucinations occur in 10% to 20% of individuals with normal cognition and are not considered a psychotic process.8 There is some evidence that the amygdala may be relatively spared in aging and dementing processes.9 The amygdala appears to play an important role in the consolidation of memory of emotionally arousing events, especially fear.3,5 Emotional arousal and encoding of emotional memories that are mediated through the amygdala may bypass these dysfunctional pathways. It has been suggested that the amygdala responds rapidly and before awareness to emotional stimuli in the environment. This may lead to facilitation of attention and the overall increased vigilance observed in the presence of an emotional stimulus.3 It also means that intact cortical function may be of less importance for memory in the setting of emotional stimuli. This phenomenon may underlie the relative sparing of emotional memory in Alzheimer’s disease and related disorders.
LETTERS TO THE EDITOR
405
There have been only a small number of cases reported in which emotional memory is preserved in the setting of memory disturbance,10 and this process may be underrecognized. Nonetheless, such cases demonstrate the differences between creation of emotional memories and declarative memories. Thripura Thirtala, MD Kanwaldeep Kaur, MD Yonglin Gao, MD Steven Lippmann, MD Rif S. El-Mallakh, MD Mood Disorders Research Program Department of Psychiatry and Behavioral Sciences School of Medicine University of Louisville Louisville Kentucky
ACKNOWLEDGMENTS Conflict of Interest: Dr. El-Mallakh is on the speakers’ bureau for Astraz Zeneca and Otsuka. None of the other authors have any conflicts of interest to report. Author Contributions: All authors contributed to all aspects of this paper. Sponsor’s Role: None.
REFERENCES 1. Dolcos F, LeBar KS, Cabeza R. Remembering one year later: Role of the amygdala and the medial temporal lobe memory system in retrieving emotional memories. Proc Natl Acad Sci USA 2005;102:2626–2631. 2. Cahill L, Babinsky R, Markowitsch HJ et al. The amygdala and emotional memory. Nature 1995;377:295–296. 3. Amaral DG, Behniea H, Kelly JL. Topographic organization of projections from the amygdala to the visual cortex in the macaque monkey. Neuroscience 2003;118:1099–1120. 4. Mori E, Ikeda M, Hirono N et al. Amygdalar volume and emotional memory in Alzheimer’s disease. Am J Psychiatry 1999;156:216–222. 5. McGaugh JL. Memory reconsolidation hypothesis revived but restrained: Theoretical comment on Biedenkapp and Rudy. Behav Neurosci 2004;118:1140–1142. 6. McGaugh JL, Cahill L, Roozendaal B. Involvement of the amygdala in memory storage: Interaction with other brain systems. Proc Natl Acad Sci USA 1996;93:13508–13514. 7. Cordero MI, Venero C, Kruyt ND et al. Prior exposure to a single stress session facilitates subsequent contextual fear conditioning in rats: Evidence for a role of corticosterone. Horm Behav 2003;44:338–345. 8. Ohayon MM, Priest RG, Caulet M et al. Hypnagogic and hypnopompic hallucinations: Pathological phenomena. Br J Psychiatry 1996;169: 459–467. 9. Scheibe S, Carstensen LL. Emotional aging: Recent findings and future trends. J Gerontol B Psychol Sci Soc Sci 2010;65B:135–144. 10. Okada A, Matsuo J. Emotional memory in patients with Alzheimer’s disease: A report of two cases. Case Rep Psychiatry 2012;2012:313906.
SPLENIC MARGINAL ZONE LYMPHOMA INVOLVING THE BREAST: LESSONS IN PATIENT-CENTERED CARE To the Editor: Splenic marginal zone lymphoma (SMZL) is a rare, indolent non-Hodgkin’s lymphoma that commonly involves the spleen, bone marrow, and blood. This letter describes a 78-year-old woman with long-standing SMZL presenting with a rare case of breast involvement. Treatment options are not well researched in older adults who have limited life expectancy because of multiple comorbidities. The complex decision-making process included
406
LETTERS TO THE EDITOR
exploring the woman’s life circumstances and behaviors. The only therapeutic option available was radiation therapy, which would not reduce mortality but might reduce disease-specific morbidity. Quality of life may be compromised because of side effects from radiation therapy. This case describes additional challenges of decision-making in the context of cognitive impairment.
CASE REPORT A 78-year-old woman presented to the clinic with a new left breast mass that she had noticed incidentally while bathing. She denied a change in the size of the mass, pain, or nipple discharge. Her past medical history included type 2 diabetes mellitus (glycosylated hemoglobin 7.7 mmol/L), hyperlipidemia, hypertension, vitamin D deficiency, dementia of the Alzheimer’s type (Functional Assessment Staging scale 5), and interstitial lung disease. She had had three recent hospital admissions for dyspnea in relation to her interstitial lung disease. She also had a history of splenic marginal zone lymphoma stage IV A, treated 8 years before with splenectomy. Recurrent disease presenting with anemia that was treated with rituximab followed this surgery 9 months later. She was independent in basic activities of daily living but needed help with instrumental activities of daily living. Physical examination was remarkable for a thin older woman sitting comfortably in the chair. There was a 3-by 3-cm firm mass in the right upper quadrant of the left breast. There were no skin changes, tenderness, redness, or palpable axillary lymph nodes. She was able to stand from the chair without help and ambulate independently. A mammogram demonstrated an ill-defined mass in the upper inner quadrant at the 11:00 position 7 cm from the nipple measuring 3.5 by 2.5 by 0.9 cm corresponding to the palpable mass. No axillary lymph nodes were noted. The biopsy results showed low-grade B-cell lymphoma consistent with marginal zone type. Computed tomography showed no other areas suspicious for lymphomatous involvement. It was not possible to perform a positron emission tomography scan due because of her high serum glucose level. In view of the localized nature of her disease, a trial of radiation therapy was recommended. She asked about her overall prognosis and her quality of life with and without treatment.
FEBRUARY 2014–VOL. 62, NO. 2
JAGS
Willebrand disease). Other features are acquired C1 esterase inhibitor deficiency and high paraprotein levels. It is uncommon to find lymphadenopathy or extralymphatic organ involvement. B symptoms and high lactic dehydrogenase levels are features of the aggressive form of this disease. In some individuals, there may be an association with hepatitis C, and in these individuals, the lymphoma may respond to antiviral treatment of the hepatitis C. The disease prognosis is fairly good, with median survival of 10 years. Online calculators were used to determine that this woman had a high risk of all-cause mortality (Lee Index: 4-year mortality 44–46%; Schonberg Index: 5-year mortality 43% aged 39–47, 9-year mortality 75% aged 69–80).3,4 The only treatment option available was radiation therapy, which might not have extended her life expectancy but might have had long-term morbidity benefit by shrinking the mass and preventing local complications. Radiation therapy could have associated undesirable side effects that might affect quality of life. In summary, this was a community-dwelling older woman with mild dementia, multiple medical problems, three recent hospitalizations for dyspnea, and a remote history of SMZL now presenting with breast involvement. This presenting problem was not likely to cause her death but in combination with her other comorbidities increased her mortality risk. It was not possible to find information describing the course of the illness in individuals who do not receive treatment. Decision-making was based on the woman’s and her family’s preferences.5,6 The process of adapting best evidence to the care of individuals is called “patient-centered decision-making.” Failure to individualize care is a type of medical error termed “contextual error.” Contextual errors are due to inattention to the individual’s context (e.g., social support, economic situation, personal beliefs, past experiences), as opposed to biomedical errors. In this case, the woman’s comorbid diseases, especially dementia and interstitial lung disease causing multiple hospitalizations; social circumstances; and personal preferences were important components in the decision-making process. Given the indolent nature of the disease and possible side effects from radiation therapy, she decided against treatment with radiation therapy. She did not keep her follow-up appointments, and repeated efforts to contact her were unsuccessful. The family and case manager indicated that she was not interested in additional interventions to treat her breast condition.
DISCUSSION Splenic marginal zone lymphoma is a rare form of nonHodgkin lymphoma, accounting for 1% to 2% of all cases.1,2 It is postulated to arise from a postgerminal center, memory B-cell of splenic type. It usually involves the spleen, bone marrow, and peripheral blood; the median age at presentation is 65. It is common for the disease to be asymptomatic. Typical presenting features include splenomegaly, fatigue, abdominal discomfort, lymphocytosis, and cytopenias. Diagnosis is based on lymphocyte morphology, immunophenotype, marrow, and splenic histology. Autoimmune phenomena are found in 10% of cases (including warm antibody autoimmune hemolysis, cold agglutinins, immune thrombocytopenia, anticardiolipin antibodies, lupus anticoagulant, and acquired von
Ariba Khan, MBBS, MPH Michael L. Malone, MD Charles Bomzer, MD Aurora Health Care, Milwaukee, Wisconsin School of Medicine and Public Health, University of Wisconsin, Milwaukee, Wisconsin
ACKNOWLEDGMENTS The authors would like to thank Kathy Strube, Aurora Health Care Librarian, for her excellent bibliographic search. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors
JAGS
FEBRUARY 2014–VOL. 62, NO. 2
LETTERS TO THE EDITOR
407
and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Khan: acquisition of subject, study concept, bibliographic search, preparation of manuscript. Malone: preparation and revision of manuscript. Bomzer: acquisition of subject, study concept, revision of manuscript. Sponsor’s Role: No sponsor.
further diagnostic examination including polysomnography and nocturnal penile tumescence and rigidity monitoring. He was diagnosed with SRPEs and initiated on low-dose clonazepam 0.5 mg at bedtime. After 2 weeks of treatment, with frequency of nocturnal erections reduced to approximately three per night, the dose was increased to 1 mg/d, leading to complete resolution of the erections. He remains on 1 mg with no further symptoms 1 year since diagnosis.
REFERENCES
DISCUSSION
1. Oscier D, Owen R, Johnson S. Splenic marginal zone lymphoma. Blood Rev 2005;19:39–51. 2. Thieblemont C, Davi F, Noguera ME et al. Splenic marginal zone lymphoma: Current knowledge and future direction. Oncology (Williston Park) 2012;26:194–202. 3. Yourman LC, Lee SJ, Schonberg MA et al. Prognostic indices for older adults: A systemic review. JAMA 2012;307:182–192. 4. Weiner SJ, Schwartz A, Sharma G et al. Patient-centered decision making and health care outcomes: An observational study. Ann Intern Med 2013;158:573–579. 5. ePrognosis. Estimating prognosis for elders. Available at www.ePrognosis. org Accessed April 22, 2013. 6. Patient-centered care for older adults with multiple chronic conditions: A stepwise approach from the American Geriatrics Society: American Geriatrics Society Expert Panel on the Care of Older Adults with Multimorbidity. J Am Geriatr Soc 2012;60:1957–1968.
SLEEP-RELATED PAINFUL ERECTIONS IN AN ELDERLY MAN SUCCESSFULLY TREATED USING CLONAZEPAM To the Editor: Sleep-related painful erections (SRPE) are characterized by deep penile pain that occurs during erections in the rapid eye movement (REM) stage of sleep. Since its description in 1971,1 only 33 cases have been reported in the literature albeit none in older men. We report a case of nocturnal painful erections in an elderly man successfully treated using clonazepam.
CASE A 77-year-old Caucasian man with a long-standing history of essential tremors well controlled on propranolol presented with a 2-year history of nocturnal erections with recent worsening. He complained of painful penile erections after midnight, lasting for 3 to 5 minutes, which woke him up from sleep and that urination only partially relieved. He was able to fall back asleep only to have the pain wake him again. The frequency gradually increased from two to three episodes a night to one every hour and would continue until he woke up in the morning. There was no history of other sleep disorder or parasomnia. He reported being satisfied with his sexual life with his girlfriend and had normal rigid painless erections during sexual intercourse. He had not noticed any change in the quality of his erections or ejaculations since the onset of symptoms. He denied obsession with any sexual thoughts and was not taking any testosterone supplements or sexual stimulants. He had an unremarkable urological, digital rectal, and neurological examination. Blood and urine testing revealed no uropathogenic infection. Serum prostate-specific antigen and testosterone were within normal limits. He had a disappointing consultation with the urologists. He refused
Erectile pain is seen in conditions such as Peyronie’s disease, phimosis, urethritis, and metastatic carcinoma, with painful erection occurring during all erectile episodes and not just during sleep. The prevalence of nocturnal painful erections is rare, occurring in fewer than one in 100 men presenting with sexual and erectile problems,2 with the overall mean age at onset for all reported cases being 40.3 Autonomic dysfunction has been implicated as a possible etiology for this rare condition,2 with beta adrenergic hyperactivity during sleep, which may explain the transient efficacy of propranolol in prior reported cases. It is intriguing that the current case had painful erections despite taking propranolol. The only effective drugs over the long term appear to be clozapine, baclofen, and clonazepam.4 Agents suppressing endogenous testosterone production can decrease painful erections but are not used because of their effect on sexual desire4 and because low testosterone can result in diminished muscle strength and bone mineral density, which may subsequently result in greater rates of falls and fractures.5 Other drugs that reduce SRPE and REM sleep with long-term efficacy, such as clozapine and monoamine oxidase inhibitors, are often avoided because of potential side effects such as myocarditis, agranulocytosis, and epileptic seizures.4 Benzodiazepines have shown promising effects with their REM-suppressing properties and need further investigation.4 This is the first reported case of SRPE in an older adult. This man was symptomatic despite taking propranolol and was successfully treated with clonazepam. This case illustrates that this rare condition of unclear etiology may present at any age. It is important to report such cases to estimate the true prevalence of SRPEs and further clarify diagnostic and treatment options. Nitesh D. Kuhadiya, MD, MPH Akshata Desai, MBBS Division of Endocrinology, Diabetes and Metabolism, University of Buffalo (UB), Buffalo, New York Michelle Reisner, MD Department of Geriatrics, Mount Sinai (Jersey City) Medical Center, Jersey City, New Jersey
ACKNOWLEDGMENTS Conflict of Interest: Nitesh D. Kuhadiya has received funding from the Endocrine Fellows Foundation to investigate the effects of liraglutide in type 1 diabetes mellitus. Author Contributions: Kuhadiya, Reisner: study concept and design, acquisition of subject and data, analysis
FEBRUARY 2014–VOL. 62, NO. 2
temporal lobe memory system.1 This type of memory makes evolutionary sense. For example, one does not need to be stung repeatedly by wasps before one finally remembers to avoid them; a single exposure is forever sufficient. Although emotional and declarative memories appear to follow different paths, it is not clear whether decrements in memory function also follow separate paths in memory disease. Herein is presented a case of a woman with vascular dementia in whom emotional memory was relatively spared.
CASE PRESENTATION An 80-year-old woman had a history of progressive vascular dementia, type II diabetes mellitus, and congestive heart failure (CHF). Her dementia limited her overall function, and she was unable to perform instrumental activities of daily living. She received donepezil 10 mg/d, memantine 10 mg twice daily, isosorbide dinitrate extended release 40 mg twice daily, glipizide extended release 20 mg/d, nifedipine extended release 10 mg twice daily, and nitroglycerine 0.4 mg sublingual, the last as needed for angina pectoris. One night, she experienced a hypnopompic hallucination of a man standing at the foot of her bed. The event was frightening to her, and she reported it with the belief that it had happened. There was no other evidence of psychosis, and she was not treated with any antipsychotic drugs. She continued to recall the event spontaneously over the subsequent 14 months until her death of CHF, although she was unable to recall other important events that occurred in her life at approximately the same time (e.g., the death of a dear friend), and she continued to have difficulty with all short-term memories (e.g., losing items, forgetting appointments, inability to manage funds).
DISCUSSION This woman with vascular dementia, demonstrated by decline in function and inability to form new declarative memories, was able to recall a single frightful event for longer than a year, as demonstrated by her spontaneous verbalization of the memory. Although the event she recalled was not real, but the result of a hypnopompic hallucination, it was experienced and recalled as real. Hypnopompic hallucinations occur in 10% to 20% of individuals with normal cognition and are not considered a psychotic process.8 There is some evidence that the amygdala may be relatively spared in aging and dementing processes.9 The amygdala appears to play an important role in the consolidation of memory of emotionally arousing events, especially fear.3,5 Emotional arousal and encoding of emotional memories that are mediated through the amygdala may bypass these dysfunctional pathways. It has been suggested that the amygdala responds rapidly and before awareness to emotional stimuli in the environment. This may lead to facilitation of attention and the overall increased vigilance observed in the presence of an emotional stimulus.3 It also means that intact cortical function may be of less importance for memory in the setting of emotional stimuli. This phenomenon may underlie the relative sparing of emotional memory in Alzheimer’s disease and related disorders.
LETTERS TO THE EDITOR
405
There have been only a small number of cases reported in which emotional memory is preserved in the setting of memory disturbance,10 and this process may be underrecognized. Nonetheless, such cases demonstrate the differences between creation of emotional memories and declarative memories. Thripura Thirtala, MD Kanwaldeep Kaur, MD Yonglin Gao, MD Steven Lippmann, MD Rif S. El-Mallakh, MD Mood Disorders Research Program Department of Psychiatry and Behavioral Sciences School of Medicine University of Louisville Louisville Kentucky
ACKNOWLEDGMENTS Conflict of Interest: Dr. El-Mallakh is on the speakers’ bureau for Astraz Zeneca and Otsuka. None of the other authors have any conflicts of interest to report. Author Contributions: All authors contributed to all aspects of this paper. Sponsor’s Role: None.
REFERENCES 1. Dolcos F, LeBar KS, Cabeza R. Remembering one year later: Role of the amygdala and the medial temporal lobe memory system in retrieving emotional memories. Proc Natl Acad Sci USA 2005;102:2626–2631. 2. Cahill L, Babinsky R, Markowitsch HJ et al. The amygdala and emotional memory. Nature 1995;377:295–296. 3. Amaral DG, Behniea H, Kelly JL. Topographic organization of projections from the amygdala to the visual cortex in the macaque monkey. Neuroscience 2003;118:1099–1120. 4. Mori E, Ikeda M, Hirono N et al. Amygdalar volume and emotional memory in Alzheimer’s disease. Am J Psychiatry 1999;156:216–222. 5. McGaugh JL. Memory reconsolidation hypothesis revived but restrained: Theoretical comment on Biedenkapp and Rudy. Behav Neurosci 2004;118:1140–1142. 6. McGaugh JL, Cahill L, Roozendaal B. Involvement of the amygdala in memory storage: Interaction with other brain systems. Proc Natl Acad Sci USA 1996;93:13508–13514. 7. Cordero MI, Venero C, Kruyt ND et al. Prior exposure to a single stress session facilitates subsequent contextual fear conditioning in rats: Evidence for a role of corticosterone. Horm Behav 2003;44:338–345. 8. Ohayon MM, Priest RG, Caulet M et al. Hypnagogic and hypnopompic hallucinations: Pathological phenomena. Br J Psychiatry 1996;169: 459–467. 9. Scheibe S, Carstensen LL. Emotional aging: Recent findings and future trends. J Gerontol B Psychol Sci Soc Sci 2010;65B:135–144. 10. Okada A, Matsuo J. Emotional memory in patients with Alzheimer’s disease: A report of two cases. Case Rep Psychiatry 2012;2012:313906.
SPLENIC MARGINAL ZONE LYMPHOMA INVOLVING THE BREAST: LESSONS IN PATIENT-CENTERED CARE To the Editor: Splenic marginal zone lymphoma (SMZL) is a rare, indolent non-Hodgkin’s lymphoma that commonly involves the spleen, bone marrow, and blood. This letter describes a 78-year-old woman with long-standing SMZL presenting with a rare case of breast involvement. Treatment options are not well researched in older adults who have limited life expectancy because of multiple comorbidities. The complex decision-making process included
406
LETTERS TO THE EDITOR
exploring the woman’s life circumstances and behaviors. The only therapeutic option available was radiation therapy, which would not reduce mortality but might reduce disease-specific morbidity. Quality of life may be compromised because of side effects from radiation therapy. This case describes additional challenges of decision-making in the context of cognitive impairment.
CASE REPORT A 78-year-old woman presented to the clinic with a new left breast mass that she had noticed incidentally while bathing. She denied a change in the size of the mass, pain, or nipple discharge. Her past medical history included type 2 diabetes mellitus (glycosylated hemoglobin 7.7 mmol/L), hyperlipidemia, hypertension, vitamin D deficiency, dementia of the Alzheimer’s type (Functional Assessment Staging scale 5), and interstitial lung disease. She had had three recent hospital admissions for dyspnea in relation to her interstitial lung disease. She also had a history of splenic marginal zone lymphoma stage IV A, treated 8 years before with splenectomy. Recurrent disease presenting with anemia that was treated with rituximab followed this surgery 9 months later. She was independent in basic activities of daily living but needed help with instrumental activities of daily living. Physical examination was remarkable for a thin older woman sitting comfortably in the chair. There was a 3-by 3-cm firm mass in the right upper quadrant of the left breast. There were no skin changes, tenderness, redness, or palpable axillary lymph nodes. She was able to stand from the chair without help and ambulate independently. A mammogram demonstrated an ill-defined mass in the upper inner quadrant at the 11:00 position 7 cm from the nipple measuring 3.5 by 2.5 by 0.9 cm corresponding to the palpable mass. No axillary lymph nodes were noted. The biopsy results showed low-grade B-cell lymphoma consistent with marginal zone type. Computed tomography showed no other areas suspicious for lymphomatous involvement. It was not possible to perform a positron emission tomography scan due because of her high serum glucose level. In view of the localized nature of her disease, a trial of radiation therapy was recommended. She asked about her overall prognosis and her quality of life with and without treatment.
FEBRUARY 2014–VOL. 62, NO. 2
JAGS
Willebrand disease). Other features are acquired C1 esterase inhibitor deficiency and high paraprotein levels. It is uncommon to find lymphadenopathy or extralymphatic organ involvement. B symptoms and high lactic dehydrogenase levels are features of the aggressive form of this disease. In some individuals, there may be an association with hepatitis C, and in these individuals, the lymphoma may respond to antiviral treatment of the hepatitis C. The disease prognosis is fairly good, with median survival of 10 years. Online calculators were used to determine that this woman had a high risk of all-cause mortality (Lee Index: 4-year mortality 44–46%; Schonberg Index: 5-year mortality 43% aged 39–47, 9-year mortality 75% aged 69–80).3,4 The only treatment option available was radiation therapy, which might not have extended her life expectancy but might have had long-term morbidity benefit by shrinking the mass and preventing local complications. Radiation therapy could have associated undesirable side effects that might affect quality of life. In summary, this was a community-dwelling older woman with mild dementia, multiple medical problems, three recent hospitalizations for dyspnea, and a remote history of SMZL now presenting with breast involvement. This presenting problem was not likely to cause her death but in combination with her other comorbidities increased her mortality risk. It was not possible to find information describing the course of the illness in individuals who do not receive treatment. Decision-making was based on the woman’s and her family’s preferences.5,6 The process of adapting best evidence to the care of individuals is called “patient-centered decision-making.” Failure to individualize care is a type of medical error termed “contextual error.” Contextual errors are due to inattention to the individual’s context (e.g., social support, economic situation, personal beliefs, past experiences), as opposed to biomedical errors. In this case, the woman’s comorbid diseases, especially dementia and interstitial lung disease causing multiple hospitalizations; social circumstances; and personal preferences were important components in the decision-making process. Given the indolent nature of the disease and possible side effects from radiation therapy, she decided against treatment with radiation therapy. She did not keep her follow-up appointments, and repeated efforts to contact her were unsuccessful. The family and case manager indicated that she was not interested in additional interventions to treat her breast condition.
DISCUSSION Splenic marginal zone lymphoma is a rare form of nonHodgkin lymphoma, accounting for 1% to 2% of all cases.1,2 It is postulated to arise from a postgerminal center, memory B-cell of splenic type. It usually involves the spleen, bone marrow, and peripheral blood; the median age at presentation is 65. It is common for the disease to be asymptomatic. Typical presenting features include splenomegaly, fatigue, abdominal discomfort, lymphocytosis, and cytopenias. Diagnosis is based on lymphocyte morphology, immunophenotype, marrow, and splenic histology. Autoimmune phenomena are found in 10% of cases (including warm antibody autoimmune hemolysis, cold agglutinins, immune thrombocytopenia, anticardiolipin antibodies, lupus anticoagulant, and acquired von
Ariba Khan, MBBS, MPH Michael L. Malone, MD Charles Bomzer, MD Aurora Health Care, Milwaukee, Wisconsin School of Medicine and Public Health, University of Wisconsin, Milwaukee, Wisconsin
ACKNOWLEDGMENTS The authors would like to thank Kathy Strube, Aurora Health Care Librarian, for her excellent bibliographic search. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors
JAGS
FEBRUARY 2014–VOL. 62, NO. 2
LETTERS TO THE EDITOR
407
and has determined that the authors have no financial or any other kind of personal conflicts with this paper. Author Contributions: Khan: acquisition of subject, study concept, bibliographic search, preparation of manuscript. Malone: preparation and revision of manuscript. Bomzer: acquisition of subject, study concept, revision of manuscript. Sponsor’s Role: No sponsor.
further diagnostic examination including polysomnography and nocturnal penile tumescence and rigidity monitoring. He was diagnosed with SRPEs and initiated on low-dose clonazepam 0.5 mg at bedtime. After 2 weeks of treatment, with frequency of nocturnal erections reduced to approximately three per night, the dose was increased to 1 mg/d, leading to complete resolution of the erections. He remains on 1 mg with no further symptoms 1 year since diagnosis.
REFERENCES
DISCUSSION
1. Oscier D, Owen R, Johnson S. Splenic marginal zone lymphoma. Blood Rev 2005;19:39–51. 2. Thieblemont C, Davi F, Noguera ME et al. Splenic marginal zone lymphoma: Current knowledge and future direction. Oncology (Williston Park) 2012;26:194–202. 3. Yourman LC, Lee SJ, Schonberg MA et al. Prognostic indices for older adults: A systemic review. JAMA 2012;307:182–192. 4. Weiner SJ, Schwartz A, Sharma G et al. Patient-centered decision making and health care outcomes: An observational study. Ann Intern Med 2013;158:573–579. 5. ePrognosis. Estimating prognosis for elders. Available at www.ePrognosis. org Accessed April 22, 2013. 6. Patient-centered care for older adults with multiple chronic conditions: A stepwise approach from the American Geriatrics Society: American Geriatrics Society Expert Panel on the Care of Older Adults with Multimorbidity. J Am Geriatr Soc 2012;60:1957–1968.
SLEEP-RELATED PAINFUL ERECTIONS IN AN ELDERLY MAN SUCCESSFULLY TREATED USING CLONAZEPAM To the Editor: Sleep-related painful erections (SRPE) are characterized by deep penile pain that occurs during erections in the rapid eye movement (REM) stage of sleep. Since its description in 1971,1 only 33 cases have been reported in the literature albeit none in older men. We report a case of nocturnal painful erections in an elderly man successfully treated using clonazepam.
CASE A 77-year-old Caucasian man with a long-standing history of essential tremors well controlled on propranolol presented with a 2-year history of nocturnal erections with recent worsening. He complained of painful penile erections after midnight, lasting for 3 to 5 minutes, which woke him up from sleep and that urination only partially relieved. He was able to fall back asleep only to have the pain wake him again. The frequency gradually increased from two to three episodes a night to one every hour and would continue until he woke up in the morning. There was no history of other sleep disorder or parasomnia. He reported being satisfied with his sexual life with his girlfriend and had normal rigid painless erections during sexual intercourse. He had not noticed any change in the quality of his erections or ejaculations since the onset of symptoms. He denied obsession with any sexual thoughts and was not taking any testosterone supplements or sexual stimulants. He had an unremarkable urological, digital rectal, and neurological examination. Blood and urine testing revealed no uropathogenic infection. Serum prostate-specific antigen and testosterone were within normal limits. He had a disappointing consultation with the urologists. He refused
Erectile pain is seen in conditions such as Peyronie’s disease, phimosis, urethritis, and metastatic carcinoma, with painful erection occurring during all erectile episodes and not just during sleep. The prevalence of nocturnal painful erections is rare, occurring in fewer than one in 100 men presenting with sexual and erectile problems,2 with the overall mean age at onset for all reported cases being 40.3 Autonomic dysfunction has been implicated as a possible etiology for this rare condition,2 with beta adrenergic hyperactivity during sleep, which may explain the transient efficacy of propranolol in prior reported cases. It is intriguing that the current case had painful erections despite taking propranolol. The only effective drugs over the long term appear to be clozapine, baclofen, and clonazepam.4 Agents suppressing endogenous testosterone production can decrease painful erections but are not used because of their effect on sexual desire4 and because low testosterone can result in diminished muscle strength and bone mineral density, which may subsequently result in greater rates of falls and fractures.5 Other drugs that reduce SRPE and REM sleep with long-term efficacy, such as clozapine and monoamine oxidase inhibitors, are often avoided because of potential side effects such as myocarditis, agranulocytosis, and epileptic seizures.4 Benzodiazepines have shown promising effects with their REM-suppressing properties and need further investigation.4 This is the first reported case of SRPE in an older adult. This man was symptomatic despite taking propranolol and was successfully treated with clonazepam. This case illustrates that this rare condition of unclear etiology may present at any age. It is important to report such cases to estimate the true prevalence of SRPEs and further clarify diagnostic and treatment options. Nitesh D. Kuhadiya, MD, MPH Akshata Desai, MBBS Division of Endocrinology, Diabetes and Metabolism, University of Buffalo (UB), Buffalo, New York Michelle Reisner, MD Department of Geriatrics, Mount Sinai (Jersey City) Medical Center, Jersey City, New Jersey
ACKNOWLEDGMENTS Conflict of Interest: Nitesh D. Kuhadiya has received funding from the Endocrine Fellows Foundation to investigate the effects of liraglutide in type 1 diabetes mellitus. Author Contributions: Kuhadiya, Reisner: study concept and design, acquisition of subject and data, analysis
![[Splenic marginal zone lymphoma].](/assets/img/hold.png)
