1320 LETTERS TO THE EDITOR Jacob Menczel, MD Department of Geriatrics Hebrew University Hadassah Medical School Jerusalem, Israel Institute of Osteoporosis Herzog Hospital Jerusalem, Israel ACKNOWLEDGMENTS We would like to thank Dr. Sheva Mann for her help in editing the letter, Dr. Lisa Deutsch, for statistical analysis, and Professor Josef Wisman for the vitamin D determinations. Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflict with this letter. Author Contributions: All of the authors took part in every aspect of this letter including data analysis and manuscript preparation. Sponsor’s Role: No sponsors. REFRENCES 1. Broe KE, Chen TC, Weinberg J et al. A higher dose of vitamin D reduces the risk of falls in nursing home residents: A randomized multiple-dose study. J Am Geriatr Soc 2007;55:234–239. 2. Teno JM, Mor V, DeSilva D et al. Use of feeding tubes in nursing home residents with severe cognitive impairment. JAMA 2002;287:3211–3212. 3. Norton D. Calculating the risk: Reflections on the Norton scale. Decubitus 1989;2:24–31. 4. Teasdale G, Jennett B. Assessment of coma and impaired consciousness: Practical scales. Lancet 1974;2:81–84. 5. Abbasi AA, Rudman D. Observation on the prevalence of protein-calorie undernutrition in VA nursing home. J Am Geriatr Soc 1993;41:117–121. 6. Roux C, Bischoff-Ferrari HA, Papapoulos SE et al. New insights into the role of vitamin D and calcium in osteoporosis management. An expert roundtable discussion. CMRO 2008;24:1363–1370. DISPARITIES IN LATE-LIFE PLANNING AND TREATMENT To the Editor: Shayna et al.1 report the important finding that, when entering nursing homes, black residents or their significant others were less likely to have discussed treatment restrictions with healthcare providers than were whites. What is more, disparity in treatment restrictions narrowed when discussions occurred. One crucial bit of data is not in their report. Nearly one-third of eligible patients were not enrolled in the study, apparently because they did not consent. The racial breakdown of this group is important, because the study’s questions depend in part on willingness to discuss treatment restrictions. Enrolled subjects were older and more often female than eligible nonparticipators, and black subjects were significantly younger and more often male than white subjects. If more eligible blacks than whites declined to be studied, then the finding that more black subjects than white wanted additional information from physicians might be undermined. JULY 2009–VOL. 57, NO. 7 JAGS Regardless, the authors teach us that, regardless of race, many family members want more information from physicians in considering these tragic choices. Thomas Finucane, MD Johns Hopkins Bayview Medical Center Baltimore, MD ACKNOWLEDGMENTS Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the author and has determined that the author has no financial or any other kind of personal conflicts with this letter. Author Contributions: The author is the sole contributor to this letter. Sponsor’s Role: None. REFERENCE 1. Shayna RE, Ann GL, Charlene QC et al. Discussion as a factor in racial disparity in advance directive completion at nursing home admission. J Am Geriatr Soc 2008;57:146–152. ANOSOGNOSIA FOR RIGHT HEMIPLEGIA FROM DOMINANT ANTERIOR CEREBRAL ARTERY STROKE To the Editor: Anosognosia for hemiplegia (AHP), neglect of weakness after an acute brain lesion, is a phenomenon most commonly described in nondominant-hemisphere strokes,1,2 and when occurring in dominant hemisphere infarcts it has not been characterized according to specific arterial territorial infarction. We describe a unique righthanded patient presenting with anosognosia for right hemiparesis found to have a dominant anterior cerebral artery (ACA) infarct. A 90-year-old truly right-handed woman with hypertension, prior transient ischemic attacks, and recent pneumonia presented with sudden right-sided weakness. Despite being unable to stand, she was unaware of weakness and was concerned about her cough. She was alert and oriented and had no language disturbance. She was profoundly unaware of limb weakness but lacked visual or hemispatial neglect, easily recognizing her paretic side. Despite significant right hemiparesis (1–2 of 5), she insisted that she was moving her right limbs as usual when confronted directly. She had no cranial nerve or sensory deficits or ataxia and did not extinguish double simultaneous stimuli. Deep tendon reflexes were 11, with a right Babinski sign. Intravenous tissue plasminogen activator was administered 2 hours after symptom onset. Magnetic resonance imaging revealed an acute infarct in the left ACA territory (Figure 1). Carotid ultrasound estimated bilateral 50% to 79% stenoses. She was started on aspirin and was discharged with improved hemiparesis but remained unaware of her weakness. Two months later, she was readmitted for congestive heart failure and atrial fibrillation and remained mildly hemiparetic. Although she did not complain JAGS JULY 2009–VOL. 57, NO. 7 LETTERS TO THE EDITOR 1321 Figure 1. Axial diffusion-weighted imaging revealed restricted diffusion in the left frontal parasagittal region (A through E) consistent with an acute infarct in the left anterior cerebral artery territory. of weakness, she was quick to report right weakness when asked if she felt strong or weak. She was aware of her initial hemiparesis and subsequent improvement. COMMENT The incidence of AHP in stroke ranges from 7% to 73%,2 attributed to the different criteria applied. Some detect anosognosia based on spontaneous answers to questioning, whereas others use a scale that includes explicit questioning about right- and left-sided strength, incorporating the patient’s response after a demonstration of weakness.3 Detection methods with greater sensitivity include implicit measurements, like selection of unimanual versus bimanual tasks4 or identifying a discrepancy between clinician’s and patient’s assessment of weakness.5 Most studies of AHP from stroke have emphasized a much higher incidence with right (7–68%) than left (5– 32%) hemisphere lesions.6 One study found severe AHP only in patients with right hemisphere lesions,7 but anosognosia in left-hemispheric-lesion patients may be underestimated because of concomitant aphasia. Of studies examining for anosognosia in left-hemisphere stroke, one series detected AHP in only 6% of patients.6 Studies employing more-sensitive, implicit detection methods reported anosognosia in 24% to 30% of patients with left hemisphere stroke during rehabilitation.5,8 These studies highlight a further contrast: right-hemispheric patients almost always have associated hemispatial neglect8,9 whereas left-hemispheric patients rarely do.6 Nevertheless, left-hemispheric patients had poorer recovery than patients with similar weakness without anosognosia.8 Beyond hemispheric localization, several studies have attempted to document the responsible anatomic lesions. Ischemia to motor programming areas in the right hemisphere may be responsible.7,9 Fewer studies have described the localization of AHP in patients with left hemisphere lesions. Most left-hemispheric patients with AHP had small subcortical rather than large cortical lesions.8 Whether this reflects a different mechanism, or that larger cortical lesions typically have aphasia that obscures AHP, is uncertain. None of these studies have described left-hemisphere AHP in terms of a discrete vascular territory. To our knowledge, this is the first AHP patient described with a dominant ACA infarct. The possibility that this patient may be right handed with an atypical pattern of hemisphere dominance seems unlikely, based on the rare incidence of crossed-dominance in strongly right-handed individuals. Our patient’s anosognosia was severe on a standardized rating scale (maximal score: 3); over 2 months, AHP improved to mild (score: 1).3 Typical of left-hemi- spheric AHP, this patient had no hemispatial neglect. Because language areas were spared, no aphasia manifested. We postulate that, in this patient, left ACA infarction damaged dorsomedial frontal premotor areas, disrupting a sensory-motor integration network that maintains limb function awareness. This network functions as a monitoring system, comparing motor intent (feedforward) with action (feedback).1 This network may function analogously to language and vision working memory systems that also depend on frontal lobe circuitry for awareness.10 This system allows limb function representation to be translated into longer-term awareness and memory. The improved awareness of hemiparesis displayed 2 months after her stroke suggests functional recovery of the proposed sensory-motor monitoring system. Her recovered recollection of earlier severe hemiparesis demonstrates that longer-term awareness of past weakness has the capacity to be updated as AHP resolves. Matthew S. Robbins, MD Shira Markowitz, MD Mariam Saleeb, MD Michael L. Swerdlow, MD Peter C. Mabie, MD The Saul R. Korey Department of Neurology Albert Einstein College of Medicine Bronx, NY ACKNOWLEDGMENTS Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this letter. Author Contributions: Drs. Robbins, Markowitz, Saleeb, Swerdlow, and Mabie were involved in identifying subject, designing report, interpretation of data, and preparation of manuscript. Sponsor’s Role: None. REFERENCES 1. Heilman KM, Barrett AM, Adair AC. Possible mechanisms of anosognosia: A defect in self-awareness. Philos Trans R Soc Lond B Biol Sci 1998;353:1903– 1909. 2. Orfei MD, Robinson RG, Prigatano GP et al. Anosognosia for hemiplegia after stroke is a multifaceted phenomenon: A systematic review of the literature. Brain 2007;130:3075–3090. 3. Bisiach E, Vallar G, Perani D et al. Unawareness of disease following lesions of the right hemisphere: Anosognosia for hemiplegia and anosognosia for hemianopia. Neuropsychologia 1986;24:471–482. 1322 LETTERS TO THE EDITOR 4. Ramachandran VS. Anosognosia in parietal lobe syndrome. Conscious Cogn 1995;4:22–51. 5. Hartman-Maeir A, Soroker N, Oman SD et al. Awareness of disabilities in stroke rehabilitation: A clinical trial. Disabil Rehabil 2003;25:35–44. 6. Beis JM, Keller C, Morin N et al. Right spatial neglect after left hemisphere stroke. Neurology 2004;63:1600–1605. 7. Karnath HO, Baier B, Nagele T. Awareness of the functioning of one’s own limbs mediated by the insular cortex? J Neurosci 2005;25:7134–7138. 8. Hartman-Maeir A, Soroker N, Katz N. Anosognosia for hemiparesis in stroke rehabilitation. Neurorehabil Neural Repair 2001;15:213–222. 9. Berti A, Bottini G, Gandola M et al. Shared cortical anatomy for motor awareness and motor control. Science 2005;309:488–491. 10. Linden DE. The working memory networks of the human brain. Neuroscientist 2007;13:257–267. SNEDDON-WILKINSON DISEASE: A CASE REPORT OF A RARE DISEASE IN A NONAGENARIAN To the Editor: We report the case of a 93-year-old woman admitted to the Medical Division for oral ulcers, bilateral conjunctivitis, and cutaneous bullae. The patient had a history of congestive heart failure due to valvular disease, hypertension, permanent atrial fibrillation, chronic renal insufficiency, and atrophic gastritis and of iatrogenic hypothyroidism due to chronic amiodarone therapy. Two days before she was evaluated, the patient had developed white lesions in the mouth, followed by eruption of skin vesicles and bullae. The white lesions on the buccal and tongue mucosa became ulcerated and painful, and the other skin lesions were occasionally painful and pruritic. On examination at admission, there were white, slightly oval plaques on the oral mucosa. Tense bullae, containing clear to slightly cloudy fluid, were located on the dorsum of all toes, extending slightly into the interdigital region of the feet and on the anterior surface of the legs. The same lesions were present bilaterally in the submammary area. Several lesions were confluent. There were no nail changes or scarring of the skin. Nicolsky’s sign (ready removal of epidermis with slight tangential pressure) was negative. The patient was apyrexial. Chest and abdominal examination were normal. Ears, nose, and throat examination and laryngoscopy were normal. Peripheral blood cell counts showed normal values, erythrocyte sedimentation rate and C-reactive protein were mildly elevated (30 mm/h and 15.6 mg/dL, respectively), and moderate chronic renal insufficiency (creatinine 2.4 mg/ dL and urea 82 mg/dL) and moderate hyperuricemia (11.0 mg/dL) were present. Serum electrophoresis, immunoglobulin dosage (immunoglobulin (Ig)A, IgG, and IgM), and complement factors (C3 and C4) were normal. Vitamin blood levels of were in the normal range, except for a significant deficiency of vitamin B6 (2.4 mg/L, normal value 3.6–18.0). Chest X-ray, total-body computed axial tomography, gastric fibroscopy, and breast X-ray did not show malignancies. Biopsy of a skin lesion for pathological examination was performed 3 days after admission. During the first week of her hospital stay, the skin lesions evolved, showing polycyclic erythematous and crusty lesions with a pustulous border on the legs, trunk, breast, and face. There were resolving bullae with minimal overlying erythema and slight hemorrhagic crust on the legs. Buccal and tongue lesions became necrotic and painful, interfering significantly with food and drink intake, with a JULY 2009–VOL. 57, NO. 7 JAGS subsequent visible deterioration in clinical condition and physical performance. Skin histological findings led to the diagnosis of subcorneal pustular dermatosis, also known as Sneddon-Wilkinson disease (SWD), a benign and rare disorder characterized by a chronic, relapsing vesiculopustular eruption of unknown etiology that mainly affects patients aged 30 to 70.1,2 Generally, SWD is associated with pyoderma gangrenosum3 and benign monoclonal IgA gammopathy.4 Other anecdotally associated conditions include inflammatory and neoplastic diseases.5 Dapsone is the treatment of choice. Systemic and topical corticosteroids are generally ineffective, but may provide some control.6 The patient was started on oral prednisolone (25 mg/d), and a few days later, the oral, ocular and submammary lesions resolved completely. Skin lesions showed a hyperkeratotic evolution without eruption of new bullae or vesicles. The corticosteroid therapy was gradually reduced until it was suspended after 10 days. Two weeks later, facial, oral, and submammary lesions relapsed, so oral corticosteroids were started again. Complete remission of the dermatitis was achieved after 6 weeks of treatment. This is the first case reported of SWD in a nonagenarian. With the increasing survival of elderly patients, rare diseases will be more frequent in older subjects and characterized by atypical presentation. Even in a 93-year-old patient, a complete diagnostic procedure is necessary, not with the aim of clarifying the pathogenetic mechanism but to select the most appropriate treatments (increasing efficacy and reducing side effects). For this purpose, geriatricians may need to collaborate with other specialists. In this case, the skin biopsy (rarely performed in a nonagenarian) was important to exclude zoster or fungal infections, two conditions in which the use of corticosteroids is contraindicated, whereas it is first-line treatment for the majority of cutaneous bullous dermatitis in elderly people (e.g., pemphigo, pemphigoides). Oral prednisone treatment was selected because of the unavailability in Italy of dapsone. In geriatric medicine, once the risk of relevant side effects have been excluded, drugs with even a low probability of efficacy can be prescribed, and the high variability of biological conditions may allow unexpected clinical results to be obtain. Piera Ranieri, MD Angelo Bianchetti, MD Department of Internal Medicine Istituto Clinico S. Anna Brescia, Italy Geriatric Research Group Brescia, Italy Marco Trabucchi, MD Geriatric Research Group Brescia, Italy ACKNOWLEDGMENTS Conflict of Interest: The editor in chief has reviewed the conflict of interest checklist provided by the authors and has determined that the authors have no financial or any other kind of personal conflicts with this letter.