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Kleine-Levin syndrome: A neuropsychological case report
Abstracts from the 19th Annual Meeting
647
lective Reminding Test. Specifically, greater than 40 % of both genders displayed impairments on this measure's Total Recall, Long Term Retrieval, and Consistent Long Term Retrieval tasks. Similar mean MMPI-2 clinical profiles were also exhibited by both male and female patients. Their profiles indicated that they were experiencing an array of symptomatology ranging from somatic complaints, to lethargy and fatigue, and that they may have been functioning at a reduced level of efficiency. These findings are discussed in light of patients' end-stage pulmonary disease and factors possibly contributing to their neuropsychological test performances. Implications for clinical practice and future research are also provided.
Tucker, K. A., & Gouvier, W. D. Neuropsychological Decline Following Herpes Zoster Without Encephalitis: A Case Study. Global anterograde memory deficits and structural changes within the medial temporal lobes have been documented following cases of herpes simplex encephalitis. However, little is known about possible neuropsychological sequelae following herpes infection that does not progress into encephalitis, and in particular, cognitive changes associated with herpes zoster rash. This poster presents the neuropsychological test results of a 45-year-old immunocompetent patient with a history of herpes zoster rash on the right side of his head in the dermatome affecting the trigeminal nerve. The rash drew near, but did not enter his right eye or cause ocular complications. The patient experienced approximately 2 weeks of rash followed by 2 months of postherpetic neuralgia, which were treated with Zovirax, Neurontin, and Tegretol. Testing occurred approximately 3 months after rash onset. A clinically significant discrepancy was apparent between verbal and visual intellectual abilities that favored the former (WAIS-R Verbal IQ = 104, Performance IQ = 85). A comparison of the corresponding deviation quotients with an estimate of premorbid intellectual functioning was suggestive of a decline in visuospatial functions in the presence of relatively preserved verbal abilities (WAIS-R Verbal Comprehension = 111, Perceptual Organization = 77, Barona estimated Full Scale IQ = 116). Immediate memory/learning was intact; however, a greater than expected decay in memory was observed following a 30-minute delay (General Memory Index = 119, Verbal Memory Index = 113, Visual Memory Index = 119, Delayed Recall Index = 98). The results of the present report were consistent with prior case studies of patients with herpes zoster, in which the side of rash was ipsilateral to the hemisphere with relative neuropsychological dysfunction. This case study adds another instance of support to the observation that right-sided rash, regardless of dermatome affected, is generally associated with declines in visuospatial intellectual abilities and/or visual memory. The current results were significant also in that they suggested significant diminution of neuropsychological functions may occur in the absence of encephalitis in an immunocompetent patient with herpes zoster. Extended antiviral therapy may be warranted to attempt to reduce the possibility of neuropsychological sequelae. Muilin, J. P., & Podeli, K. Kleine-Levin Syndrome: A Neuropsychological Case Report. A neuropsychologieal case report is presented of a patient with probable Kleine-Levin syndrome. This rare and etiologically unclear syndrome often occurs subsequent to a viral infection or mild head injury. It is characterized by hypersomnia and megaphagia accompanied by behavioral changes, including hypersexuality, exhibitionism, depression, hallucinations, delusions, memory deficits, and disorientation. The symptomatic periods
648
Abstracts from the 19th Annual Meeting
are typically abrupt in onset, with symptoms lasting several days or weeks with spontaneous recovery. The patient is a 29-year-old right-handed high school educated male with history of mild head injury (at age 22) and no psychiatric or substance abuse history. He first presented to his family physician with a high-grade fever accompanied by sinusitis. After the fever decreased, the patient demonstrated hypersomnia of up to 20 hours a day, accompanied by lethargy, disorientation, and memory disturbance. During a subsequent neurological exam, the patient demonstrated significant hypersexual behavior toward the physician and was subsequently hospitalized on a neurology unit. Throughout his hospitalization, he demonstrated increasing sexual disinhibition including inappropriate sexual advances toward staff and visitors, in addition to sexually oriented delusions and paranoia regarding the health care personnel. A complete neurological workup including brain MRI, spinal tap, blood labs, and E E G was negative, and no viral etiology was identified. A subsequent brain SPECT revealed a single focus of increased radiotracer activity in the anterior right temporal lobe. A limited inpatient neuropsychological examination was completed, revealing poor novel problem-solving and working memory skills, but intact anterograde memory. Upon discharge, he continued to demonstrate sexual aggression, poor personal hygiene, and excessive food intake. He was subsequently admitted to an inpatient psychiatric unit and treated successfully with a variety of psychotropic medications. However, methylphenidate proved highly effective. A complete neuropsychological evaluation was conducted 3 months later. The patient presented without evidence of hypersexuality, hyperphagia, hypersomnia, or delusional symptoms and demonstrated almost no recall of the aforementioned symptoms or events. The neuropsychological evaluation revealed subtle, mild deficits in sustained attention/concentration and executive control. The case is discussed in light of recent hypotheses on the origins of Kleine-Levin syndrome, as well as possible brain systems involved and use of stimulants for treatment.
Raymond, M. J. Neurobehavioral Sequelae of lmrnunotherapy Anaphylaxis. Anaphylaxis is an immediate hypersensitivity or reaction to the immune system following sensitization of a foreign substance (e.g., drug). This reaction is the most pronounced form of hypersensitivity and if not treated immediately, may result in death. Anaphylaxis often manifests with a variety of clinical symptoms, which may progress rapidly, and include hypertension and airway obstruction. Less severe symptoms may include urticaria, angioneurotic edema, and gastrointestinal distress, including abdominal cramping, nausea, and vomiting. This case study describes the neuropsychological test results and behavioral sequelae of a 24-year-old, right-handed female who developed anaphylaxis following allergen immunotherapy. Specifically, she was administered an allergen that was 1,000 times the concentration of her prescribed allergen dose. Initial symptoms included shortness of breath, abdominal cramping with associated nausea and bowel and bladder incontinence, and urticaria. Symptoms persisted despite treatment with epinephrine. This resulted in a 1-week acute hospitalization resulting in numerous cognitive and emotional alterations. Postdischarge, she complained of reduced attention/concentration, distractibility, depression, frustration, oversuspiciousness, and generalized deconditioning. Subsequently, she was referred for neuropsychological evaluation at 4 months postonset. For the most part, neuropsychological test results were well within the normal range and consistent with premorbid expectations. However, she presented with an agitated depression and evidence of posttraumatic stress. Test results were correlated with negative neurodiagnostics (MRI). This case study underscores the importance of correlative data in the development of diagnostic impressions and treatment recommendations.
647
lective Reminding Test. Specifically, greater than 40 % of both genders displayed impairments on this measure's Total Recall, Long Term Retrieval, and Consistent Long Term Retrieval tasks. Similar mean MMPI-2 clinical profiles were also exhibited by both male and female patients. Their profiles indicated that they were experiencing an array of symptomatology ranging from somatic complaints, to lethargy and fatigue, and that they may have been functioning at a reduced level of efficiency. These findings are discussed in light of patients' end-stage pulmonary disease and factors possibly contributing to their neuropsychological test performances. Implications for clinical practice and future research are also provided.
Tucker, K. A., & Gouvier, W. D. Neuropsychological Decline Following Herpes Zoster Without Encephalitis: A Case Study. Global anterograde memory deficits and structural changes within the medial temporal lobes have been documented following cases of herpes simplex encephalitis. However, little is known about possible neuropsychological sequelae following herpes infection that does not progress into encephalitis, and in particular, cognitive changes associated with herpes zoster rash. This poster presents the neuropsychological test results of a 45-year-old immunocompetent patient with a history of herpes zoster rash on the right side of his head in the dermatome affecting the trigeminal nerve. The rash drew near, but did not enter his right eye or cause ocular complications. The patient experienced approximately 2 weeks of rash followed by 2 months of postherpetic neuralgia, which were treated with Zovirax, Neurontin, and Tegretol. Testing occurred approximately 3 months after rash onset. A clinically significant discrepancy was apparent between verbal and visual intellectual abilities that favored the former (WAIS-R Verbal IQ = 104, Performance IQ = 85). A comparison of the corresponding deviation quotients with an estimate of premorbid intellectual functioning was suggestive of a decline in visuospatial functions in the presence of relatively preserved verbal abilities (WAIS-R Verbal Comprehension = 111, Perceptual Organization = 77, Barona estimated Full Scale IQ = 116). Immediate memory/learning was intact; however, a greater than expected decay in memory was observed following a 30-minute delay (General Memory Index = 119, Verbal Memory Index = 113, Visual Memory Index = 119, Delayed Recall Index = 98). The results of the present report were consistent with prior case studies of patients with herpes zoster, in which the side of rash was ipsilateral to the hemisphere with relative neuropsychological dysfunction. This case study adds another instance of support to the observation that right-sided rash, regardless of dermatome affected, is generally associated with declines in visuospatial intellectual abilities and/or visual memory. The current results were significant also in that they suggested significant diminution of neuropsychological functions may occur in the absence of encephalitis in an immunocompetent patient with herpes zoster. Extended antiviral therapy may be warranted to attempt to reduce the possibility of neuropsychological sequelae. Muilin, J. P., & Podeli, K. Kleine-Levin Syndrome: A Neuropsychological Case Report. A neuropsychologieal case report is presented of a patient with probable Kleine-Levin syndrome. This rare and etiologically unclear syndrome often occurs subsequent to a viral infection or mild head injury. It is characterized by hypersomnia and megaphagia accompanied by behavioral changes, including hypersexuality, exhibitionism, depression, hallucinations, delusions, memory deficits, and disorientation. The symptomatic periods
648
Abstracts from the 19th Annual Meeting
are typically abrupt in onset, with symptoms lasting several days or weeks with spontaneous recovery. The patient is a 29-year-old right-handed high school educated male with history of mild head injury (at age 22) and no psychiatric or substance abuse history. He first presented to his family physician with a high-grade fever accompanied by sinusitis. After the fever decreased, the patient demonstrated hypersomnia of up to 20 hours a day, accompanied by lethargy, disorientation, and memory disturbance. During a subsequent neurological exam, the patient demonstrated significant hypersexual behavior toward the physician and was subsequently hospitalized on a neurology unit. Throughout his hospitalization, he demonstrated increasing sexual disinhibition including inappropriate sexual advances toward staff and visitors, in addition to sexually oriented delusions and paranoia regarding the health care personnel. A complete neurological workup including brain MRI, spinal tap, blood labs, and E E G was negative, and no viral etiology was identified. A subsequent brain SPECT revealed a single focus of increased radiotracer activity in the anterior right temporal lobe. A limited inpatient neuropsychological examination was completed, revealing poor novel problem-solving and working memory skills, but intact anterograde memory. Upon discharge, he continued to demonstrate sexual aggression, poor personal hygiene, and excessive food intake. He was subsequently admitted to an inpatient psychiatric unit and treated successfully with a variety of psychotropic medications. However, methylphenidate proved highly effective. A complete neuropsychological evaluation was conducted 3 months later. The patient presented without evidence of hypersexuality, hyperphagia, hypersomnia, or delusional symptoms and demonstrated almost no recall of the aforementioned symptoms or events. The neuropsychological evaluation revealed subtle, mild deficits in sustained attention/concentration and executive control. The case is discussed in light of recent hypotheses on the origins of Kleine-Levin syndrome, as well as possible brain systems involved and use of stimulants for treatment.
Raymond, M. J. Neurobehavioral Sequelae of lmrnunotherapy Anaphylaxis. Anaphylaxis is an immediate hypersensitivity or reaction to the immune system following sensitization of a foreign substance (e.g., drug). This reaction is the most pronounced form of hypersensitivity and if not treated immediately, may result in death. Anaphylaxis often manifests with a variety of clinical symptoms, which may progress rapidly, and include hypertension and airway obstruction. Less severe symptoms may include urticaria, angioneurotic edema, and gastrointestinal distress, including abdominal cramping, nausea, and vomiting. This case study describes the neuropsychological test results and behavioral sequelae of a 24-year-old, right-handed female who developed anaphylaxis following allergen immunotherapy. Specifically, she was administered an allergen that was 1,000 times the concentration of her prescribed allergen dose. Initial symptoms included shortness of breath, abdominal cramping with associated nausea and bowel and bladder incontinence, and urticaria. Symptoms persisted despite treatment with epinephrine. This resulted in a 1-week acute hospitalization resulting in numerous cognitive and emotional alterations. Postdischarge, she complained of reduced attention/concentration, distractibility, depression, frustration, oversuspiciousness, and generalized deconditioning. Subsequently, she was referred for neuropsychological evaluation at 4 months postonset. For the most part, neuropsychological test results were well within the normal range and consistent with premorbid expectations. However, she presented with an agitated depression and evidence of posttraumatic stress. Test results were correlated with negative neurodiagnostics (MRI). This case study underscores the importance of correlative data in the development of diagnostic impressions and treatment recommendations.