0
Get Alert
Please Wait... Processing your request... Please Wait.
You must sign in to sign-up for alerts.

Please confirm that your email address is correct, so you can successfully receive this alert.

1
Clinical Case Conference   |    
Successful Multimodality Treatment of Severe Behavioral Disturbance in a Patient With Advanced Huntington’s Disease
David M. Blass, M.D.; Martin Steinberg, M.D.; Iracema Leroi, M.D.; Constantine G. Lyketsos, M.D., M.H.S.
Am J Psychiatry 2001;158:1966-1972. doi:10.1176/appi.ajp.158.12.1966

Huntington’s disease is a genetic neurodegenerative disorder with a prominent psychiatric symptom profile. Huntington’s disease follows an autosomal-dominant pattern of inheritance and has 100% penetrance. The gene responsible for the disease has been located on the short arm of chromosome 4 and has been found to be an expanded trinucleotide repeat (1). The pathophysiology of the disease includes prominent cellular degeneration in the caudate nucleus, as well as cell loss in a number of other brain regions, including the cortex and cerebellum (2).

In North America, Huntington’s disease afflicts five to eight people per 100,000. The age at onset is generally in the late 30s, and the typical disease course runs 17 years until death (3). The clinical syndrome reflects a triad of symptoms, including movement disorders, dementia, and other psychiatric problems. Movement difficulties begin with incoordination, repetitive fine finger movements, facial and limb chorea, and abnormalities of eye movements. Dystonia and athetosis eventually become superimposed on the chorea, and the chorea eventually involves the trunk as well. The gait becomes ataxic, and ultimately bradykinesia, dysarthria, dysphagia, spasticity, and urinary incontinence develop, necessitating total nursing care (4). The resulting dementia is progressive and global (5).

In some patients, psychiatric symptoms precede the onset of the movement disorder. Mood disorders, both unipolar major depression and bipolar disorder, are extremely common, and the suicide rate has been found to be significantly higher than that found in the general population (6, 7). Hallucinations and delusions also occur in frequencies above the rates found in the baseline population (8). Moreover, patients frequently develop disturbances of behavior, including aggression, hypersexuality, and paraphilia (8). Often the behavioral disturbances result in psychiatric hospitalization (9).

Treatment of the psychiatric manifestations of Huntington’s disease include pharmacological and behavioral interventions. There are few controlled studies of either (3). Here we discuss the case of a young man with advanced Huntington’s disease and severe behavioral, affective, perceptual, and cognitive disturbances who was successfully treated by using a combination of approaches within a highly structured, multidisciplinary inpatient setting. This case discussion will also illustrate the complex psychiatric symptoms that emerge in Huntington’s disease or as a result of its treatment, the use of a differential reinforcement schedule in advanced dementia, and the important role of a tertiary care center in the treatment of this kind of complicated illness. This approach to treatment can be generalized to the care of patients with complicated dementia, regardless of the etiology of the dementia. This case highlights many challenges inherent in the care of chronically ill patients with degenerative disorders and ultimately illustrates the clear benefits of applying intensive diagnostic and treatment methods, despite the impossibility of curing the underlying disorder.

Mr. A was a 32-year-old single African American man with advanced Huntington’s disease who was referred to the inpatient unit of the neuropsychiatry service of the Johns Hopkins Hospital from his nursing home for evaluation and treatment of complex behavioral disturbances. These included attempts to escape, assaults, and inappropriate touching of female staff members. Mr. A’s history was obtained from his mother and sister, the nursing home staff, previous psychiatric records, and the patient himself.

+

Family History

Mr. A’s father and three paternal aunts had all been afflicted with Huntington’s disease. His father had first developed motor symptoms in his late 30s and died at age 52. A number of Mr. A’s paternal cousins also had Huntington’s disease. His sister had developed Huntington’s disease in her early teens and died at the age of 28. Mr. A’s two older siblings had not been genetically tested for Huntington’s disease and were asymptomatic at age 34. There was no other known psychiatric or neurological family history.

+

Personal and Social History

Mr. A had been born 2 months prematurely and weighed 5 pounds. There were no known residua of the prematurity, and he had developed normally. He was described as a hyperactive child who frequently got into trouble, was known as a class clown, and engaged in theft at an early age. There was no known history of setting fires, cruelty to animals, or running away from home.

Mr. A had dropped out of school after the eighth grade, having been educated to that point in a regular classroom. He had been a poor student academically. After leaving school, he had worked for a number of years in janitorial services, until he developed symptoms of Huntington’s disease and it became impossible for him to work.

Mr. A had lived at home with his mother until 1 year before admission, when his behavioral disturbances had necessitated placement into a nursing home. He was heterosexual but had never had a long-term relationship and had no children. There was no history of smoking, alcohol abuse, or illicit drug use nor was there any history of arrest.

+

Medical History

Mr. A had had an inguinal hernia repaired at the age of 6. He had suffered from mild asthma as a child, which he apparently outgrew. He had recently tested negative for HIV. He had no known medication allergies. His medications at admission were 15 mg of flurazepam at bedtime, 2.5 mg b.i.d. of diazepam, 25 mg of doxepin at bedtime, 75 mg b.i.d. of venlafaxine, 200 mg b.i.d. of carbamazepine, 50 mg b.i.d. of quetiapine, 50 mg q.i.d. of chlorpromazine, 1 mg/day of folic acid, 0.5 mg b.i.d. of benztropine as needed, and 1 mg of lorazepam every 4 hours as needed for agitation.

+

Psychiatric and Neurological History

Mr. A had first developed motor symptoms at the age of 15; these were mild abnormal hand and trunk movements. He had been subsequently diagnosed with Huntington’s disease at the age of 20, when his unstable gait led to frequent falls and a neurological consultation. Despite his early symptoms Mr. A had continued to function responsibly at work and at home.

Psychiatric symptoms had been noted at age 24, which was 8 years before admission. According to the family, he had undergone a marked change in personality, becoming shy, withdrawn, and apathetic. He had remained in his room watching television for much of the day. He had not appeared sad nor did he express feelings of sadness. Neither Mr. A nor his family reported any change in his appetite or sleep pattern, but his energy had appeared diminished.

Five years before admission, at the age of 27, his gait instability had become so severe that Mr. A had to stop working. Concurrently, he had developed difficulty feeding himself. He had continued to live at home under his family’s care, spending the majority of his time watching television in his bedroom.

+

History of Present Illness

One year before admission, Mr. A had developed auditory hallucinations of people speaking outside his window. He had developed the delusional belief that his body was inhabited by several people, including God’s daughter, and that his food was being poisoned. He had been noted to be religiously preoccupied. He had often wandered out of the house, becoming locked out on a number of occasions. The development of command auditory hallucinations to harm his family had prompted emergency admission to a local psychiatric hospital; he had been discharged to a nursing home because it had been deemed unsafe for him to remain at home.

Over the next year multiple problematic behaviors had developed. Mr. A regularly touched female staff members and residents inappropriately. He frequently masturbated in public. He often was found wandering the halls without clothes. He had frequent falls, often resulting in injury. He usually refused to wear his prescribed protective equipment while walking (a helmet and knee and elbow pads had been prescribed to prevent injury as a consequence of his frequent falls). He also made frequent attempts to escape from the facility. When redirected, he often struck out at staff members. Of note, he was never violent or aggressive outside of demand situations. Neuroleptics (chlorpromazine and quetiapine) and benzodiazepines (flurazepam, diazepam, and lorazepam) had been gradually added to his drug regimen to help control his behavior. The doses ultimately were increased to the point at which Mr. A was nearly continuously sedated. When the doses were lowered to improve his quality of life, his problematic behaviors returned.

Mr. A had been admitted to local hospitals on a number of occasions because of these behaviors. During these admissions he was noted to have visual hallucinations in addition to the described symptoms. A computerized tomography scan of his head was read as showing the absence of caudate nuclei and mild generalized atrophy but no other abnormalities. An EEG could not be interpreted because of motion artifact. Medications prescribed in that year (aside from those listed) included haloperidol, risperidone, valproic acid, and nortriptyline. Despite these multiple medication trials and psychiatric admissions, there was no improvement in Mr. A’s condition. He was then referred to the neuropsychiatry inpatient service for further evaluation.

+

Admission Examination

Mr. A was a thin African American man who was sitting up in bed wearing a helmet. He was awake but only intermittently attentive. Eye contact was poor. There was frequent eye blinking and chin twitching. His speech was slow, quiet, dysarthric, and quite difficult to understand. His answers were generally restricted to one or two words. He stated that he was happy, although his affect appeared to be quite blunted and limited to a restricted range. He said that his energy and appetite were good, and he reported no suicidal or homicidal ideation. He acknowledged auditory hallucinations, but it was difficult to understand his report of their content. He reported no experiences of passivity or delusions. He reported no obsessions, compulsions, or phobias. On the Mini-Mental State Examination (MMSE) (10), he scored 10 of 30, including 0 of 10 for orientation, 0 of 5 for calculation, and 0 of 3 for recall. He scored 3 of 3 for registration, 2 of 2 for naming objects, 3 of 3 for following a three-step instruction, 1 point for reading, and 1 point for repeating a sentence. He lost 2 points because of his inability to write a sentence or copy interlocking pentagons.

On physical examination, his vital signs were found to be normal. He appeared adequately nourished, and his dentition was good. He had marked rigidity in all four extremities. Choreiform movements were present in his trunk and proximal extremities. Occasional hemiballismus was noted. He had poor upward gaze with his right eye and poor lateral and upward gaze with his left eye, but his eye movements were smooth. He was unable to suppress blinks, and his initiation of eye movements was delayed. He was able to protrude his tongue, but other voluntary facial movements were limited. Cranial nerves VII, XI, and XII were otherwise intact, as was his hearing. His strength was rated 4 of 5 and was symmetrical in all four extremities. The results of a sensory examination were normal. His deep tendon reflexes were rated 2+ and symmetrical in the upper extremities and 3+ and symmetrical in the lower extremities. His plantar reflexes were flexor bilaterally. He could stand and walk only with assistance. The remainder of the physical examination was unremarkable.

+

Initial Formulation

The initial formulation focused on the fact that Mr. A had advanced Huntington’s disease and a broad array of psychiatric symptoms. The latter were felt to reflect a number of distinct but interrelated syndromes. These included disturbances of behavior (aggression, unwanted touching of others, and wandering), as well as psychosis with auditory and visual hallucinations and persecutory delusions. His cognition was impaired globally, and he also had an impaired ability to sustain a consistent level of attention. There was no evidence of affective disturbance initially. His history and the results of his mental status examination showed clear evidence of delirium, most likely as a result of polypharmacy in a patient vulnerable to delirium due to advanced Huntington’s disease. Although delirium could partially account for his behavioral disturbances, psychotic symptoms, and cognitive impairment, his history reflected the presence of all of these symptoms before exposure to polypharmacy. Given the chronicity of his motor symptoms, he almost certainly had dementia as a result of Huntington’s disease. His disturbed behavior and psychotic symptoms may also have been a primary consequence of brain damage due to Huntington’s disease. We entertained the possibility that he had a psychotic affective syndrome, something that would not have been unusual in a patient with Huntington’s disease (6).

+

Initial Treatment Plan

The initial goal was to remove as many medications as possible from Mr. A’s regimen in order to clarify his differential diagnosis and to reach a conclusive formulation of his illness before developing a long-term treatment plan. A workup to rule out any medical complications that might have been exacerbating his condition was undertaken.

+

Test Results

At the time of admission, a series of laboratory tests were conducted, including a CBC with differential, an electrolyte panel, tests of liver function, a urinalysis, and measures of levels of thyroid-stimulating hormone, vitamin B12, and folate; the results were found to be normal. His rapid plasma reagin was nonreactive. His trough carbamazepine level was 5.3 mg/liter. An ECG was unremarkable. Brain magnetic resonance imaging revealed small putaminal and caudate nuclei, as well as minimal diffuse cerebral volume loss.

Mr. A’s 6:00 a.m. serum testosterone level was 566 ng/dl (normal range=286–1510); an HIV antibody test was negative. A serum sample was sent for confirmation of the diagnosis of Huntington’s disease (results of which were only available after discharge but confirmed the presence of the expanded trinucleotide repeat, at 65 copies).

+

Hospital Course

Over the initial 5 hospital days, chlorpromazine, quetiapine, doxepin, venlafaxine, and benztropine were tapered completely without complications or discontinuation symptoms. Diazepam and flurazepam were switched to 1 mg t.i.d. of clonazepam, which was reduced to 0.5 mg t.i.d. by hospital day 11. Carbamazepine was also tapered and discontinued by hospital day 11. All of the tapers occurred concurrently.

During the first week in the hospital, Mr. A remained extremely sedated, spending most of the day in bed or in a reclining chair with a restraining tray (to prevent falls); his only activity was eating and occasional masturbation. He was frequently incontinent of urine, and his sleep did not follow a regular pattern. There were no behavioral disturbances during this time. These symptoms were all felt to be consistent with delirium.

With the reduction in polypharmacy, Mr. A’s delirium cleared gradually over time, and several problematic behaviors reemerged. There were multiple episodes of grabbing female staff members, public masturbation, and attempts to escape. Mr. A often walked without his protective equipment and, when redirected, would often refuse or attempt to strike the redirecting staff member. As more days passed, psychomotor agitation, rapid, slurred speech, and jogging in the hallway became prominent symptoms. Mr. A became suspicious that his food was poisoned and greatly reduced his caloric intake. He frequently refused to take his medications. Of note, he became completely alert and continent. His sleep pattern returned to normal.

+

Revised Formulation

At this point we felt that Mr. A’s delirium had disappeared and that he was in a manic episode. We postulated that his behavioral disturbances were now being exacerbated by the mania, although according to his history, they had clearly preceded the onset of psychomotor agitation, pressured speech, paranoia, or generalized hyperactivity, all of which were now present. There was no evidence of any complicating medical problems, and he did not have an elevated serum testosterone level (which might have explained his hypersexuality). He clearly had advanced dementia, which could now be appreciated as distinct from his delirium, as evidenced by his continued memory difficulties in the absence of a fluctuating level of consciousness.

+

Later Hospital Course

There was now sufficient information to formulate a comprehensive multidisciplinary treatment plan. We initiated antimanic pharmacotherapy with carbamazepine and thioridazine, watching carefully for the development of oversedation or delirium. Doses were ultimately increased to 200 mg of carbamazepine in the morning and 300 mg at bedtime and 20 mg t.i.d. of thioridazine. Treatment with medroxyprogesterone acetate was initiated to help reduce Mr. A’s sexual drive and hypersexual behavior, as well as to reduce his aggression.

+

Choice of Medications

Formulation of his medication regimen was guided by a thorough review of previous medication trials, a desire to minimize side effects, and the sparse literature available to specifically guide our decisions. Thioridazine was chosen for its sedating properties, given Mr. A’s manic symptoms and his lack of benefit from quetiapine, risperidone, haloperidol, and chlorpromazine. These considerations were balanced against the acknowledged risk of delirium with thioridazine; certainly this agent would not have been a first-line choice, particularly in a patient with a complex neurological condition. Clonazepam was chosen because of its intermediate-length half-life and our desire to avoid agents with either a very short or very long half-life, such as alprazolam and diazepam, respectively. Carbamazepine was selected as the mood stabilizer because of concern about Mr. A’s ability to tolerate lithium, especially with his fluctuating food and fluid intake. Moreover, the available medical records indicated that Mr. A had been treated with valproic acid with unclear benefit. The use of medroxyprogesterone to reduce his sexual drive was supported by early data regarding antiandrogen therapy in the treatment of inappropriate sexual behavior in patients with other forms of dementia (11), although we found no specific data regarding such therapy for patients with Huntington’s disease.

Within a number of days of initiating these medications, there was a significant reduction in Mr. A’s symptoms of paranoia, pressured speech, and psychomotor agitation. However, his problematic behaviors continued essentially unchanged. Consultations from physical and occupational therapy services were obtained regarding the use of protective equipment and gait-assisting devices and the development of bathing and eating protocols to maximize independence, minimize injuries, and standardize Mr. A’s daily care.

At this point, the focus of treatment turned toward behavior management, through the use of applied behavioral analysis (12). A consultation was obtained from the behavioral psychology division of the Kennedy Krieger Institute. We were advised to initiate a period of detailed data collection for behavioral analysis. The goal was to identify the precise context and time course of Mr. A’s behavioral disturbances, the salient reinforcers of this behavior, time spent in various activities, as well as the usual staff reactions and their effects. Detailed information was obtained from the inpatient nursing staff, the nursing home staff, and Mr. A’s family, in addition to numerous hours of direct observation of Mr. A by the behavioral psychology team. An activity monitoring system was implemented to document his activities every 15 minutes, including the specific times and contexts of any of the problematic behaviors.

After 5 days of data collection, the following preliminary observations were made: Mr. A’s problematic behaviors occurred most often in demand situations, such as when he was asked to take medication or to interrupt a pleasant activity, such as walking or watching television. The touching of female staff members tended to occur during bathing or dressing. There was no clear pattern to his public masturbation. His preferred activities were identified as watching television, eating snack food, and walking.

A complex behavior modification plan was devised and implemented. The first component was the development of a consistent and highly structured daily schedule for Mr. A. This schedule was placed in the line of sight from his bed and reviewed with him regularly. Activities he preferred would be written into the schedule as frequently as possible, and less preferred activities (e.g., taking medications) would always be followed by preferred activities. Transitions from one activity or task to another would be preceded by notifications from the staff ahead of time. The goal was to minimize surprise or change and to create a situation in which compliance with required activities led to consistent rewards.

The second aspect of the behavioral plan was the development of a differential reinforcement schedule, that is, a structured system to ensure that desired behaviors were reinforced and undesired behaviors were not inadvertently reinforced. Initially, reinforcement was to be provided at 1-hour intervals only if Mr. A adhered to a few clear rules:

1. No aggressive behavior toward staff or other patients.

2. No inappropriate touching of staff or other patients.

3. Medications must be taken as prescribed.

4. Walking must be performed with protective equipment (helmet, shoes, and knee and elbow pads) in place.

5. A break from walking should be taken if he was so tired that he started falling.

6. Masturbation must occur only in his room.

Basic principles of behavior modification were made explicit to the inpatient staff as part of the behavior plan. For example, each reinforcement was to be accompanied by an explanation of why Mr. A was receiving it, as well as generous verbal praise. Mr. A was to be given the option of choosing which reward he wanted (e.g., candy versus chips versus soda), and, even if he did not make a choice, one should be provided anyway. If he did not earn the reward, then a brief explanation of why he was not getting it should be provided. Inappropriate behavior was to be "blocked," that is, interrupted with a brief reprimand but with as little attention as possible given to the behavior. Opportunities for success were to be maximized.

Demand situations were to be preceded by a small reinforcer and an explanation of what was about to occur and of what was expected from the patient. If Mr. A successfully complied with the demand, more reinforcement and praise should be given. If he refused the request, standard unit rules were to be implemented to deal with the situation.

Finally, it was recommended that a staff member be individually assigned at predetermined times during the day (on Mr. A’s schedule) to walk with him. This was one of his preferred activities during the day and the one that frequently generated conflict as a result of the staff’s concerns about his safety and his desire to walk ad lib.

The behavior plan in its entirety was documented in Mr. A’s chart in a typed note and reviewed in detail with the entire staff working directly with Mr. A. The detailed monitoring of his behavior continued so that the success of the plan could be assessed and changes made when necessary.

Five days after the implementation of the behavior modification plan (which was 2 weeks after the initiation of antimanic medications), Mr. A’s behavior improved dramatically. He began to initiate the wearing of his protective gear, and when he failed to do so and was reminded, he generally complied by putting the gear on. He also became completely compliant with taking medications. Initially, it was necessary on a few occasions to have security officers present at the time of these requests, but the need for this intervention was short-lived. His aggressive behavior was completely extinguished, and Mr. A also was able to comply with restricting masturbation to his room. The touching of female staff members diminished significantly with the initial plan but was not extinguished, and some adjustments to the plan were made. As noted, Mr. A had been given medroxyprogesterone acetate to help reduce his sexual drive somewhat. The reinforcement interval was spaced gradually to every 3–4 hours.

Within 2 weeks of initiating the behavior plan, Mr. A was prepared for discharge to the nursing home facility. The nursing home staff visited the inpatient unit on several occasions to be trained in the implementation of the behavior plan. Mr. A was discharged to the nursing home after a 32-day hospital stay. At discharge he was walking well and had only one or two falls per day. His medication regimen at discharge was 200 mg of carbamazepine in the morning and 300 mg at bedtime (trough level=7.5 mg/liter), 20 mg t.i.d. of thioridazine, 0.5 mg t.i.d. of clonazepam, 5 mg/day of medroxyprogesterone acetate, 1 mg/day of folic acid, and a daily multivitamin.

+

Follow-Up

Mr. A did well at the nursing home for a number of months; there was continued successful implementation of the behavioral plan. Approximately 4 months after discharge, his condition worsened again, without any obvious precipitant. Mr. A made frequent attempts to escape, exhibited public masturbation and nudity, walked without protective gear, and had violent outbursts when redirected by staff members. Haloperidol, 5 mg bi.d., was substituted for thioridazine by the nursing home physician, and Mr. A received 1–2 mg/day of lorazepam, both orally and intramuscularly. He was referred for readmission to our unit.

+

Readmission Examination

Mr. A was awake and minimally interactive and appeared confused. He was unable to sit unassisted without falling over. His eye contact was minimal. His answers were quiet, slow, and generally monosyllabic; there was significant latency. He stated that his mood was "okay," but his affect appeared quite constricted. He reported no suicidal or homicidal ideation. It was difficult to assess hallucinations or delusions. His MMSE score was 0 of 30. The results of a neurological examination were not appreciably different from the one documented previously.

As before, routine laboratory tests included a CBC, an electrolyte panel, tests of liver function, a urinalysis, a measure of thyroid-stimulating hormone level, and a chest radiograph; all results were normal. His trough serum carbamazepine concentration was 7.8 mg/liter. His serum testosterone level at 7:00 a.m. was 388 ng/dl.

+

Initial Formulation

The initial impression was again one of delirium, likely the result of polypharmacy; the high dose of lorazepam was the most proximal agent. The most likely scenario was that Mr. A had had a breakthrough of manic symptoms leading to a worsening of behavior and increased medication use, a deterioration in the implementation of the behavior plan over time leading to a reemergence of his symptoms, or a combination of these two possibilities.

+

Hospital Course

Mr. A was readmitted to the neuropsychiatry service inpatient unit with the drug regimen previously described. His clonazepam dose was tapered to 0.25 mg at bedtime. His medroxyprogesterone acetate dose was increased to 10 mg/day given that his testosterone level was still within the normal range. The behavior modification plan previously formulated was reinstituted. When it became apparent that Mr. A was experiencing auditory hallucinations, his haloperidol dose was increased to 7.5 mg b.i.d.

As during his first hospitalization, Mr. A’s behavior improved significantly with strict implementation of the behavior plan, with the exception of ongoing attempts to touch female staff members during direct care. Once the behavior plan was in place, his aggression, public masturbation, attempts to escape, and walking without protective gear stopped. The inpatient unit staff again worked with the nursing home staff to improve implementation of the behavior plan in the nursing home environment. Mr. A was discharged to the nursing home after a 13-day hospitalization. His discharge medications included 200 mg of carbamazepine in the morning and 300 mg at bedtime, 7.5 mg b.i.d. of haloperidol, 0.25 mg of clonazepam at bedtime, 10 mg/day of medroxyprogesterone acetate, 1 mg/day of folic acid, and a daily multivitamin.

This case conference illustrates a number of important points about the natural history of Huntington’s disease and the clinical management of patients with advanced dementia in general. First, this case illustrates how the complex psychiatric phenomena in patients with advanced dementia can interact and indirectly exacerbate each other. This patient developed highly disruptive behaviors, either as a result of brain disease, mania (which probably also resulted from his brain disease), or both. To combat these behavioral symptoms, he was prescribed increasing doses of psychotropic medications even as his condition deteriorated. Ultimately, these medications produced a new syndrome, delirium, to which he was particularly vulnerable given his advanced dementia. The delirium then exacerbated his existing symptoms and probably generated new ones, such as visual hallucinations, a worsened gait, and an increased risk of falls and subsequent injury. Once a course of pharmacological treatment was embarked on, it was difficult to reverse course and taper medications until Mr. A arrived in the secure environment of the inpatient unit. The significant morbidity caused by polypharmacy is one component of the general risk of pharmacological treatment, which also includes the well-documented risk of medication errors (13).

Both pharmacological and behavioral approaches were used with this patient, neither of which could have succeeded without the implementation of the other. The behavioral interventions would have been of limited use while the patient was delirious or manic. The initial 2 weeks of hospitalization were used to taper medications and allow the symptoms of the underlying disorder to emerge so that an accurate and comprehensive formulation could be made and a more targeted medication regimen could be employed. Once the delirium had cleared and the mania emerged and began to be treated, a comprehensive behavioral analysis could be undertaken, something that could not have been done as effectively before that point. We think it unlikely that medications alone would have managed the patient’s behaviors without the comprehensive behavior plan. The patient had already been treated with an impressive pharmacopoeia without resolution of his behavioral symptom profile, except when he was completely sedated. In his case, the behavioral symptoms were probably not simply related to mania and may have been caused directly by brain damage from Huntington’s disease.

The response of a patient with advanced dementia to a differential reinforcement schedule is notable. With the increasing number of patients surviving until they have late-stage dementia in our country, strategies for treating the very common behavioral disturbances associated with advanced dementia need to be as comprehensive and the least restrictive possible (12). Further research into behavioral treatment strategies for patients with advanced Huntington’s disease as well as other dementias is needed.

Another critical but easily overlooked element in the success of this treatment was the close communication between the tertiary center and the referring facility throughout every stage of evaluation and treatment. This interaction took place with all professional groups involved: physicians, psychologists, nurses, social workers, and administrators. Without the time spent obtaining detailed clinical information from the nursing home staff about the patient’s behavior pattern, training the nursing home staff in person in the proper implementation of the behavior plan, and providing clear documentation of the details of the behavior plan, the long-term success of the comprehensive plan would have been doubtful.

A final point relates to use of resources in the care of patients with progressive and irreversible conditions. This patient spent a total of 43 days on a specialty inpatient psychiatric unit in a tertiary care center, certainly a growing rarity as concerns over cost containment shorten inpatient lengths of stay. However, in the 1 year before his admission to Johns Hopkins Hospital, the patient had three briefer hospitalizations in the community, all of which were of fewer than 10 days, as well as multiple consultations and emergency department visits with comprehensive workups and diagnostic studies of changes in mental status. The nursing home staff were routinely exposed to the risk of injury due to his aggressive behavior. Worst of all, the patient spent several months sedated and confined to his bed, often physically restrained and unable to have any meaningful interaction with his environment. The investment of time and resources available only in a tertiary center are well spent in ameliorating any of these problems and are cost-effective. The willingness to make these investments affirms our profession’s commitment to all patients as full members of the human community, irrespective of their ability to contribute materially.

This patient with advanced Huntington’s disease demonstrates the variety and complexity of psychiatric symptoms that can develop in this condition and the challenges that arise in the course of treatment. Polypharmacy and its complications were demonstrated, as well as the need for an often lengthy inpatient stay to untangle the symptoms. Synchronizing pharmacotherapy and behavior modification therapy proved very effective for this patient. The use of close communication between the inpatient and referring facility was also seen. This case also illustrates the great value, both in humanitarian and economic terms, inherent in investing time and resources in a comprehensive diagnosis and aggressive treatment of these easily overlooked but very important members of our society.

Received April 4, 2001; revision received Aug. 21, 2001; accepted Aug. 29, 2001. From the Neuropsychiatry Service, Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine. Address reprint requests to Dr. Blass, Department of Psychiatry and Behavioral Sciences, Meyer 279, Johns Hopkins Hospital, 600 North Wolfe St., Baltimore, MD 21287; dmblass@yahoo.com (e-mail). The authors thank Drs. Malka G. Blass, Paul R. McHugh, and Peter V. Rabins for their comments on this article.

Huntington’s Disease Collaborative Research Group: A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell  1993; 72:971-983
[PubMed]
[CrossRef]
 
de la Monte SM, Vonsattel JP, Richardson EP: Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington’s disease. J Neuropathol Exp Neurol  1988; 47:516-525
[PubMed]
[CrossRef]
 
Leroi I, Michalon M: Treatment of the psychiatric manifestations of Huntington’s disease: a review of the literature. Can J Psychiatry  1998; 43:933-940
[PubMed]
 
Young AB, Shoulson I, Penney JB, Starosta-Rubinstein S, Gomez F, Travers H, Ramos-Arroyo MA, Snodgrass SR, Bonilla E, Moreno H, Wexler NS: Huntington’s disease in Venezuela: neurological features and functional decline. Neurology  1986; 36:244-249
[PubMed]
 
McHugh PR, Folstein MF: Psychiatric syndromes of Huntington’s chorea: a clinical and phenomenologic study, in Psychiatric Aspects of Neurologic Disease. Edited by Benson DDF, Blumer D. New York, Grune & Stratton, 1975, pp 267-286
 
Morris M: Psychiatric aspects of Huntington’s disease, in Huntington’s Disease. Edited by Harper PS. Philadelphia, WB Saunders, 1991, pp 81-126
 
Schoenfeld M, Myers RH, Cupples LA, Berkman B, Sax DS, Clark E: Increased rate of suicide among patients with Huntington’s disease. J Neurol Neurosurg Psychiatry  1984; 47:1283-1287
[PubMed]
[CrossRef]
 
Mendez MF: Huntington’s disease: update and review of neuropsychiatric aspects. Int J Psychiatry Med  1994; 24:189-208
[PubMed]
[CrossRef]
 
Dewhurst K, Oliver JE, McKnight AL: Socio-psychiatric consequences of Huntington’s disease. Br J Psychiatry  1970; 116:255-258
[PubMed]
[CrossRef]
 
Folstein MF, Folstein SE, McHugh PR: "Mini-Mental State": a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res  1975; 12:189-198
[PubMed]
[CrossRef]
 
Haddad PM, Benbow SM: Sexual problems associated with dementia, part 2: aetiology, assessment and treatment. Int J Geriatr Psychiatry  1993; 8:631-637
[CrossRef]
 
Lundervold DA, Jackson T: Use of applied behavior analysis in treating nursing home residents. Hosp Community Psychiatry  1992; 43:171-173
[PubMed]
 
Kohn LT, Corrigan JM, Donaldson MS (eds): To Err Is Human: Building a Safer Health System. Washington, DC, National Academy Press, 1999
 
+

References

Huntington’s Disease Collaborative Research Group: A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell  1993; 72:971-983
[PubMed]
[CrossRef]
 
de la Monte SM, Vonsattel JP, Richardson EP: Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington’s disease. J Neuropathol Exp Neurol  1988; 47:516-525
[PubMed]
[CrossRef]
 
Leroi I, Michalon M: Treatment of the psychiatric manifestations of Huntington’s disease: a review of the literature. Can J Psychiatry  1998; 43:933-940
[PubMed]
 
Young AB, Shoulson I, Penney JB, Starosta-Rubinstein S, Gomez F, Travers H, Ramos-Arroyo MA, Snodgrass SR, Bonilla E, Moreno H, Wexler NS: Huntington’s disease in Venezuela: neurological features and functional decline. Neurology  1986; 36:244-249
[PubMed]
 
McHugh PR, Folstein MF: Psychiatric syndromes of Huntington’s chorea: a clinical and phenomenologic study, in Psychiatric Aspects of Neurologic Disease. Edited by Benson DDF, Blumer D. New York, Grune & Stratton, 1975, pp 267-286
 
Morris M: Psychiatric aspects of Huntington’s disease, in Huntington’s Disease. Edited by Harper PS. Philadelphia, WB Saunders, 1991, pp 81-126
 
Schoenfeld M, Myers RH, Cupples LA, Berkman B, Sax DS, Clark E: Increased rate of suicide among patients with Huntington’s disease. J Neurol Neurosurg Psychiatry  1984; 47:1283-1287
[PubMed]
[CrossRef]
 
Mendez MF: Huntington’s disease: update and review of neuropsychiatric aspects. Int J Psychiatry Med  1994; 24:189-208
[PubMed]
[CrossRef]
 
Dewhurst K, Oliver JE, McKnight AL: Socio-psychiatric consequences of Huntington’s disease. Br J Psychiatry  1970; 116:255-258
[PubMed]
[CrossRef]
 
Folstein MF, Folstein SE, McHugh PR: "Mini-Mental State": a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res  1975; 12:189-198
[PubMed]
[CrossRef]
 
Haddad PM, Benbow SM: Sexual problems associated with dementia, part 2: aetiology, assessment and treatment. Int J Geriatr Psychiatry  1993; 8:631-637
[CrossRef]
 
Lundervold DA, Jackson T: Use of applied behavior analysis in treating nursing home residents. Hosp Community Psychiatry  1992; 43:171-173
[PubMed]
 
Kohn LT, Corrigan JM, Donaldson MS (eds): To Err Is Human: Building a Safer Health System. Washington, DC, National Academy Press, 1999
 
+
+

CME Activity

There is currently no quiz available for this resource. Please click here to go to the CME page to find another.
Submit a Comments
Please read the other comments before you post yours. Contributors must reveal any conflict of interest.
Comments are moderated and will appear on the site at the discertion of APA editorial staff.

* = Required Field
(if multiple authors, separate names by comma)
Example: John Doe



Web of Science® Times Cited: 15

Related Content
Books
Dulcan's Textbook of Child and Adolescent Psychiatry > Chapter 9.  >
The American Psychiatric Publishing Textbook of Psychopharmacology, 4th Edition > Chapter 37.  >
The American Psychiatric Publishing Textbook of Psychopharmacology, 4th Edition > Chapter 54.  >
APA Practice Guidelines > Chapter 0.  >
The American Psychiatric Publishing Textbook of Substance Abuse Treatment, 4th Edition > Chapter 9.  >
Topic Collections
Psychiatric News
APA Guidelines