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  1. Home
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  3. Case Studies in Dementia
  • Cited by 1
Publisher:
Cambridge University Press
Online publication date:
May 2011
Print publication year:
2011
Online ISBN:
9780511997433
DOI:
https://doi.org/10.1017/CBO9780511997433
Subjects:
Psychiatry and Clinical Psychology, Neurology and Clinical Neuroscience, Medicine, Psychiatry
Series:
Case Studies in Neurology
Information

Book description

Dementia is amongst the greatest challenges facing the medical profession as the population ages. Accurate diagnosis is essential as many rarer forms of the disease are treatable if recognized early. This collection of case studies from around the world illustrates both common and unusual causes of dementia, emphasizing clinical reasoning, integrative thinking and problem-solving skills. Each case consists of a clinical history, examination findings and special investigations, followed by diagnosis and discussion. The aim is to reinforce diagnostic skills through careful analysis of individual presenting patterns, and to guide treatment decisions, using state-of-the-art diagnostic classification and tools. The reader will be able to distinguish patients who need reassurance, closer follow-up or immediate referral to specialized services. Written and edited by internationally recognized experts in dementia, these case studies will inform and challenge clinicians at all stages of their careers.

Reviews

' … accessible and insightful, and easily digested …'

Source: ACNR Journal

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Contents

Page 2 of 2

Contents
  • Case 23 - Thiamine for dementia in a young woman
    pp 172-179
  • View abstract

    Summary

    This chapter talks about a 50-year-old man who was referred for dementia evaluation a few months after losing his job as head of a department in a large company. An MRI scan was initially described with white matter lesions in the temporal and parietal lobes, but no focal atrophy. A revised report also described cortical atrophy of the frontal lobes. The diagnosis of the Frontotemporal Dementia (FTD) was given based on the dominant clinical symptoms that had developed gradually over a couple of years: apathy, disinhibition, loss of social conduct, impaired empathy, stereotypic behavior, neglect of self-care, altered eating pattern, and impaired insight. Frontotemporal Lobar Degeneration (FTLD) is used here as the general label for neurodegenerative diseases involving primarily the anterior regions of the brain. The patient presented here is a prototypical example of the behavioral variant of FTLD, exhibiting most of the characteristic features of this clinical subtype.
  • Case 24 - Dizziness in someone with HIV
    pp 180-187
  • View abstract

    Summary

    This chapter talks about a 62-year-old right-handed lady who consulted her family doctor when she noticed that she was unable to use her knitting needle properly with her left hand. The provisional diagnosis was Corticobasal Syndrome (CBS) based on the combination of subtle asymmetric extrapyramidal findings and prominent apraxia. CBS is an akinetic-rigid clinical syndrome characterized by lateralized motor and cognitive signs, such as rigidity, dystonia, spontaneous or stimulus-sensitive myoclonus, apraxia, alien limb phenomenon, cortical sensory loss, and aphasia. This clinical syndrome has been associated with many pathological entities other than CBD, including Progressive Supranuclear Palsy (PSP), Alzheimer's disease, Creutzfeldt-Jakob disease, and many diseases in the spectrum of Frontotemporal Lobar Degeneration (FTLD): Pick's disease, Frontotemporal Dementia (FTD) with ubiquitin-positive inclusions, FTD due to mutations of MAPT and PGRN. Ante-mortem diagnosis can be difficult because proposed criteria lack sensitivity and specificity.
  • Case 25 - Case of young man smoking pot but with a mother who died of dementia
    pp 188-192
  • View abstract

    Summary

    This chapter talks about Mary who was admitted to Tertiary Hospital in early June of 2003 having stumbled to the ground being unable to get up. CT of her head confirmed general parenchymal volume loss with decreased attenuation periventricularly. No significant areas of ischemia or space-occupying lesions were noted. PET scan was suggestive of a neurodegenerative process of Alzheimer's type. No scan evidence of frontal lobe dementia. The diagnosis prior to the PET scan being performed was frontotemporal dementia. The final clinical diagnosis after the PET scan and on discharge was that of Alzheimer's type dementia. She was not started on acetylcholine esterase therapy. She was discharged to a low level residential facility. This case is interesting in that it does not on face value fit into one category of neurodegenerative disorders. False beliefs related to misinterpretation of the environment due to significant visual impairment is known as Charles-Bonnet syndrome.
  • Case 26 - University educated man with childish behavior
    pp 193-200
  • View abstract

    Summary

    This chapter talks about a 69-year-old man who developed a Kluver-Bucy syndrome (KBS), pseudobulbar affect, depression, and psychosis after multiple cerebral ischemic lesions. Detailed neurological and cognitive evaluations were conducted beginning at age 71. The initial diagnostic impression was that this patient developed a complex and severe neuropsychiatric symptomatology associated with a dementia syndrome secondary to vascular disease, although the presence of a neurodegenerative disease could not be ruled out. This patient presented with a significant neuropsychiatric symptomatology after several episodes of strokes. However, the severity of the vascular disease detected by MRI did not explain these symptoms, which initially led to the assumption that a cortical neurodegenerative disorder was also present. The most interesting aspect of this patient was that he had persistent behavioral symptomatology in the context of a stable or improving cognitive syndrome.
  • Case 27 - Frontotemporal lobar dementia vs. solvent intoxication: A piece that does not fit the puzzle
    pp 201-207
  • View abstract

    Summary

    This chapter talks about a 75-year-old retired sailor who was in very good overall physical condition. At the first assessment, the sailor reported a gradual cognitive decline, subjectively described as memory impairment, during the last 2 years. The neurologist diagnosed mild right-sided Parkinsonism in upper body, with tremor and bradykinesia, but no rigor. Walking was near normal, but he was unsteady on turning and had a pathological pull-test. This case presented with asymmetric Parkinsonism, and cognitive impairment was noted at the time of diagnosis. Thus, the important differential diagnoses were possible: dementia associated with Lewy Bodies (DLB) or Dementia associated with Parkinson's disease (PDD). DLB is characterized by dementia accompanied by idiopathic Parkinsonism, cognitive fluctuation, and persistent visual hallucinations. PDD and DLB have similar clinical presentation as well as underlying brain changes, and differentiation between the two syndromes is entirely based on the relative timing of Parkinsonism and cognitive impairment.
  • Case 28 - Man with difficulty keeping pace
    pp 208-214
  • View abstract

    Summary

    This chapter talks about a 47-year-old Caucasian man who was referred due to memory problems and depression following surgical treatment of aortic dissection 1 year earlier. After surgery, he was unable to return to work and began receiving disability benefits. Recent laboratory tests included normal complete blood count and chemistry laboratory examinations. Neuropsychological evaluation included a neurobehavioral status examination, face-to-face neurocognitive testing, and computer-administered measures. This patient illustrates the clinical manifestations of hypoperfusive dementia and he fulfilled NINDS-AIREN diagnostic criteria for vascular dementia including: presence of dementia; evidence of stroke; and a temporal relation between the first two conditions. Despite his relatively young age, and the absence of extracranial and intracranial Cerebrovascular Disease (CVD), he had watershed infarction as a result of relentless hypotension following aortic dissection. Heavy smoking could have impaired the effective maintenance of cerebral blood flow (CBF).
  • Case 29 - Young woman with abnormal movements but no family history
    pp 215-221
  • View abstract

    Summary

    This chapter talks about a 41-year-old man who was referred to memory clinic because of memory loss, poor verbal expression, loss of interest, and blunted response to environment. Cognitive status was evaluated by the mini-mental state examination (MMSE) and a detailed neuropsychological battery for assessment of memory, language and executive function, attention, and concentration. The examination documented moderate dementia with moderate amnesia and dysexecutive syndrome. Neuropsychological tests showed evidence of cognitive impairment of multiple domains, including memory, executive function, and attention and speed of information processing. He received 3-week penicillin therapy at an Infectious Diseases Hospital. During the follow-up check-up 3 months after penicillin therapy, the serum Rapid Plasma Reagin (RPR) was negative. Performance of verbal fluency and color trail making test was improved dramatically, suggesting executive function was restored after anti-syphilitic therapy. The follow-up magnetic resonance imaging (MRI) scan was unaltered compared with the initial visit.
  • Case 30 - Elderly lady with right-sided visual hallucinations
    pp 222-228
  • View abstract

    Summary

    This chapter talks about a 39-year-old woman who was reported to the "German National Reference Center for the Surveillance of Transmissible Spongiform Encephalopathies" (NRC). The patient's medical history comprised allergic asthma, hyperlipidemia, nasal sinus surgery, and pulmonary embolism. The family's medical history comprised allergies, asthma, and psoriasis, cerebrovascular disease, colon cancer, but no early onset dementia. The combination of rapidly progressive dementia, psychosyndrome, ataxia, visual disturbances, and the Pulvinar Sign revealed by MRI as well as the exclusion of encephalitis or many other possible causes of that symptom constellation made the involved physicians consider a prion disease, namely variant Creutzfeldt-Jakob disease, as a differential diagnosis. Rapidly progressive dementia, the "Hockey Sticks" and the exclusion of encephalitis led to the diagnosis of variant Creutzfeldt-Jakob disease (vCJD) in the first place. Wernicke's disease was not as strongly considered initially since the patient was non-alcoholic.
  • Case 31 - Woman with gait impairment and difficulty reading
    pp 229-237
  • View abstract

    Summary

    This chapter presents the case of a 57-year-old right-handed man with a 1-year history of poor balance, increasing forgetfulness, and difficulties following conversations. There is a family history of malignancy in two first-degree relatives. Neurological examination reveals saccadic pursuit eye movements but no nystagmus, impaired fine finger movements, depressed ankle jerks, and impaired vibration and temperature sensation in both distal lower limbs. HIV associated neurocognitive disorder (HAND) varies in severity from the asymptomatically neurocognitively impaired to those with dementia. Risk factors for the development of HIV dementia (HAD) include: persistently high viral load, low nadir CD4 cell count, increasing age and female sex, low body mass index, anaemia, and intravenous drug abuse. HAND varies in severity from the asymptomatically neurocognitively impaired to those with dementia. The CNS damage underlying HAND occurs secondarily to the direct toxic action of virus-produced proteins and as a consequence of immune activation.
  • Case 32 - Man with problems with reading and calculating
    pp 238-244
  • View abstract

    Summary

    This chapter talks about a 30-year-old man who was referred in 2001 to a neurologist because of memory complaints affecting his ability to perform at work as a cashier in a liquor store. His latest assessment in 01/2010 showed some dysarthria, dragging of his left leg during ambulation, mild face bradykinesia, hyperreflexia of all four limbs, equivocal left plantar response, unsustained clonus of both ankles, and decreased foot tapping bilaterally. There was much hesitation in diagnosing a progressive neurodegenerative condition until 8 years into the symptoms, because of the use of marijuana and the dependant personality. A follow-up is proposed to offer the patient a trial of a cholinesterase inhibitor, considering the finding of a reduced cortical choline-acetyl-transferase (CAT) activity in his mother's autopsy. The objective findings on neuro-imaging allow him to obtain full medical disability for a well-defined neurological condition.
  • Case 33 - Man with gait impairment and urine incontinence
    pp 245-252
  • View abstract

    Summary

    This chapter talks about a 39-year-old man, a human resource executive, who was seen at the outpatient clinic of the Behavioral and Cognitive Neurology Unit of the Hospital das Clı´nicas, a public university hospital in Sao Paulo, Brazil. Physical examination showed a well-nourished man, with a blood pressure of 130/80 mmHg, and neurologic examination revealed only dysarthria, with normal and reactive pupils and normal reflexes. His insight about his problems seemed to be normal. The initial diagnostic impression was a Frontotemporal Dementia (FTD), but the final diagnosis was of paretic dementia, a neuropsychiatric condition caused by neurosyphilis. He was treated with intravenous aqueous crystalline penicillin G, 4 million units every 4 hours for 21 days, and with fluoxetine. FTD or behavioral subtype of fronto-temporal lobar degeneration was the initial diagnosis in this case because the clinical picture was dominated and confirmed the presence of frontal or fronto-temporal involvement.
  • Case 34 - Falling before forgetting
    pp 253-258
  • View abstract

    Summary

    This chapter talks about a 43-year-old male who was referred to a Memory Clinic by a psychiatrist to evaluate the possibility of Frontotemporal Lobar Dementia. Neuropsychological evaluation revealed a general cognitive functioning within normal limits, but weaknesses in some executive functions such as abstraction, attention/concentration, and working memory. The patient was taken out of his job environment. On the follow-up visit 11 months later, he felt improved significantly, but still complained of lack of energy and attention difficulties. When he tried returning to his previous professional duties doing mechanics and paint, he experienced readily mild acute intoxication symptoms. This is probably caused by hypersensitivity to chemicals associated with chronic solvent intoxication. Solvents are widely used in many industrial processes in our society. Since these agents are used to dissolve fatty substances, they easily penetrate cell membranes and they cross the blood-brain barrier.
  • Case 35 - Man who stopped drinking
    pp 259-264
  • View abstract

    Summary

    This chapter presents a case study of a 60-year-old man who had complained of stumbling for 2 years without dizziness or stiffness of the legs. There is an impression of mild dementia on neuro-cognitive examination with obvious slowness of mental processing. Neurological examination of the upper body and head is unremarkable. His feet are high-arched, and power in the flexor and extensor muscles of the feet is diminished to grade MRC 4 on the left side and grade MRC 3 on the right side with an inverse position of the right foot. Taking the information from history, neurological, and neuro-cognitive examination together, the chapter concludes that the old man suffers from cognitive impairments in combination with a bi-pyramidal syndrome with poly-neuropathy and urine incontinence. It appears that the cognitive impairment leads to a presenile form of dementia, characterized not only by memory deficits but also by mental slowness and dysfunction.
  • Case 36 - Woman with dementia and hepatic disease
    pp 265-271
  • View abstract

    Summary

    This chapter talks about a 45-year-old right-handed woman who was admitted to a Neurology clinic with several years of cognitive difficulty. She has a long-standing history of depression, which has been fairly well-controlled on sertraline 100mg daily. There are no other chronic medical problems or regular medications. CT of the head demonstrated very mild generalized atrophy with more pronounced atrophy of the head of the caudate nucleus. The initial diagnostic impression was Huntington disease, despite the lack of a clear family history. Genetic testing was performed, which demonstrated an expansion of the CAG repeat region in the huntingtin gene on chromosome 4. Additionally, a more thorough family history revealed that her father and several of his relatives had involuntary movements. Huntington disease (HD) is a neurodegenerative disease characterized clinically by the triad of a movement disorder, dementia, and behavioral disturbances.
  • Case 37 - Young woman with recurrent “stroke” attacks
    pp 272-276
  • View abstract

    Summary

    This chapter speaks about an 82-year-old woman who was admitted with apparently sudden-onset neurological symptoms. On examination, this patient appeared well and comfortable. The patient's clinical evolution was characterized by a relentlessly progressive course over several weeks. The visual field defect expanded into a right homonymous hemianopia, and optic ataxia also developed on the left. Dysarthria and dysphagia worsened until she was dependent on tube feeding. The human prion diseases are a comparatively rare cause of dementia, with an estimated incidence of 1 case per million people per year, although some uncorroborated reports would place the true incidence much higher. Therapeutic options for all the human prion diseases are currently limited to palliation, as there are no agents capable of reliably causing a sustained improvement in clinical course. A large and diverse selection of drugs have been tried with limited success, including antivirals, antifungals, antibiotics, antimalarials, antidepressants, antioxidants, and analgesics.
  • Case 38 - Young man with slow cognitive decline
    pp 277-281
  • View abstract

    Summary

    This chapter speaks about a 75-year-old woman admitted with a 4-year history of progressive social withdrawal, decreased fluency, and difficulty handling complex tasks. Magnetic Resonance Imaging (MRI) of the brain showed prominent mesencephalic atrophy, dilation of the third ventricle, and mild cerebral atrophy. Based upon her history and examination, the patient met National Institute of Neurological Disorders and Stroke - Society for Progressive Supranuclear Palsy (NINDS-SPSP) diagnostic criteria for probable Progressive Supranuclear Palsy (PSP). Available data showed that the diagnosis of PSP was pathologically confirmed in essentially 100% of patients who meet these criteria. The patient's motor function progressively worsened, and she died from respiratory complications, approximately 6 years following symptom onset. Progressive supranuclear palsy is a sporadic neurodegenerative disease, which is defined clinicopathologically by the constellation of atypical parkinsonism, supranuclear vertical gaze palsy, and a characteristic pattern of tau accumulation within the brainstem and basal ganglia.
  • Case 39 - Young woman with lateralized motor symptoms
    pp 282-291
  • View abstract

    Summary

    This chapter presents a case study of a 69-year-old right-handed man who was presented in June 2006 with a 1-year history of progressive word finding difficulties and mild phono-articulatory problems. It provides the general history, family history, examination, initial diagnosis and follow-up data of the patient. In a simple delayed recall test, he was able to remember five out of ten figures, which is considered slightly impaired. Based on the overall clinical, neuropsychological, language, and neuroimaging data, a diagnosis of Progressive Non-Fluent Aphasia (PNFA) was made. Duloxetine was started for the depressive symptoms with good clinical response. Primary progressive aphasia (PPA) is a clinical syndrome characterized by progressive dissolution of language with relative preservation of other cognitive abilities for at least 1 to 2 years. Recent studies have classified the clinical presentations of PPA into three main subtypes: agrammatic, logopenic, and semantic variant.

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