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Hier, Daniel B.
Brint, Steven U.
Wunsch, Donald C.
Olbricht, Gayla R.
Evaluation of standard and semantically-augmented distance metrics for neurology patients.
BMC Medical Informatics and Decision Making,
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.
' … accessible and insightful, and easily digested …'
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This chapter talks about a 70-year-old woman who consulted for a severe cognitive complaint encompassing numerous domains. MRI showed no focal atrophy especially of the hippocampal regions. The initial diagnostic impression was a subjective cognitive complaint with an attention disorder of probable psychogenic origin. Subjective cognitive complaint (SCC) is frequent in normal aging with a prevalence of 50% after 55 years of age. Episodic memory is the capacity to recall personal events that can be identified in time or in space. Memory disorders in everyday life can result from attention disorders, retrieval difficulties as well as genuine memory deficit due to Alzheimer's disease. The best way to disentangle the diagnostic problem is to assess memory by objective tests that may control for attention and that can facilitate the retrieval. This is the case of the Free and Cued Selected Recall Test (FCSRT).
This chapter conveys that a 65-year-old technical worker with previous management function had been unable to work because of pulmonary problems for 7 years. This highly educated man had been diagnosed with extrinsic allergic alveolitis 7 years ago. A diagnosis of subjective memory complaints (SMC) was made. The fact that cerebrospinal fluid (CSF) values were abnormal was not taken into account at that time because of lack of a clinical diagnosis of dementia. Referral to a psychiatrist was advised for evaluation of a possible mood disorder and for counseling on how to deal with his behavioral disturbances. SMC or subjective cognitive impairment (SCI) in the absence of mild cognitive impairment (MCI) or dementia is more and more accepted as a separate clinical entity. Depression, anxiety, disturbed sleep, fatigue, and loss of energy were responsible for a conversion from SCI to MCI, but subsequently reverted back to SCI after follow-up.
This chapter talks about an 85-year-old man who was enrolled in a longitudinal study of healthy aging and Alzheimer disease (AD) at the Washington University Alzheimer Disease Research Center (ADRC). He was diagnosed with dementia of the Alzheimer's type (DAT) and died shortly after his 91st birthday. A psychometric battery was performed within 2 weeks of each annual clinical assessment. The detection of dementia at the ADRC is based on observations from a collateral source of decline from an individual's previously attained level of cognitive function that is sufficient to interfere with that individual's customary activities. This case illustrates that the accurate diagnosis of DAT can be made in individuals who meet criteria for mild cognitive impairment (MCI). The DAT diagnosis was based on informant observations that the individual had declined from previously attained levels of cognitive function, rather than comparing his cognitive performance to normative values.
This chapter presents a case of a 67-year-old right-handed woman with a history of a 3 cm mid left parietal lobe intra-cerebral hemorrhage (ICH) of uncertain etiology admitted to the inpatient neurology service after an acute episode of witnessed productive aphasia and inability to obey commands during an outpatient neurology assessment. Cognitive history indicates that she has noticed minor deficits of short-term memory and word finding since her stroke, but denied any functional deficits associated with her cognition. The patient was started on citalopram with the goal of improving her initiative and a cholinesterase inhibitor for her vascular cognitive impairment (VCI). A trial of cholinesterase inhibitor with measurable outcomes identified at the outset is reasonable, particularly since a neurodegenerative process cannot be ruled out as a contributing factor. Ongoing partial seizures may contribute to cognitive impairment. This patient's lamotrigine should be titrated to minimize future seizures.
This chapter describes right-sided numbness using the case of an 82-year-old woman who had a former history of hypertension, type 2 diabetes treated with oral hypoglycemic agents, rheumatoid arthritis, dysthymic disorder, and left dorsal herpes zoster with residual postherpetic neuralgia. The physical examination showed a conscious patient with moderate motor aphasia, right homonymous hemianopsia, and balanced faciobrachiocrural hemiparesis in association with hemihypoesthesia of the right hemibody. The brain magnetic resonance imaging (MRI) scan showed acute cerebral ischemia in the territory of the left middle cerebral artery. A diagnosis of cerebral infarction of cardioembolic origin was made. A final tentative diagnosis of vascular cognitive impairment that fulfilled criteria of multi-infarct dementia was established. Living performance after stroke was severely impaired and home assistance for daily living activities was needed. Treatment for the cognitive impairment includes speech therapy and physical rehabilitation.
This chapter presents the case of a 72-year-old man with a 10-year history of Parkinson's disease. He experienced visual hallucinations with preserved insight 2 years previously, subsiding on withdrawal of selegiline. Physical examination revealed a fairly symmetric and moderately severe parkinsonian syndrome, with akinetic-rigid features dominant and little in the way of rest tremor. The initial diagnostic impression was of a dementia syndrome relating to his Parkinson's disease, although the doctor was initially uncomfortable with what he regarded as a normal mini-mental state examination (MMSE) score of 27 out of 30. After 3 and 6 months, repeat MMSE scores were 23 and 25, respectively. Despite some initial diagnostic uncertainty, the patient was commenced on a cholinesterase inhibitor with considerable improvement in his psychotic features and lessening of his periods of confusion. Rationalization of anti-parkinsonian and other medications is an important first step in the management of PD-D.
This chapter describes hemiparesis followed by dementia using the case of a 73-year-old woman who presented to the emergency room with mild left hemiparesis since awakening the day before. Computed tomography (CT) scan showed a known old right parieto-temporal infarct and a right caudate nucleus lacune. Magnetic resonance imaging (MRI) done within 3 days of presentation show the same ischemic lesions without diffusion restriction, with mild leukoaraiosis and atrophy for age. Neuropsychological tests showed severe executive functions deficits, moderate to severe memory deficits, and mild visuoconstructive and ideomotor apraxia. The initial diagnostic impression was mixed dementia, although a purely vascular dementia could not be ruled out. Repeated mini-mental state examination (MMSE) showed decline over time, despite temporary improvement after initiation of a cholinesterase inhibitor, galantamine (Reminyl ER), 3 months after presentation. Blood pressure remained high during initial follow-up, requiring several anti-hypertensive medication adjustments.
This chapter presents a case of a 54 year old man Mr. H who had some neurotic personality features associated with some impulsivity. A majority of patients develop neuropsychiatric symptoms, also called behavioral and psychological symptoms of dementia (BPSD) during the course of their illnesses. Neuropsychiatrie symptoms are primary manifestations of the disease process, but other psychological and social factors also play a role in determining which patients will manifest behavioral symptoms. For Mr. H, irritability, anxiety, and delusion are related both to the personal history of the patient and his wife. They are also the consequences of the cognitive deficit and the patient psychological reaction to the perception of his memory loss. Mr. H. has a major loss of self-initiated behavior and interest. However, he is able to respond to external cognitive and emotional stimulation. This helps in choosing the best non-pharmacological strategies.
This chapter focuses on visual complaints using the case of a 53-year-old woman who reports difficulties over the past 6 months in handling her work as a specialized clothes cutter in a garment factory. A quantified regional perfusion study using 99mTc SPECT eleven months after onset of symptoms showed significant hypoperfusion of the left parieto-temporal regions and very slight of the right parietal region. This was unchanged on repeat examination eight months later. The initial diagnostic impression was a focal cognitive deficit related to early Alzheimer's disease, non-familial. Atypical presentations of Alzheimer's disease account for about 15% of cases. This case falls under the umbrella of posterior cortical atrophy, first described by Benson et al. in 1988, usually presenting with visuospatial difficulties including visual fields defects. Donepezil was given and well tolerated up to 10 mg a day.
This chapter talks about a 77-year-old right-handed woman who presented to clinic with a 7-year history of slowly progressive difficulty with visuospatial processing followed by language and memory dysfunction. The general physical examination was normal. On mental status examination, the patient was alert and fully oriented. Her speech was fluent with occasional word finding pauses. Based on the patient's history of slowly progressive visuospatial dysfunction followed by mild deficits in language and memory, physical and cognitive examination findings suggestive of biparietal dysfunction and marked parietal atrophy on brain imaging, the patient was diagnosed with posterior cortical atrophy (PCA). The most likely underlying histopathology was felt to be Alzheimer's disease (AD), based on the strong relationship between the PCA clinical syndrome and underlying AD. Corticobasal degeneration was also considered, but was felt to be less likely given the paucity of movement abnormalities and Parkinsonian features seven years after symptom onset.
This chapter presents the case of a 63-year-old right-handed woman who was referred for language difficulties. Significant deficits were present in executive and visuospatial functions, as well as language. Language was fluent, characterized by frequent semantic paraphasias, jargonophasia, and occasional word finding difficulties, and decreased understanding. The initial clinical diagnostic impression was that of a progressive but primarily focal cognitive disorder involving predominantly language: primary progressive aphasia (PPA). Autopsy confirmed the presence of a large left frontal hematoma with intraventricular spillage. Diffuse staining for b-amyloid was found in meningeal and cortical arteries, consistent with severe cerebral amyloid angiopathy (CAA). The differential diagnosis for PPA includes variants of fronto-temporal dementia (FTD) and atypical Alzheimer's disease (AD). The clinical profile, with the early onset, strong family history, absence of severe memory complaints on history and amnestic deficits on testing, behavioral changes, and a predominance of executive dysfunction, swayed the diagnosis towards FTD.
This chapter talks about a 61-year-old woman with a 5-year history of difficulty finding words and slowly progressive memory decline. At initial presentation, mood was good. General neurological exam was unremarkable. Language examination showed that spontaneous speech was fluent. Single photon emission computed tomography (SPECT) revealed reduced cortical perfusion in the left frontal, parietal, and temporal lobes compared to the right. The initial clinical impression was semantic dementia (SD). This senile dementia comprises one of the three clinical presentations of primary progressive aphasia (PPA). The other two are progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia. PPA has been recognized under several different labels and concepts over the last century. The term SD was coined in 1989 to describe three patients with fluent aphasia, impaired word comprehension and visual comprehension deficits. SD shows insidious onset and gradual progression that is characterized by understanding of word meaning and/or object identity.
This chapter talks about a 73-year-old right-handed woman who presented at initial consultation with 6 to 8 months history of progressive weakness in her arms bilaterally. Nerve conduction and EMG studies were obtained and showed evidence of denervation changes consistent of amyotrophic lateral sclerosis. Cervical spine MRI showed no abnormal signal in the cord. Brain MRI showed extensive frontotemporal atrophy, bilateral hippocampal atrophy, and periventricular deep white matter T2 hyperintense changes. The diagnostic impression was fronto-temporal dementia with amyotrophic lateral sclerosis (FTD-ALS). Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease involving both upper motor neuron and lower motor neuron characterized by progressive muscle weakness, muscle wasting, and spasticity. ALS has been traditionally considered to be a disorder affecting only motor systems; however, recent developments show that ALS is a multi-system disorder in which motor system deficits are the most prominent manifestations, but non-motor manifestations can also be observed.
This chapter talks about an 83-year-old man with a 3-4-year history of progressive speech difficulty. Past medical history was remarkable for hypertension and ischemic heart disease. Psychiatric history was unremarkable. General neurological exam was remarkable for bilateral cogwheeling in the upper extremities, rigid posture, and bilateral decreased arm swing. Magnetic Resonance Imaging (MRI) of the brain showed diffuse atrophy. Single-Photon Emission Computed Tomography (SPECT) showed decreased perfusion in the left temporo-parietal region. The findings suggested a diagnosis of progressive non-fluent aphasia (PNFA). The possibility of corticobasal syndrome (CBS) was also raised. A variety of neuropathological changes have been associated with PNFA. The most common are non-Alzheimer tauopathies. Alzheimer pathology has also being identified in PNFA, with some reports showing these in up to 30% of cases. In these patients, the distribution of AD pathology may be unusual, showing a frontotemporal pattern.
This chapter talks about a 54-year-old right-handed man who was brought to medical attention by his daughter because of progressive speech difficulty over the last 2 years. The patient was clinically diagnosed with fronto-temporal dementia with non-fluent progressive aphasia as well as behavioral symptoms. Sensory and motor nerve conduction studies were normal. EMG needle electromyography showed mixed denervation pattern in the right FDI and left biceps with 2_ fasciculation potentials, positive sharp waves, and fibrillations. Motor units were polyphasic with increase in sharp waves. External examination of the formalin-fixed brain showed no obvious cerebral atrophy or no focal lesions. The base of the brain was unremarkable apart from mild patchy atherosclerosis. Serial coronal sections through the cerebral hemispheres showed a normal ventricular system and deep gray structures. Sections of the brainstem and cerebellum were also unremarkable apart from mild loss of pigmentation of substantia nigra.