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Q5B3 Neuro

Terms in this set (140)

Subacute combined degeneration of the spinal cord
This patient is at risk for B12 deficiency. This disorder usually presents with neuropathy first with diminished reflexes if the disease is not treated then spinal cord symptoms appear (Babinski's). This is one of only a few disorders that may present with both upper motor and lower motor symptoms (ALS, Syphilis, Friedreich's ataxia, vitamin E deficiency, cervical spondylitic myelopathy). Sensory ataxia due to the peripheral neuropathy prevents her proprioceptive feed back and she is unable to navigate in the dark without visual cues (hence the positive Romberg test). This is very common in patients with pernicious anemia (however the neurologic symptoms may predate by months, the macrocytic anemia found with the serum test or before the Shillings test becomes positive for pernicious anemia). Diagnosis is made by using chemiluminescence assay of for cobalamin (normal range 230 - 931 pg/mL). Treatment is with intramuscular cobalamin (B12) 1000 ug, or sublingual 2000 ug usually monthly. I have treated patients with severe depletion (0 - 230 pg/mL) with weekly injections for 1 month then biweekly for 1 month then monthly then rechecking the serum after they have been on the once/monthly treatment for 2-3 months, in order, to see if they maintain a mid to high range cobalamin level. The neuropathy may take months to 1 year to improve and the spinal cord may never improve. This is why your index of suspicion should be greatest when potential malnutrition occurs in the presence of neuropathy, diagnose it before the spinal cord becomes involved.
Dementia with Lewy-body

Dementia with Lewy bodies overlaps clinically with Alzheimer's disease and Parkinson's disease, but is more associated with the latter.[1] In DLB, loss of cholinergic (acetylcholine-producing) neurons is thought to account for degeneration of cognitive function (similar to Alzheimer's), and loss of dopaminergic (dopamine-producing) neurons for degeneration of motor control (similar to Parkinson's) - in some ways, therefore, it resembles both diseases. The overlap of neuropathologies and presenting symptoms (cognitive, emotional, and motor) can make an accurate differential diagnosis difficult. In fact, it is often confused in its early stages with Alzheimer's disease and/or vascular dementia (multi-infarct dementia), although, where Alzheimer's disease usually begins quite gradually, DLB often has a rapid or acute onset, with especially rapid decline in the first few months. DLB tends to progress more quickly than Alzheimer's disease.[2] Despite the difficulty, a prompt diagnosis of DLB is important because of the risks of sensitivity to neuroleptic drugs and because appropriate treatment of symptoms can improve life for both the person with DLB and their caregivers.[2]
DLB is distinguished from the dementia that sometimes occurs in Parkinson's disease by the time frame in which dementia symptoms appear relative to Parkinson symptoms.[3] Parkinson's disease with dementia (PDD) would be the diagnosis when dementia onset is more than a year after the onset of Parkinson's. DLB is diagnosed when cognitive symptoms begin at the same time or within a year of Parkinson symptoms.
A 55-year-old man, premorbidly described by his family as "thoughtful, accomplished, and intelligent," began neglecting his home and work responsibilities over a 2-year period. He became increasingly inflexible and uncaring. At work he missed several deadlines, and clients complained that he "forgot" about them. Consequently, he stopped working. He became more impulsive, driving late at night without reason. He obsessively checked his furnace numerous times each day and night, unconcerned about this safety risk.
His wife became increasingly tearful and anxious, whereas he seemed unaware of her turmoil and his change in personality. The patient's personal hygiene declined; he stopped shaving and dressed sloppily. At social functions, he interrupted conversations, touched people inappropriately, and spoke in a tasteless and loud fashion, often embarrassing his wife. Despite these changes, he continued to garden and perform other favored activities, albeit with less attention to detail.
Examination results revealed a malodorous and unshaven man with disheveled clothes. He spoke out of turn and was repetitive, stating, 'I have to go." At times he attempted to leave the examination room but returned with gentle coaxing. His affect was otherwise flat. He gave concrete, terse responses to questions, mostly affirmative, negative, or stating, "I don't know." Naming was impaired. He followed some simple commands, but more complex sequences were incomplete or disorganized. His memory was relatively intact, although retrieval of relatives' names was impaired. He listed only 5 animals in 1 minute, a significant impairment given his postgraduate education level. Other than motor impersistence and mild rooting reflex bilaterally, the results of the neurologic examination were relatively unremarkable. Over the next 2 years, the patient became increasing withdrawn, spoke less, and required prompting for virtually every activity.

The most likely diagnosis is

Parkinson's dementia complex
Huntington's disease
Wilson's disease
Fronto-temporal dementia
Lewy-body dementia
Alzheimer's disease

Alzheimer's is the most common cause of dementia among older adults. The major pathogenesis is the production and accumulation of beta-amyloid peptide, bringing about the formation of neurofibrillary tangles, oxidation and lipid peroxidation, glutamatergic excitotoxicity, inflammation, and activation of the cascade of apoptotic cell death. Furthermore, the other hypothesis regarding the pathophysiology of AD stresses tau-protein abnormalities, heavy metals, vascular factors, and viral infections.[4] The natural course of AD averages 10 years. The cardinal features are insidious onset, progressive course, and early memory loss; at least one other cognitive impairment such as language dysfunction, apraxia, agnosia, visuospatial disorder, as well as executive dysfunction, must be seen. These impairments should constitute a decline from the previous level of cognitive functioning, interfering with daily activities.

Memory decline is the hallmark of cognitive change in AD. It is characterized as a storage deficit, meaning that material cannot be recalled with cue. In the early stage, memory impairment for recent events is common whereas long-term memory remains intact. As the disease progresses, individuals with AD are increasingly unable to recall more distant memories. Typically, the motor signs are absent early in the course. Likewise, sensory abnormalities, seizures, and gait difficulties are uncommon until the late phase of disease. Behavioural changes, including depression, anxiety, apathy, aggression, agitation, wandering, vocalization, disinhibition, and abnormal eating, are common thereafter and cause caregiver stress as well as greater need for additional health care services.
Vascular Dementia.

There are several clinical syndromes of vascular dementia (VaD), categorized into multi-infarct dementia,
single strategic infarct (single brain infarct damaging functionally critical areas of the brain such as angular gyrus, thalamus, basal forebrain, posterior cerebral artery, and anterior cerebral artery territories),
lacunar state,
Binswanger's disease,
genetic forms (e.g., cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy [CADASIL]), and hypoxic ischemic encephalopathy.

A number of mechanisms causing these clinical syndromes are hemorrhage, ischemia/thrombosis, vasospasm, low perfusion, hematologic and rheological problems.

Since cases do not share common etiology and mechanism, patients may have different clinical presentations. For example, the onset may be abrupt or insidious. The progression may be stepwise, fluctuating, or marked by continuous worsening. Frequently, individuals with VaD present with gradual and progressive cognitive decline without any stroke events. However, typical cases of VaD are usually seen with atherosclerotic co-morbidities (diabetes mellitus, hypertension, coronary heart disease, and peripheral artery disease). The onset of cognitive decline is either subtle or abrupt, and there is psychomotor slowing, executive dysfunction, focal cognitive deficits and motor signs. The temporal association between the cerebrovascular event and the onset of dementia should be within 3 months. Nevertheless, as VaD is variable, sometimes the temporal association cannot be demonstrated easily due to an unclear onset of vascular event.