Hippocampal sclerosis, TDP-43, and the duration of the symptoms of dementia of AD patients.

OBJECTIVES: To examine the relationship between duration of the cognitive symptoms, from the earliest reported symptom to death, and hippocampal sclerosis (HS) and TAR-DNA binding protein of 43kDA (TDP-43) in Alzheimer's disease (AD) patients. METHODS: The study was conducted in 359 cognitively impaired patients who met the pathological criteria for AD (NIA-Reagan intermediate or high). The mean age at onset was 69.5 ± 8.8 years (range 37-95) and the mean duration of the symptoms was 10.5 ± 4.2 years. The association between symptoms duration and HS and TDP-43 was examined with logistic regression analyses controlling for age at death, atherosclerosis in the Circle of Willis (CW), cerebral infarcts, gender, baseline Mini Mental State Examination scores, APOE-4 allele, and presence of Lewy bodies (LB). RESULTS: HS was present in 18% (n = 64) and TDP-43 in 51.5% (n = 185) of the patients. HS and TDP-43 were more frequent in patients whose symptoms lasted more than 10 years. LBs were present in 72% of the patients with HS and in 64% of the patients with TDP-43. Age at onset was not associated with TDP-43 or HS. HS was associated with duration of symptoms and LB, TDP-43, and atherosclerosis in the CW. TDP-43 was associated with duration of symptoms, LB, and HS. INTERPRETATION: HS and TDP-43 are present in early and late onset AD. However, their presence is mainly driven by the duration of symptoms and the presence of LB. This suggests that HS and TDP-43 are part of the later neuropathological changes in AD.

Rehabilitation After Complete Hemisensory Loss: Report of Two Cases.

The role and function that proprioception plays in movement and motor learning have been debated since the 19th century but can be difficult to isolate and study. Lesions at various points along the proprioceptive pathway result in afferent paresis that can be significantly disabling. Compensatory mechanisms can help with successful rehabilitation and provide an opportunity to study the role of these mechanisms in sensory feedback. Here, we present two cases of adult patients with complete hemisensory loss after a stroke: one patient with a cortical stroke and the other one with a thalamic stroke. First, we see that that motor learning can occur without proprioception, with the help of visual feedback. Second, proprioception plays an important role in movement: in the upper limb, it can facilitate individual finger movements, and in the lower limb, it maintains sufficient knee flexion to prevent the knee from going into recurvatum (backward bending) during ambulation.

Extensive meningeal enhancement in acute central nervous system Lyme: Case series and review of literature.

INTRODUCTION: Clinical presentation of the central nervous system Lyme disease is nonspecific and therefore brain imaging and disease-specific serological testing is generally pursued to assist with diagnosis. Brain imaging findings are, however, rare and often unspecific. CASE DESCRIPTION: In the current report, we are presenting a rare magnetic resonance imaging (MRI) finding of extensive meningeal enhancement in two patients with acute Lyme disease. DISCUSSION: We discussed clinical implications and reviewed the relevant literature.

A retrospective analysis of care in patients with dementia hospitalized at a tertiary medical center.

BACKGROUND: Hospitalization is an opportunity to address various aspects related to management of dementia, including the goals of care to avoid futile care. We studied the prevalence of these factors when patients with dementia are hospitalized. DESIGN: One hundred and thirty-one charts of patients with dementia admitted to a single university-based hospital were retrospectively reviewed. Data were collected and analyzed for age, gender, the severity of dementia, co-morbidities, co-existing vascular dementia, reversible causes of dementia, mental status scores, medications, consultations, procedures, complications, availability of advance directives, decision-maker, code status, and goals of care discussion. RESULTS: In patients with dementia, co-morbidities and vascular disease burden were frequent. When these patients were hospitalized, use of psychotropic medications, invasive procedures, and multi-specialty consultations was common. Tests of mental status, screening for reversible causes, and use of FDA-approved medications for dementia is less common. Despite the lack of advance directives, goals of care were infrequently discussed. When goals of care were discussed, proxy decision-maker preferred palliative care and long-term institutionalization on discharge. CONCLUSION: Goals of care and other aspects of management are not fully addressed in hospitalized patients with dementia.

Occipital dysembryoplastic neuroepithelial tumor presenting as adult-onset temporal epilepsy.

Dysembryoplastic neuroepithelial tumor (DNET) is a benign brain tumor which commonly presents as childhood-onset temporal lobe epilepsy (TLE). We present a case of histologically proven DNET with a clinical presentation and scalp EEG suggestive of adult-onset TLE. MRI showed an occipital lesion. PET showed abnormal metabolism of the occipital lesion and the ipsilateral temporal lobe; raising concern for an abnormal functional network reorganization. Intracranial EEG showed interictal spikes and seizures originating from the occipital lesion with no seizures emanating from the temporal lobe. Occipital DNET due to their chronic nature can reorganize the network and mimic TLE.

Pure apraxia of speech due to infarct in premotor cortex.

Apraxia of speech (AOS) is now recognized as an articulation disorder distinct from dysarthria and aphasia. Various lesions have been associated with AOS in studies that are limited in precise localization due to variability in size and type of pathology. We present a case of pure AOS in setting of an acute stroke to localize more precisely than ever before the brain area responsible for AOS, dorsal premotor cortex (dPMC). The dPMC is in unique position to plan and coordinate speech production by virtue of its connection with nearby motor cortex harboring corticobulbar tract, supplementary motor area, inferior frontal operculum, and temporo-parietal area via the dorsal stream of dual-stream model of speech processing. The role of dPMC is further supported as part of dorsal stream in the dual-stream model of speech processing as well as controller in the hierarchical state feedback control model.

Bilateral persistent primitive hypoglossal arteries associated with unilateral symptomatic carotid thromboembolism.

We report the fifth case of bilateral persistent primitive hypoglossal arteries in the literature. This is also the first such case to be demonstrated on computerized tomography angiogram (CTA) and the first case to be associated with a symptomatic carotid thrombus. The sub-occlusive thrombus was distal to the take-off of the dominant persistent hypoglossal artery (PHA) from the internal carotid artery, thus sparing involvement of posterior circulation. Timely identification of the internal carotid artery thromboembolism in the setting of a PHA is important to allow for thrombectomy. Any intervention was not done in this case as the patient was out of the window and at an additional risk of inducing intracranial thromboembolism. Symptomatic carotid stenosis at the PHA take-off is typically treated with endovascular angioplasty and stenting due to the typically high level of the bifurcation in the neck.

Stroke in a young patient with neurosyphilis and HIV.

Syphilis has long been known as the great imitator. Its heterogeneity can manifest in the form of meningitis, space occupying gummas, vasculitis, strokes, cranial neuropathies, myelopathy, dementia, and seizures. The incidence has been rising with each year, mainly in men who have sex with men accounting for 83% of cases. With the coexistence of immunocompromised states, especially HIV (human immunodeficiency virus), the usually chronic and insidious course of tertiary neurosyphilis can be accelerated. Stroke can occur as a result of neurosyphilis in its meningovascular form, and the likelihood of this increases with HIV co-infection, especially in high-risk groups such as intravenous drug users and men who have sex with men. Here, we discuss a case of a young man who presented with an ischemic stroke found to have neurosyphilis and HIV and consider the management of these co-morbid conditions.

LGI1-negative faciobrachial dystonic-like seizures originating from the insula.

We expand the differential diagnosis of LGI1-positive faciobrachial dystonic seizures (FBDS) by presenting a 67-year-old woman affected by seizures of similar semiology who was found to have insular epilepsy. We report the distinct characteristics of insular faciobrachial dystonic-like seizures that would help clinicians to differentiate them from typical LGI1-positive FBDS, thus, guiding therapy while awaiting antibody results. LGI1-negative faciobrachial dystonic-like seizures should be considered when the seizure semiology includes unilateral and prolonged dystonia without loss of awareness, there is an ictal EEG correlate, MRI is suggestive of insular lesion, and when there is neither clearly associated memory impairment nor hyponatremia.

Isolated, relative aproverbia without focal lesion.

We have seen a patient with a profound, isolated, and quite selective deficit in proverb interpretation-aproverbia. The patient presented to us after an anoxic brain injury with aproverbia. Interestingly, the aproverbia appeared to be premorbid to the presenting event. Furthermore, the patient had no brain lesion that has been associated or even proposed as a cause of deficit in proverb or metaphor interpretation. The patient did have acute bilateral hippocampi lesions and associated severe anterograde amnesia, but he retained good retrograde memory with which he is able to give good, logical but concrete explanations for proverbs. This case highlights the need, importance, and interest in further neuropsychologic, imaging and functional studies of proverb and interpretation in patients and normal subjects populations.

Double Disassociation of Anosognosia for Alexia and Simultanagnosia but Quantitative Awareness of Optic Ataxia.

A 66-yr-old man with a history of atrial fibrillation and a pacemaker developed sudden onset confusion, disorientation, and visual disturbance without motor weakness. Clinically, significant deficits were found in reading (alexia) and simultaneous multiobject perception (simultanagnosia), both of which the patient denied limitation in, and in vision-right hemianopsia-which he readily acknowledged. Visual acuity in the left visual field was normal. The patient also demonstrated a symptom of optic ataxia-a lack of coordination between visual inputs and hand movements-a deficit he also acknowledged. Work-up with computed topography revealed a left posterior cerebral artery infarct affecting the occipital lobe and extending to involve the parietal lobe and the splenium of the corpus callosum. The authors describe and discuss this fascinating case-the first case to their knowledge of a double disassociation of anosognosia for alexia and simultanagnosia but full, indeed quantitative, awareness of hemianopsia and optic ataxia. This case may be informative on the mechanism of anosognosia in general and supports intentional feed-forward and exemplar reafference models. With regard to the rehabilitation process, appreciation that a patient has anosognosia for various deficits is crucial in recovery and health maintenance.