Visual deficits in a patient with `kaleidoscopic disintegration of the visual world''

We describe psychophysical, neuropsychological and neuro-ophthalmological studies of visual abilities in a patient who, following a right hemisphere stroke, had difficulty in combining parts of objects into a whole and in reading. Strikingly, her perceptual problems were accentuated when the objects moved or when she moved. Formal testing showed that her main deficits were in depth perception, various tasks of motion and object recognition of degraded stimuli. But low-level detection and discrimination of form and color were normal. Despite her deficits in visual motion and degraded static-object recognition, her visual recognition of 'biological motion' stimuli was normal. Structural magnetic resonance imaging revealed an infarct in the ventro-medial occipito-temporal region, extending ventro-laterally and leading to a 'kaleidoscopic disintegration of visible objects'.     

Use of intravenous tissue plasminogen activator in a 16-year-old patient with basilar occlusion

Intravenous tissue plasminogen activator (t-PA) is currently approved by the US Food and Drug Administration (FDA) for the treatment of ischemic stroke in patients > 18 years of age who present within 3 hours of stroke onset and meet certain criteria. We report a case of a 16-year-old, previously healthy female who presented with a basilar artery occlusion and pontine ischemic stroke. She was treated with intravenous t-PA approximately 4 hours after the onset of symptoms. The patient demonstrated a remarkable recovery 6 hours after onset of her symptoms and had minimal deficits on discharge from the hospital 1 week later. She was found to have a lupus anticoagulant and was heterozygous for the prothrombin gene G2010A mutation. These were likely contributing causes for her stroke. She was also homozygous for plasminogen activator inhibitor 1 (PAI-1) 4G/4G, which at present is a controversial stroke risk factor.     

Inflammatory cerebral amyloid angiopathy: the overlap of perivascular (PAN-like) with vasculitic (Aβ-related angiitis) form: an autopsy case

Beside advanced age, cerebral amyloid angiopathy (CAA) and hypertension (HTA) are the two most important risk factors for haemorrhagic stroke. Inflammatory changes of amyloid-laden vessels have been reported only in rare sporadic CAA cases. We present the case of a 78-year-old woman with a history of hypertension, dementia and haemorrhagic stroke of the right frontal lobe 2 years before admission. She was admitted with recurrence of symptoms of transient aphasia and central, right-side facial paresis that occurred an hour before her arrival at the hospital. In the admission unit, she was only slightly confused, with no other neurological deficits. An urgent CT scan revealed a recent haemorrhagic stroke in the left frontal lobe. In an hour her condition suddenly deteriorated. After a generalized seizure she presented with right-side hemiparesis with signs of uncal herniation and remained unconscious. A control CT scan showed a large haemorrhagic expansion that comprised the whole left brain hemisphere with 2 cm midline shift. She died about 10 hours after the onset of symptoms. At autopsy chronic inflammation of the thyroid gland, bronchopneumonia, fibrous and fatty heart degeneration and kidney haemorrhagic infarcts were documented. Amyloid deposition and systemic immune disorders in the inner organs were not demonstrated. In neuropathological examination we diagnosed inflammatory form of CAA with coexistence (the overlap) of two, perivascular and vascular, subtypes of CAA-related inflammation.     

A qualitative longitudinal case study of a daughter's adaptation process to her father's aphasia and stroke

Background: Aphasia has repercussions on the lives of families who adjust to the new situation of their parent. Most data concerning how family members adapt to aphasia concern spouses, and less so children. However, adult children are likely to encounter specific problems because of the different nature of parent–child relationships.

Aims: This study aimed to describe the experience of a daughter's adaptation process to her father's stroke and aphasia during the first year post‐stroke.

Methods & Procedures: A qualitative approach was employed to explore the experience of one individual recounted in the context of face‐to‐face interviews. The participant was a 31‐year‐old woman living with her parents at the time her father became aphasic at the age of 60 years. Three interviews were conducted: (1) when her father began outpatient rehabilitation, (2) near the end of outpatient rehabilitation, and (3) three months later.

Outcomes & Results: Results revealed that the sources of stress for this participant evolved over time from initial concerns for her father's survival and health, to preoccupations concerning her father's communication and their relationship, and lastly to concerns about how her own life had been altered. In reaction to stress the participant was proactive, obtained information, modified her communication with him, and oversaw his health and treatments. Over time some of the negative consequences diminished and more positive indicators emerged. The strategies the participant employed allowed her to feel she did the right thing, and also she preserved her self‐esteem. However, at the last interview she still suffered from the changes in her relationship with her father and their communication restrictions.

Conclusions: The results indicate that the experience of a daughter was an evolving process, involving negative and more positive consequences. The process of adaptation was influenced by the particular life situation of this participant, her perception of stresses, and the types of strategies she employed. These results show that children may be stressed by many changes associated with a stroke, such as communication limitations. When possible, adult children should be included in the rehabilitation process since they provide skills, abilities, and affection in caring for their parent with aphasia. These may contribute to the reduction of overall stress related to a family's adaptation to the consequences of aphasia and stroke.     

Familial antithrombin III abnormality accompanied with progressing ischemic stroke

We report a case of familial antithrombin III (AT-III) abnormality accompanied with progressing ischemic stroke. The patient was a 31-year-old female who developed consciousness disturbance and left hemiparesis on December 1 in 1987. She had a history of two transient ischemic attacks and three episodes of thrombophlebitis of the extremities. Cerebral CT scan showed a low density area on the right temporal lobe that had extended to the right parietal and on the left frontal lobe as clinical symptoms worsened. Cerebral angiogram revealed branch occlusions of the right middle cerebral artery and showed no cerebral venous and sinus obstruction. When her symptoms had been progressing to show semi-comatose state, left hemiplegia and transient Cheyne-Stokes respiration, we found her decreased biological activity and normal immunological level of AT-III. The diagnosis of familial AT-III abnormality had been made by familial investigation. As the treatment of AT-III concentrates transfusion was started from the third day, her symptoms gradually recovered and the low density area stopped extending. Further examinations revealed that she was a homozygote of AT-III abnormality presenting no affinity for heparin and that her parents were heterozygotes. It was suggested that the homozygous AT-III abnormality was the main cause of her progressing ischemic stroke.     

Changes in differential gene expression during a fatal stroke

We present a young woman (with an identical twin sister) who arrived at the Emergency Department (ED) within 1 hour of her initial stroke symptoms. Previous microarray studies have demonstrated differential expression of multiple genes between stroke patients and healthy controls. However, for many of these studies there is a significant delay between the initial symptoms and collection of blood samples, potentially leaving the important early activators/regulators of the inflammatory response unrecognised. Blood samples were collected from the patient for an analysis of differential gene expression over time during the evolution of a fatal stroke. The time points for blood collection were ED arrival (T0) and 1, 3 and 24 hours post ED arrival (T1, T3 and T24). This was compared to her identical twin and an additional two age and sex-matched healthy controls. When compared to the controls, the patient had 12 mRNA that were significantly upregulated at T0, and no downregulated mRNA (with a cut off fold change value ±1.5). Of the 12 upregulated mRNA at T0, granzyme B demonstrated the most marked upregulation on arrival, with expression steadily declining over time, whereas S100 calcium-binding protein A12 (S100A12) gene expression increased from T0 to T24, remaining >two-fold above that in the healthy controls at T24. Other genes, such as matrix metalloproteinase 9, high mobility group box 2 and interleukin-18 receptor I were not upregulated at T0, but they demonstrated clear upregulation from T1–T3, with gene expression declining by T24. A greater understanding of the underlying immunopathological mechanisms that are involved during the evolution of ischaemic stroke may help to distinguish between patients with stroke and stroke mimics.     

Alexia for Braille following bilateral occipital stroke in an early blind woman

Recent functional imaging and neurophysiologic studies indicate that the occipital cortex may play a role in Braille reading in congenitally and early blind subjects. We report on a woman blind from birth who sustained bilateral occipital damage following an ischemic stroke. Prior to the stroke, the patient was a proficient Braille reader. Following the stroke, she was no longer able to read Braille yet her somatosensory perception appeared otherwise to be unchanged. This case supports the emerging evidence for the recruitment of striate and prestriate cortex for Braille reading in early blind subjects.     

Multiple Cerebrovascular Insults in Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE) is a rare systemic genetic disorder and an uncommon cause of ischaemic and haemorrhagic strokes. Its rarity and variable presentation may delay recognition and diagnosis of the primary disorder or associated conditions. Here, we describe a patient of European ancestry diagnosed with PXE in her 20s who presented in her 50s with a haemorrhagic stroke. Subsequent workup additionally revealed clinically silent ischaemic cerebral infarcts, critical stenosis of the right internal carotid artery and intracranial vasculopathy. Though she had some typical vascular risk factors, they were well-controlled. Antiplatelet therapy has traditionally been avoided in PXE due to increased risk of GI (and potentially retinal and cerebral) haemorrhage, but the medical team opted to start aspirin for secondary stroke prevention because she had no history of GI or retinal bleed, and her risk of ischaemic stroke was considered unacceptably high compared with that of clinically significant haemorrhage. Judicious use of antiplatelet therapy may be relatively safe in carefully selected patients. Anticipatory surveillance and management of the numerous manifestations of this potentially debilitating disorder are also important to preserve function and quality of life.     

Bow Hunter's Stroke Caused by a Severe Facet Hypertrophy of C1-2

Bow hunter''s stroke is a rare symptomatic vertebrobasilar insufficiency in which vertebral artery (VA) is mechanically occluded during head rotation. Various pathologic conditions have been reported as causes of bow hunter''s stroke. However, bow hunter''s stroke caused by facet hypertrophy of C1-2 has not been reported. A 71-year-old woman presented with symptoms of vertebrobasilar insufficiency. Spine computed tomography showed massive facet hypertrophy on the left side of C1-2 level. A VA angiogram with her head rotated to the right revealed significant stenosis of left VA. C1-2 posterior fixation and fusion was performed to prevent serious neurologic deficit from vertebrobasilar stroke.     

Residual tactile sensitivity with self-directed stimulation in hemianaesthesia.

A case of hemianaesthesia caused by right hemisphere stroke was found to have good sensitivity and cutaneous localisation when she touched her impaired hand with her good contralateral hand or with a probe held by that hand. The capacity did not depend solely upon movement or positioning of her right arm, and she could also discriminate whether her own left fingers, as opposed to someone else's inter-digitated fingers, were being touched, but only when she did the touching. Thus, the threshold of the impaired hand appeared to be markedly affected by active involvement of the contralateral limb in delivering the stimulus.     

Cortical reorganization and somatic delusional psychosis: a magnetoencephalographic study

A woman complained of feeling a "metal-like thing" in her oral cavity 4 years after a stroke. She was convinced of the physical nature of her complaint despite intact dental and neurological findings. Somatosensory evoked magnetic fields suggested that her decreased right SII function was compensated for by the right SI region, probably contributing to the delusional symptom.     

Premature stroke in a family with lupus anticoagulant and antiphospholipid antibodies

Lupus anticoagulant and antiphospholipid antibodies are associated with thromboembolic phenomena in individuals both with and without systemic lupus erythematosus. A 32-year-old woman (the index case) with lupus anticoagulant, multiple cerebrovascular events, and a family history of premature stroke raised the possibility of a familial diathesis. Histories or interviews, examinations, and blood tests were obtained for 23 members of four generations of her family. Four individuals had suffered strokes and three more had suffered neurologic symptoms. Two living individuals who had suffered strokes, two individuals with neurologic symptoms, and five asymptomatic individuals had antiphospholipid activity in their blood. In addition, a cousin of the index case was found to have systemic lupus erythematosus and antiphospholipid activity. Elevated concentrations of von Willebrand factor antigen were found associated with some positive lupus anticoagulant assays, the highest concentrations in the two individuals with stroke. The characteristic presentation of the index case and her good response to treatment suggests that further studies of families in whom antiphospholipid antibodies may represent a risk factor for stroke is worthwhile.     

Retinal signs and stroke: revisiting the link between the eye and brain

BACKGROUND AND PURPOSE: The retinal and cerebral vasculature share similar anatomic, physiological, and embryological characteristics. We reviewed the literature, focusing particularly on recent population-based studies, to examine the relationship between retinal signs and stroke. Summary of Review- Hypertensive retinopathy signs (eg, focal retinal arteriolar narrowing, arterio-venous nicking) were associated with prevalent stroke, incident stroke, and stroke mortality, independent of blood pressure and other cerebrovascular risk factors. Diabetic retinopathy signs (eg, microaneurysms, hard exudates) were similarly associated with incident stroke and stroke mortality. Retinal arteriolar emboli were associated with stroke mortality but not incident stroke. There were fewer studies on the association of other retinal signs such as retinal vein occlusion and age-related macular degeneration with stroke, and the results were less consistent. CONCLUSIONS: Many retinal conditions are associated with stroke, reflecting possible concomitant pathophysiological processes affecting both the eye and the brain. However, the incremental value of a retinal examination for prediction of future stroke risk remains to be determined.     

Multitasking disorder from right temporoparietal stroke.

Acute behavioral syndromes after right hemisphere stroke (eg, hemi-inattention) resolve in most patients by 6 months. Less is known about the nature of chronic disorders after these lesions when the initial deficits are no longer apparent. We report the case of a woman who suffered a cerebral infarct in the right temporoparietal region and who, after the resolution of neglect, demonstrated a significant disorder of multitasking in her everyday life. Neuropsychologic tests failed to reveal the extent of disruption in her daily functioning. A journal of daily activities elucidates the nature of her deficits and demonstrates, from the patient's perspective, the impact of the lesion on her ability to multitask. Our findings are consistent with the concept of a right frontoparietal attentional axis that governs the ability to hold several tasks in working memory at the same time. We conclude that right hemisphere lesions can disrupt the cognitive system of multitasking through disruption of the attentional matrix.     

Risk factors and subtype analysis of acute ischemic stroke

Background and Purpose: Stroke risk factors and subtypes were examined for associations with mortality and recurrence rate in Taiwanese patients with first-ever and recurrent stroke. Methods: This study examined patients with initial and recurrent stroke from 2003 to 2005 for risk factors, demographic data, Bamford subtypes and transient ischemic attack. Results: One thousand and twenty-one patients with 1,085 stroke episodes were recruited. Significant factors associated with recurrent stroke were hyperlipidemia, atrial fibrillation and smoking. A significant incidence of lacunar infarction was noted in the patient population (37.82% in all patients; 41.02% in first stroke vs. 35.67% in recurrent stroke patients). Patients with diabetes mellitus were more likely to have lacunar rather than total anterior circulation infarction (TACI) or partial anterior circulation infarction (PACI). Hyperlipidemia was more often attributable to lacunar stroke than PACI. Atrial fibrillation was significantly associated with nonlacunar infarcts, TACI and PACI but not lacunar stroke. Conclusions: The incidence of lacunar infarction was significantly higher in initial rather than recurrent stroke patients. Diabetes mellitus or hyperlipidemia was highly associated with lacunar infarction. Atrial fibrillation was associated with nonlacunar infarction in Taiwan, similar to the result of White and Black populations in the UK.     

Use of computer games as an intervention for stroke

Current rehabilitation for persons with hemiparesis after stroke requires high numbers of repetitions to be in accordance with contemporary motor learning principles. The motivational characteristics of computer games can be harnessed to create engaging interventions for persons with hemiparesis after stroke that incorporate this high number of repetitions. The purpose of this case report was to test the feasibility of using computer games as a 6-week home therapy intervention to improve upper extremity function for a person with stroke. One person with left upper extremity hemiparesis after stroke participated in a 6-week home therapy computer game intervention. The games were customized to her preferences and abilities and modified weekly. Her performance was tracked and analyzed. Data from pre-, mid-, and postintervention testing using standard upper extremity measures and the Reaching Performance Scale (RPS) were analyzed. After 3 weeks, the participant demonstrated increased upper extremity range of motion at the shoulder and decreased compensatory trunk movements during reaching tasks. After 6 weeks, she showed functional gains in activities of daily living (ADLs) and instrumental ADLs despite no further improvements on the RPS. Results indicate that computer games have the potential to be a useful intervention for people with stroke. Future work will add additional support to quantify the effectiveness of the games as a home therapy intervention for persons with stroke.     

A case of foreign accent syndrome resulting in regional dialect

BACKGROUND: Foreign Accent Syndrome (FAS) is a rare acquired syndrome following neurological damage that results in articulatory distortions that are commonly perceived as a "foreign" accent. The nature of the underlying deficit of FAS remains controversial. We present the first reported Canadian case study of FAS following a stroke. We describe a stroke patient, RD, who suffered an acute infarction to the left internal capsule, basal ganglia and frontal corona radiata. She was diagnosed as having FAS without any persistent aphasic symptoms. Family, friends, and health care professionals similarly described her speech as sounding like she had a Canadian East Coast accent, a reported change from her native Southern Ontario accent. METHOD: An investigation of this case was pursued, incorporating neuroimaging, neuropsychological and speech pathology assessments, and formalized linguistic analyses. RESULTS: Linguistic analyses confirmed that RD's speech does in fact have salient aspects of Atlantic Canadian English in terms of both prosodic and segmental characteristics. However, her speech is not entirely consistent with an Atlantic Canadian English accent. INTERPRETATION: The fact that RD's speech is perceived as a regional variant of her native language, rather than the "generic foreign accent" of FAS described elsewhere, suggests that the perceived "foreignness" in FAS is not primarily due to dysfluencies which indicate a non-native speaker, but rather due to very subtle motor-planning deficits which give rise to systemic changes in specific phonological segments. This has implications for the role of the basal ganglia in speech production.     

Isolated asymptomatic pulmonary arteriovenous malformation presenting with ischaemic stroke

Young onset stroke is uncommon, and may be due to conditions other than traditional vascular risk factors. A 42-year-old woman with an ischaemic stroke was found to have left atrial bubble study positivity on transthoracic echocardiogram (TTE) suggestive of patent foramen ovale, however she also had low peripheral oxygen saturation. Investigation revealed an isolated pulmonary arteriovenous malformation (PAVM), visible on admission chest radiograph. This can cause embolic stroke and is an alternate cause of the TTE findings. The PAVM was able to be closed via endovascular intervention, removing the shunt and therefore removing her risk of recurrent stroke events. This is a rare cause of embolic stroke in young people which can be easily missed on investigation yet is amenable to treatment.