Welcome to the WSC 2022 Interactive Program

Background and Aims

Genetic stroke syndromes are extremely rare and clinically heterogeneous.

Methods

A case series of four patients is described.

Results

Patient 1: A 34 years-old male developed sudden vertigo and ataxia, with torsional nystagmus and left cerebellar signs. MRI brain showed multiple small pontine and cerebellar acute infarcts. MR angiography revealed bilateral vertebral artery dissection. Clinical exome sequencing (CES) demonstrated a likely pathogenic heterozygous variant in the MYLK gene, confirming the diagnosis of famililal thoracic aortic aneurysm, type 7.

Patient 2: A 41 years-old female presented with a single seizure, followed by a persistent neurobehavioral syndrome. MRI brain showed bilateral frontal gyral T2/FLAIR hyperintensities with restricted diffusion. Mitochondrial genetics showed a pathogenic variant in the MT-TL1 gene, confirming mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).

Patient 3: A 39 years-old male developed acute onset aphasia. He had frontal balding and joint hyperlaxity. MRI brain showed acute and chronic infarcts in bilateral middle cerebral artery territories, with intracranial vasculopathy. CES revealed a variant in the ABCC6 gene, suggesting pseudoxanthoma elasticum.

Patient 4: A 25 years-old male presented with recurrent episodes of binocular painless vision loss for 18 months, and behavioural issues. He also had recently detected diabetes, and seizures. MRI showed multifocal gyral swelling and hyperintensity. CSF lactate was elevated. Mitochondrial genetic sequencing showed a pathogenic variant in the MT-TL1 gene, confirming MELAS.

None of the patients had positive family history.

Conclusions

Genetic stroke syndromes are diverse. In the presence of appropriate clinical-radiological features, absence of family history must not deter from genetic testing.

Background and Aims

Spontaneous spinal epidural hematoma (SSEH) is the accumulation of blood in the epidural space which causes spinal cord compression and acute neurological deficits. SSEH is a rare pathology, estimated at 0,1 per 100.000 people per year. The exact etiology of SSEH is still unknown in 40-60% of cases. Prompt diagnosis and predictive factors of post-operative outcome are still undetermined owing to the rarity of this entity.

Methods

case presentation

Results

A 64-year-old female came to the emergency department with sudden weakness in all four extremities since one day before hospital admission. She felt severe throbbing pain followed by numbness in both legs up to the chest. A few hours later, the patient could not move her legs and could not sense her urination. The patient has a history of hypertension but no anticoagulant consumption. There was tetraparesis and pathological reflex was positive bilaterally. The sensory examination showed hypoesthesia at C5-Th3, anesthesia below Th4, and proprioception impairment. There was also urinary and fecal incontinence. MRI showed tubular lesions causing spinal cord compression and myelopathy. The patient underwent surgical decompression laminectomy on day-10 following onset. Tissue obtained from the surgical procedure was clotted lumps of blood. Post-operative neurological dysfunction did not change. After the procedure, the patient was treated in the intensive care unit (ICU) where she had lengthened hospital stay due to comorbidities during treatment.

Conclusions

Discussion: The source of bleeding is commonly from the vertebral venous plexus system. Interval of surgical decompression procedures and previous neurological deficits are the main predictive outcome factors.

Background and Aims

Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG) and laboratory features of patients with this very rare phenomenon.

Methods

We review published English and Japanese literature over the past 25 years, and supplemented by one original case.

Results

The median age of the patients was 71 years (range 56–84 years); 12 were women. In 20 patients CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal weakness (n=13), ataxia (n=9) and aphasia (n=8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while the EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 3 months (range: 19 days–14 months).

Conclusions

This series of cases is reported in order to increase awareness of the possibility that CJD may present in an apoplectic form, which may lead to erroneous management. Since MRI is now more commonly performed in acute stroke, the characteristic ribbon-like cortical signal should indicate the correct diagnosis. While we have focused on the acute onset of symptoms, probably an important red flag should be the progressive clinical deterioration of the patient afterwards. CJD may have an apoplectic onset and clinicians should be alert to this possibility to avoid the consequences of misdiagnosis.

Background and Aims

Eagle syndrome is a rare entity causing recurrent neck pain, sensation of a foreign body in the throat, otalgia, and dysphagia due to the elongated styloid process or calcified stylohyoid ligament. A less common variant, the stylocarotid syndrome, patients can present with symptoms ranging from headache to TIA or stroke due to the elongated styloid process impinging on the internal or external carotid.

Methods

We report a case of a 43-year-old right-handed woman who arrived at the emergency department with blurred vision, diffuse headaches, and left-sided weakness. Head and neck CT angiography showed bilateral distal ICA dissection with severe luminal stenosis and a distal left ICA saccular aneurysm. Revision of the cervical computed tomography angiography showed elongation of approximately 40 mm of both styloid processes. On 3D reformat of cervical CTA visualized both ICAs in the vicinity of the styloid process (as close as 2-3 mm). The patient reported that symptoms reoccur when she performs Pilates training, especially with cervical rotation and hyperextension, suggesting bilateral Eagle Syndrome.

Results

Due to persistent symptoms despite maximal medical treatment (anticoagulation, double antiaggregants), we performed stenting of the right ICA with an excellent clinical outcome.

Conclusions

The elongation of the styloid process may have different paths, compressing the surrounding anatomical structures. CT imaging is an excellent imaging technique in the diagnosis of stylocarotid syndrome. The relationship between an elongated styloid process and adjacent blood vessels can be better established when using three-dimensional reformatting with imaging

Background and Aims

Mevalonate kinase deficiency (MKD) is an autosomal recessive monogenic autoinflammatory diseases characterized by recurrent systemic inflammation attacks with interleukin-1beta hypersecretion, fever and pain. Despite interconnections with inflammation, thrombosis is rare or underreported in autoinflammatory diseases, and causation often undetermined. We report an ischemic stroke patient whose investigation unprecedentedly supports MKD as the cause.

Methods

Case report.

Results

A 39-year-old French-Canadian patient consulted for stroke. He reported hospitalizations nearly monthly since birth for recurrent inflammatory attacks triggered by infections or stress, which lasted 3-7 days and included fever up to 41°C, painful lymphadenopathies, abdominal pain, polyarthralgia and maculopapular rash. Attributed to FMF, attacks kept recurring despite colchicine prophylaxis. His only sister had similar symptoms, but not their non-consanguineous parents or children (n=5). Stroke culminated his most recent inflammatory attack. Brain MRI confirmed acute infarct but showed no chronic ischemic damage. Blood tests documented increased C-reactive protein, amyloid A and immunoglobulinemia-D. Infections were excluded. Prothrombotic and autoantibody tests, cervicocephalic CT-angiography, transthoracic and transesophageal echocardiography, cardiac monitoring, and toxic screen were unremarkable. Gene sequencing identified two heterozygous mevalonate kinase pathogenic mutations (c.803T>C; c.1129G>A). Following treatment with anti-interleukin-1beta monoclonal antibodies, he no longer had inflammatory attacks or stroke in 2 years.

Conclusions

This MKD patient experienced ischemic stroke during an attack, presumably from inflammation-associated endothelial dysfunction/injury and hypercoagulability. Extensive workup excluded other stroke etiologies. Recurrent febrile attacks starting before age 1, gastrointestinal symptoms, painful cervical lymphadenopathies, maculopapular rash, and ineffectiveness of colchicine prophylaxis help differentiating MVK from FMF. Anti-interleukin-1 therapy prevents recurrent autoinflammatory attacks. Thromboprophylaxis remains unproven.

Background and Aims

Reduced systemic perfusion, which is usually caused by cardiac pump failure and systemic hypotension, is a recognized stroke mechanism.

Methods

We report a case with fluctuating level of consciousness due to global hypoperfusion ischemic stroke.

Results

A 90-year-old female patient was admitted to the emergency room with dyspnea, chest pain occurring one week after biliary stenting for treating acute pancreatitis. On arrival, the patient was awake, tachycardia, blood pressure 100/60 mmHg, no focal neurology deficit was recorded. Thirty minutes after admission, her blood pressure decreased to 80/50mmHg and the patient became lethargy, right-side weakness was revealed through examination. Head non-contrast CT scan and CT angiogram didn’t reveal early ischemic lesions or large vessel occlusion. The patient was then given normal saline and noradrenalin to raise blood pressure. She had complete improvement in symptoms when blood pressure returned to normal, and there were two subsequent episodes of impaired consciousness which are also associated with hypotension and recovered completely after blood pressure normalization. For investigating cause of hypotension, CT pulmonary angiogram was performed, recording massive pulmonary embolism. She was treated with intravenous alteplase and vasopressors including noradrenaline and dobutamine. The patient's hemodynamic was stable and vasopressors were stopped after 2 days, and she was discharged with no more focal neurological symptoms.

Conclusions

Acute ischemic stroke due to diminished blood flow can occur in patients with hypotension and present with consciouness fluctuation. Pulmonary embolism is a cause to look for, especially in patients with risk factors for thromboembolism.

Background and Aims

Tumefactive demyelinating lesions, being the radiological mimickers of high‑grade central nervous system neoplasms, often cause a diagnostic dilemma for clinicians as well as radiologists. Sudden onset deficits can mimic stroke and add to diagnostic dilemma. We present a stroke mimic diagnosed later with tumefactive acute disseminated encephalomyelitis (T-ADEM).

Methods

A 60-years-old pleasant lady developed a sudden onset loss of vision in both eyes when she woke up in the morning. She also developed facial deviation to the left side and weakness on the left side of the body. On examination, the patient was conscious but lethargic with left facial palsy and left-sided hemiparesis. Stroke work up was started. MRI brain was suggestive of multiple ill-defined lesions, mainly in the left centrum semiovale, genu and splenium of the corpus callosum on the right side, right gangliothalamic region, and left occipital lobe with diffusion restriction on DWI.

PET scan and CSF results were inconclusive. VEPs showed modest right-sided demyelinating optic neuropathy(152 milliseconds). In view of the diagnostic dilemma, brain lesions were biopsied, which revealed, a foamy macrophage rich lesion suggestive of demyelination.

Results

The patient was diagnosed with Tumefactive ADEM and she was started on Inj Methylprednisolone 1gm IV for 5 days followed by oral steroids over the next few months. The patient improved significantly within a few weeks of treatment.

Conclusions

T-ADEM can be a challenging diagnosis particularly when it presents with stroke-like symptoms. Neuroimaging and biopsy when indicated can help with early diagnosis. Clinicians need to be particularly aware of the entity of tumefactive ADEM.

Background and Aims

Acute promyelocytic leukaemia (APL) is frequently associated, often from the earliest phases, with a life-threatening coagulation/bleeding syndrome; disseminated intravascular coagulation (DIC) is described in the majority of patients. We report a case of a 49-year-old male, without cardiovascular risk factors, who suddenly developed ischemic stroke and splenic infarction as presenting symptoms of APL.

Methods

A 78 years old gentleman with no past medical illness, presented with sudden onset slurring of speech, disorientation and urinary incontinence for the past 2 days. The patient also had a mild right-sided hemiparesis.

Results

MRI brain was suggestive of acute infarcts in the left parietal lobe and right frontal lobe with microbleeds in bilateral frontoparietal regions. Blood work showed anaemia (Hb 8.2 g/dL), a white blood cell (WBC) count of 16500 x10⁹/L of which 91% were atypical promyelocytes, and 14,000 x10⁹/L platelets. The underlying haematological cause was suspected for the stroke. The patient underwent a bone marrow biopsy which demonstrated diffuse infiltration of abnormal promyelocytes, confirming the rare cause of stroke as acute promyelocytic leukaemia (APL)

Conclusions

The management of thrombotic events in APL is a challenge since the increased bleeding risk associated with anticoagulation must be weighed against the potential lethality of the thrombosis if it is left untreated. Central nervous system arterial thrombosis has a particularly poor prognosis. With abnormal blood work up, a high degree of suspicion for underlying malignancy must be maintained for timely diagnosis and treatment.

Background and Aims

Background:

Tandem occlusion is occlusion of the ICA’s extracranial segment and thromboembolism of its intracranial segments. This has low recanalisation rate with intravenous (IV) tissue plasminogen activator (tPA) therapy. Endovascular recanalisation is a viable treatment however, this can be challenging and definitive strategy to minimise peri-procedural complications remains unclear.

Aim:

We report a case series of endovascular recanalisation in 3 patients with tandem ICA/MCA occlusion and the challenges faced in each case.

Methods

Cases:

1) 80-year-old male patient presented with tandem left ICA/MCA occlusion. A balloon angioplasty and aspiration were used.

2) 69-year-old male patient presented with tandem right ICA/MCA occlusion. Balloon angioplasty and aspiration were used. A stent was later deployed.

3) 61-year-old male patient with bilateral ICA occlusion with tandem left MCA occlusion. Balloon angioplasty and aspiration were used. A stent was also deployed.

Results

1) Successful angioplasty of left ICA stenosis. Angioplasty balloon was irretrievable through Benchmark necessitating replacement of Benchmark over an exchange wire. Subsequent successful left MCA revascularisation.

2) Successful recanalisation of right ICA and MCA however, the right ICA re-occluded post-procedure necessitating a stent angioplasty.

3) There was embolisation into anterior cerebral artery following ICA balloon angioplasty. The left MCA was recanalised. The ICA was stented due to re-occlusion and there was embolisation into left MCA following stenting. The stent thrombosed intra-operatively.

Conclusions

There is yet no consensus on optimal management of tandem occlusions. Prioritisation of intracranial revascularisation is paramount and can usually be achieved however, maintaining ICA patency can be challenging.

Background and Aims

Some patients with posterior circulation cerebral infarction can present a variety of involuntary movements that are called "convulsive-like movements". We aim to investigate the clinical phenotype of posterior circulation cerebral infarction presenting with convulsive-like movements and to analyze its pathophysiological mechanisms, and provide reference to the early diagnosis and treatment of the disease.

Methods

Here we report a case of posterior circulation cerebral infarction presenting with convulsive-like movements. Associated literatures were collected from the Pubmed database. Clinical data were statistically analyzed.

Results

A 56-year-old man presented with dizzness and jerks of his right lower limb. These episodes consisted of clonic contractions and shivering of the right lower extremity, last for about 2 minutes. During the visit, the episodes increased in frequency and spread to the four limbs, with loss of consciousness. Magnetic resonance imaging showed diffuse posterior circulation infarction involving cerebellum, pons, thalamus and occipital lobe. Intravenous thrombolysis was started and the patient recovered consiousness.

Literature review consists of 17 articles, including 22 patients. Most of the patients presented with bilateral limb convulsive-like movements accompanied by impaired consciousness. The majority of infarctions were found in thalamus, brainstem and cerebellum, and half of the patients had a poor prognosis.

Conclusions

Posterior circulation cerebral infarction may present with convulsive-like movements. The underlying pathophysiological mechanisms include the rupture of pontine reticular formation, stimulus-induced rhythmic, periodic, or ictal discharges (SIRPIDs) and lesion in the Guillain-Mollaret triangle, but are still to be investigated. Clinicians should identify the symptoms as early as possible to improve the prognosis of the patients.

Background and Aims

Hemichorea is an uncommon initial presentation of stroke, and usually involved in basal ganglia or subthalamic nucleus. We described an extremely rare case of hemichorea due to acute microinfarct in the left middle frontal gyrus, and explored the underlying network connecting middle frontal gyrus and extrapyramidal system.

Methods

The clinical, laboratory, and neuroimaging characteristics of the patient were evaluated.

Results

A 65-year-old man suddenly developed three-day persistent and involuntary movements of the right limbs. The abnormal movement was characterized by uncontrollable, nonrhythmic, nonpatterned, purposeless, and often jerky with variable amplitude and frequency (video). The patient had a medical history of poorly-controlled hypertension and smoking. He had no history of diabetes mellitus or a family history of neurodegenerative disease. He denied use of alcohol or illicit drugs. Neurological examination was normal. The laboratory findings, including blood glucose, routine blood analyses; liver, renal, and thyroid function; ion concentration; and the inflammatory, infectious, and immune biomarkers were unremarkable. Axial DWI demonstrated the restricted-diffusion consistent with acute micrinfarct in the left middle frontal gyrus region, while no abnormal signal was found in basal ganglia or subthalamic nucleus (Figure). Treatment with aspirin, clopidogrel, atorvastatin and clonazepam led to an evident improvement in patient’s symptoms.

Figure Brain MRI

Conclusions

Although extremely rare, our case highlights that isolated cortical infarction can cause hemichorea. The prognosis, especially the rate of disappearance of hemichorea, is better than patients with extrapyramidal lesions. The underlying mechanism is suspected to be hypoperfusion of subcortical regions due to frontal infarctions and therefore interrupt the cortical-ganglionic connections.

Background and Aims

In India, more than 2,000,000 snakebites are reported annually. Russell’s viper is the leading cause of fatal snakebite in India.The clinical manifestations include local reactions and necrosis, renal failure, and coagulopathy complicated by hemorrhagic manifestations such as pituitary and intracranial haemorrhage. There are only few case reports of Ischemic stroke associated with snakebite.

In this case report, we are presenting an unusual complication, cerebral infarction following Russell’s viper bite.

Methods

A 28/male with alleged history of Russels viper bite at his residence presented with right hemiplegia and UMN facial palsy and motor aphasia since 3hours after snake bite envenomation.his cardiovascular and respiratory staus is normal, Investigations showed normal metabolic parameters, twenty-minute whole blood clotting test is positive and is treated with ASV. CT Brain showed no evidence of haemorrhage. MRI Brain showed T2/FLAIR hyperintensities with diffusion restriction in left capsuloganglionic region and left frontal region.

Results

He was treated with anti platelets, statins and heparin, after 14 days of ICU stay he is discharged, his power is 4-/5 at the time of discharge and word output improved compared to the day of admission.

Conclusions

Snake venoms can cause cellular injury through enzymes, polypeptide toxins, cytokines, and mediators leading to cell injury and thrombosis.possible mechanisms include hypercoagulability, hypotension, endothelial injury. Early administration of ASV and timely management of haematologic and metabolic complications and supportive care can prevent the morbidity and mortality.Heparin seems to be having a beneficial role and provides a rational therapy for defibrination caused by viper envenomation.

Background and Aims

Person-in-the-Barrel Syndrome (PIBS) is a condition characterised by brachial diplegia, most commonly as a result of cerebral hypoperfusion with watershed infarcts. The authors’ objective is to present a rare case of PIBS which was caused by bilateral pontine infarct, secondary to basilar artery thrombus.

Methods

A 22-year-old male developed sudden onset frontal headache followed by a transient loss of consciousness and witnessed seizure. After full recovery of his GCS with residual ataxia and slurred speech, he was found unconscious shortly after. CT Head confirmed acute basilar artery thrombus and he subsequently underwent IR-guided mechanical thrombectomy.

Results

Post-thrombectomy CT demonstrated successful reperfusion with a patent basilar artery, accompanied by right posterior parietal and occipital ischaemia. Despite revascularisation, he continued to have persistent bilateral brachial, bulbar and facial weakness with preserved lower limb function - suspicious of PIBS. MRI brain confirmed that he had bilateral pontine ischaemic changes.

Conclusions

To date, there is only one brief neuroimaging case in the literature about PIBS caused by pontine infarction. The is the first case report of such suggesting PIBS as a presentation of acute basilar occlusion syndrome causing pontine ischaemia, due to its antero-lateral territorial supply by the basilar artery.

Background and Aims

Pseudo-abducens nerve palsy is scarcely reported as a manifestation of thalamic strokes. The condition is characterised by impaired contra-lateral abduction despite preserved vestibulo-ocular reflexes. The authors report a rare case of binocular diplopia secondary to thalamic infarct.

Methods

A 53-year-old presented with sudden-onset dizziness, dysarthria and diplopia. His examination showed vertical gaze palsy, right-sided lateral gaze palsy and mild right sided esotropia.

Results

MRI Head revealed high DW1 signal within the medial aspect of the left thalamus in keeping with acute infarction. Bubble contrast echocardiography was strongly positive to suggest intracardiac shunting, suggesting a patent foramen ovale relevant to stroke mechanism. The patient made a gradual recovery with dual antiplatelet therapies.

Conclusions

The authors describe a rare case of thalamic esotropia with supranuclear abducens palsy, due to increased convergence tonicity from the medial rectus following an acute thalamic stroke. The associated vertical gaze paralysis is reportedly attributed to lesion in the rostral interstial nucelus of the medial longitudinal fasciculus.

Background and Aims

Migraine is a chronic neurological disorder, characterized by recurrent headache attacks featuring specific clinical characteristics. It takes the second place among the world’s causes of disability, and is one of the leading causes for direct and indirect government expenses. Migraine is well known to be associated with cerebrovascular diseases, in particular with stroke. Due to the recent studies, the risk of hemorrhagic stroke (HS) is 50% higher in between migraineurs in comparison to non-migraineurs, whereas only one study showed association of migraine without aura (MwtA) with increased HS incidence. We present a case of 57 year old White woman, who is a 20-years migraine sufferer admitted to the hospital with the signs of migraineous stroke.

Methods

Upon evaluation after she had contrast enhancing brain MRI, an intracerebral neoplasm accompanied by a hemorrhage was suspected. However, the following MR spectroscopy as well as repeated contrast MRI didn`t prove the diagnosis.

Results

The patient was treated for two weeks, and discharged with complete clinical improvement.

Conclusions

The presented case highlights the importance of the accurate evaluation of the patients, suspicious of complicated migraine, even though suffering from MwtA, having no comorbidities and absent family history of cerebrovascular diseases.

Background and Aims

Delirium is characterised by impaired orientation, diminished attention and aberrant perception. It is a recognised complication following an acute stroke. However, it can present a diagnostic challenge when it is the main presenting feature as there are a myriad causes of delirium.

We present three interesting cases that highlight the neuro-psychiatric complications following infarcts of key sub-cortical structures: thalamus, internal capsule and basal ganglia.

Methods

Case 1

58-year-old lady presents with acute confusion (disoriented in time, place and person). Main risk factors were hypertension and hypercholesterolaemia. MRI confirmed infarct in the left thalamus and internal capsule.

Case 2

74-year-old lady presents with three days history of confusion, drowsiness and short-term memory loss. Risk factors were hypertension and hypercholesterolaemia. MRI confirmed right internal capsule infarct.

Case 3

58-year-old lady admitted with confusion (did not recognise family members). Her risk factors were diabetes, hypertension, hypercholesterolaemia and smoking. MRI confirmed infarct in the left thalamus and basal ganglia.

Results

All three cases show that delirium can be the only observable presenting feature in certain types of strokes.

Conclusions

Clinicians need to consider stroke as a differential in patients who present with acute confusion particularly those with cardiovascular risk factors where no other cause is apparent. These cases augment the growing body of evidence of the complex neuro-psychiatric syndromes that can ensue following thalamic, internal capsule and basal ganglia infarcts.

The mechanism of delirium in this context is likely due to disruption of projecting fibres to the frontal lobe.

Background and Aims

Malignant neoplasm is one of the important cause of cerebral infarction, which is associated with cancer-associated hypercoagulability. Uterine adenomyosis, known as a benign gynecological disease, can be an uncommon cause of cerebral thromboembolism.

Methods

Single case study.

Results

A 49-year-old woman with uterine adenomyosis shows four attacks of cerebral infarction for 8 years, despite antiplatelet or anticoagulation treatment. Blood tests demonstrates elevated levels of D-dimer and mucinous tumor markers such as CA125, CA19-9 during acute period of attacks.

Conclusions

We will discuss the stoke mechanism and treatment of this under-recognized etiology of stroke.

Background and Aims

Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare inherited disorder that results in waxing and waning nervous system and muscle dysfunction.

Methods

We present the case of a 28-year-old male with recurrent episodes of stroke-like symptoms, a strong family history of hearing loss and diabetes mellitus. He also developed seizure in one of the presentation. Typical Imaging finding with nonterritorial infarction with normal vessels and MR spectroscopy(MRS) showing increased lactate was clue to diagnosis of stroke like episodes . Molecular Genetic Analysis revealed the presence of the MELAS A3243G mitochondrial mutation in a heteroplasmic state which conformed the diagnosis of MELAS. He was managed with supportive medications, Insulin for diabetes and antiepileptic medications. We discuss the imaging features distinctive to the case, the mechanism of the disease, typical presentation, imaging diagnosis, and disease management.

Results

Patient was sucessfully treated with supportive therapy along with antiepileptic medicine and made a gradual recovery however the sensory aphasia and cognitive problem persisted.

Conclusions

MELAS is an important diagnostic consideration when neuroimaging reveals a pattern of disappearing and relapsing cortical brain lesions that may occur in different areas of the brain and are not necessarily limited to discrete vascular territories. Medications which can increase the lactic acidosis has to be avoided and strong degree of suspicion has to be considered to avoid unnecessary therapeutic and diagnostic errors.

Background and Aims

Lymphoma-associated hemophagocytic syndrome (LAHS) is a life-threatening disease with poor prognosis and may present as ischemic stroke. We report a case of a 56-year-old female with recurrent multi-territory infarcts caused by diffuse large B-cell lymphoma with secondary hemophagocytic lymphohistiocytosis.

Methods

The patient had been diagnosed with ischemic stroke and hemophagocytic syndrome probably secondary to Epstein-Barr virus (EBV) infection three months previously and treated with Dexamethasone and Aspirin. High resolution vessel wall magnetic resonance imaging (HR-vwMRI) showed vessel wall thickening at the left PCA P1 segment and the right MCA M2 segment relevant to the cerebral infarction suggesting vasculitis. CT scan revealed splenomegaly, multiple bilateral small nodules of the lung, multiple liver lesions, multiple bilateral renal masses, gastric wall thickening and multiple nodules in the omentum. Cerebrospinal fluid cytology showed increased cerebrospinal-fluid protein level. Hemophagocytosis was showed on bone marrow aspirate cytology. Gastric tissue biopsy revealed large B cell lymphoma.

Results

Chemotherapy was not given because the patient had severe pneumonia and sepsis. The patient died 28 days after the definitive diagnosis was confirmed.

Conclusions

Recurrent ischemic stroke in our patient with diffuse large B-cell lymphoma may be due to vasculitis or IVBCL. Although being a rare disease, our case should raise the alertness for consideration of IVBCL as a differential diagnosis.When diagnosis of IVBCL is doubtful, we propose early brain biopsy or random skin biopsies, even without clinically apparent cutaneous involvement.

Background and Aims

Persistent trigeminal artery (PTA), is the most common of the embryonic arteries that connect the internal carotid artery with the vertebrobasilar system. We present a case of a patient presenting with “top-of-the-basilar” syndrome secondary to an occlusion of the internal carotid artery and presence of PTA.

Methods

A 62 year old male patient with a past medical history of dyslipidemia and hypertension presented with a altered sensorium and weakness of all four limbs with unsteadiness. CT angiography of the head showed severe basilar stenosis and a PTA. The patient received recombinant tissue plasminogen activator (rtPA) and was taken for cerebral angiogram (DSA) and clot retrieval.

Cerebral angiogram revealed a complete occlusion of the petrous segment of the right internal carotid artery , with an anatomical variant of PTA and a distal basilar thrombus. He underwent mechanical thrombectomy using solitaire and achieved TICI 2b recanalization.

Results

Embolic strokes have been reported in association with a persistent trigeminal artery .They can be managed well with recombinant tissue plasminogen activator and mechanical thrombectomy as in this case.

Conclusions

Understanding variant anatomy when selecting patients for thrombolysis and thrombectomy in patients presenting with stroke syndromes helps in better management of patients.

Background and Aims

Though being a small and a thin artery, the Anterior Choroidal Artery with its multiple perforating branches supplies an important area of the brain. This area includes the posterior limb of the internal capsule, optical tract, lateral geniculate body, medial temporal lobe, and medial area of the pallidum. The Anterior Choroidal Artery Syndrome highlights the triad of contralateral hemiplegia, hemianopia and hemianesthesia.

Methods

A forty-three years old Navy officer with past history of poorly controlled hypertension and dyslipidemia presented with an acute onset of reduced vision in the both eyes specifically noted when looking at the left side for one day. He was conscious and alert with normal cognition. He had left homonymous hemianopia but, there were no other cranial nerve palsies. He didn’t have any limb weakness, numbness or cerebellar signs. His systemic examination was normal except for an elevated blood pressure of 160/100mmHg.

Results

Non contrast CT of the patient revealed a hypodensity in the right lateral thalamus and adjacent optic radiation. This was confirmed by the hyperintensity in MRI brain FLAIR images in the same respective area with diffusion restriction in DW images and corresponding changes in ADC maps. Patient had atherosclerotic changes in Carotid vessel imaging but the echocardiogram was normal.

Conclusions

The classic triad of anterior choroidal artery syndrome occurs only in the complete forms. Incomplete forms with lacunar syndromes, ataxic hemiparesis are much more commoner when the syndrome is concerned. This is due to the adequate collateral supply by the posterior and middle cerebral arteries.

Background and Aims

CADASIL is a rare hereditary vasculopathy caused by missense mutations of the NOTCH3 gene. The clinical expression is variable, dominated by migraines, ischemic strokes and psychiatric disorders in particular depression.

Methods

35-year-old lady gravida 4 para 3 at 9 weeks pregnancy with underlying history of migraine presented with recurrent strokes. There was no family history of stroke. Clinically, her motor power on the left side was 4/5 with mild left UMN facial palsy. The differentials considered were sporadic small vessel disease, cerebral venous thrombosis, vasculitis and CADASIL. Her basic bloods, ECG and ECHO was normal.. Vasculitis and procoagulant screening were negative. Genetic study for NOTCH 3 mutation was sent.

Results

Non-contrasted MRI showed acute infarcts in the right MCA territory, with an older infarct in the right frontal lobe and no evidence of cerebral venous sinus thrombosis. The large to medium sized cervical and intracranial arteries are patent with normal caliber. The rs1044009 which confers missense mutation (c.6668C>T) with an amino acid change (p.Ala2223Val) was detected. Therefore, the diagnosis of CADASIL was confirmed with the missense mutation of rs1044009 in this patient. She was started on tablet aspirin 150 mg daily.

Conclusions

This case is being presented in view of its rarity. Secondly in young patients with stroke and concomitant history of migraine attacks, the possibility of CASADIL must be considered.

Background and Aims

Stroke can also present as acute onset, abrupt abnormal movements besides typical stroke symptoms. We here present a patient, who suddenly noticed abnormal twitching movements of the left cheek and upper lip as if his left side of mustaches were jumping.

Methods

A 52-year non-diabetic, non-hypertensive male presented in emergency with abrupt onset right facial twitching one day back. This twitching of the face was limited to the right cheek and angle of the mouth, and was continuous, even during sleep. In the patients’ words, he felt as if his right face started flickering and the right half of his mustaches started jumping abruptly all of a sudden. He had blood pressure180/90-mmHg with no motor or sensory weakness of any limbs.

Results

MRI brain of the patient showed diffusion restriction in the left precentral gyrus suggestive of acute ischemic stroke. Echocardiography and cerebral angiography were normal. Diagnosis of facial epilepsia partialis continua (EPC) following acute ischemic stroke was considered due to focal continuous jerky movements of the right face, possibly resulting from the lesion in the left precentral gyrus, confirmed by epileptiform discharges in electro-encephalography. The patient was started on anti-epileptic drugs, levetiracetam and lacosamide along with aspirin, following which twitching subsided completely over 3-4 days.

Conclusions

EPC can be caused by multiple etiologies including stroke. In this case, right facial EPC were the presenting features of stoke and they lasted for over 3-4 days. In our patient, the left precentral gyral ischemic lesion may be the possible culprit cortical generator leading to facial EPC.

Background and Aims

Stroke can result in a wide array of neurolinguistic disorders, the most common of which include the aphasias. However, other disorders such as verbal apraxia may closely mimic aphasia, but are of different therapeutic and prognostic significance. The authors report a case of acute ischemic stroke with phenomenology of aphemia, a rare and severe form of verbal apraxia.

Methods

A case report.

Results

A right-handed 49-year-old woman presents with right-sided hemiparesis predominantly affecting the upper extremity with central right-sided facial and hypoglossal nerve paresis since 2.5 hours preceding admission. Upon examination, she seemed aphasic but otherwise alert. A bedside neurolinguistic assessment revealed deficits in verbal fluency with retention of auditory comprehension and writing capacity, consistent with aphemia. Head MRI revealed a hyperacute infarction of the left insular cortex (Figure 1). Thrombolytic therapy was administered. Physical rehabilitation and speech therapy was initiated, and the patient was discharged after 8 days of care with dramatic improvement of motor functions and partial improvement of aphemia.

Conclusions

In contrast to motor aphasia, aphemia constitutes a disorder of articulation characterized by complete failure of speech in the presence of preserved comprehension and writing ability. It is typically caused by lesions of the inferior precentral gyrus. While its clinical presentation may be similar, preserved skills in written communication readily discerns aphemia from motor aphasia, in which writing is affected in proportion to speech. It is crucial to discern aphemia in order to plan the appropriate rehabilitation strategies.

Background and Aims

Giant cell arteritis (GCA) is an immune-mediated systemic vasculitis usually seen in elderly women.

Methods

We describe two 76-year-old women who presented with jaw claudication and temporal headache. Biopsy of the temporal artery confirmed GCA. They were treated with oral prednisolone.

Results

Both women subsequently presented with acute unilateral visual loss and limb weakness within six months of their GCA diagnosis. Their magnetic resonance brain scans demonstrated acute bilateral multi-territory infarcts predominantly affecting posterior circulation. Both patients demonstrated features of cardio-embolic and vasculitic origins of stroke. Our first patient presented with persistent fast atrial fibrillation with vasculitic and atherosclerotic changes noted in a subsequent computed tomography angiogram. Our second patient suffered an in-hospital cardiac arrest, and following return of spontaneous circulation, coronary angiogram and echocardiogram demonstrated coronary artery and left ventricular thrombus. Both patients unfortunately continued to deteriorate and passed away within a week.

Conclusions

Coronary artery and intraventricular thrombosis and GCA are risk factors for cerebrovascular accidents. The presence of pre-existing atherosclerotic risk factors further poses a diagnostic dilemma for physicians as GCA requires a low threshold of suspicion and early treatment with corticosteroids to improve prognosis. Although a temporal artery biopsy remains to be the definitive diagnostic modality for GCA, the use of radiological investigations in the diagnosis of GCA is increasingly common. A duplex ultrasonography of the temporary artery could be used to assess GCA in highly-suspected patients. Echocardiograms and contrast-enhanced body imaging should be performed in patients with suspected or established GCA to assess secondary thromboembolic and vascular diseases.