A Diagnostic Dilemma of White Matter Lesions and Cerebral Oedema without Identifiable Cause-A Neurological Conundrum.

INTRODUCTION: This paper describes a case of bi-frontal vasogenic oedema associated with bilateral frontal lobe and left parietal lobe white matter lesions where extensive investigations, including brain biopsy, failed to establish a diagnosis. CASE REPORT: A 67-year-old female presented with three weeks' history of memory loss, fatigue, insomnia, nausea, and occasional dysphasia. Physical examination was unremarkable, yet cerebral CT and MRI showed bilateral frontal lobe vasogenic oedema. Extensive investigations, including: biochemical; radiological; immunological; microbiological; haematological; histopathological; and cytological, failed to establish a confirmed diagnosis. A multidisciplinary team could not achieve a consensus for this atypical presentation. Brain biopsy was unusual, showing destructive inflammatory and subtly granulomatous disease, but an exhaustive list of auxiliary tests could not confirm a cause, and consensus favoured glial fibrillary acidic protein (GFAP) autoimmune encephalopathy. DISCUSSION: A definitive diagnosis could not be established for this patient despite a gamut of investigations. Although some of the presenting features were consistent with GFAP astrocytopathy, initial staining of the patient's CSF for neuronal antibodies was negative. Her symptoms and radiological changes of brain imaging improved without any corticosteroid therapy. CONCLUSIONS: Through this case report, the aim is to add to the repository of neurological sciences in the hope that future similar presentations could potentially lead to discovery of a new aetiology or contribute towards better understanding of an existing disease process.

Cerebral venous thrombosis as a complication of intracranial hypotension after lumbar puncture.

BACKGROUND: Optic neuritis is recognised by the international classification of headache disorders as a painful cranial nerve lesion. A lumbar puncture may be performed in the investigation of optic neuritis. Postdural puncture headache (PDPH) due to intracranial hypotension is a frequent complication of this procedure. In contrast, cerebral venous thrombosis (CVT) is a rare but potentially fatal complication of dural puncture. A few studies have identified an association between iron deficiency anaemia and venous thrombosis. There are no reports linking CVT with lumbar puncture and iron deficiency anaemia. METHODS AND RESULTS: We present a 32-year-old woman with optic neuritis and iron deficiency anaemia complicated by a PDPH and CVT. CONCLUSION: CVT should be considered in a patient with persistent headache, recent lumbar puncture and iron deficiency anaemia. Early recognition and treatment of this condition are vital to avoiding mortality and morbidity.

Frequency, Aetiology, and Outcome of Small Cerebellar Infarction.

BACKGROUND AND PURPOSE: Strokes due to small (<2 cm) cerebellar infarction are under-recognised, and their profile and aetiology have not been well characterised. We aimed to determine the frequency, clinical features, aetiology, and outcome of small as compared to large cerebellar infarction. METHODS: This study is a retrospective analysis of clinical and imaging features of a prospectively assessed series of 108 consecutive patients with acute cerebellar infarction admitted to Liverpool Hospital, Sydney, NSW, Australia, during 2011-2015. RESULTS: The mean age of the patients was 67 years, and 33 (31%) had small cerebellar infarction. Compared to large cerebellar infarction, those with small cerebellar infarction had a comparable distribution of vascular risk factors but significantly less nausea and vomiting, gait disturbance, limb ataxia, and dysarthria. The posterior (n = 22, 67%) lobe was most commonly affected, followed by the anterior (n = 9, 27%) and flocculonodular (n = 2) lobes. Dizziness, limb ataxia, and nystagmus were significantly more common in patients with anterior lobe infarction. Vertebrobasilar disease was the presumed aetiology in 40 patients (37%), and was less commonly seen in small as compared to large cerebellar infarction. Cardioembolism affected 37% of the patients, irrespective of the size or topography of the cerebellar infarction, and there was no relation of supratentorial white matter lucencies (WMLs) to the size of cerebellar infarction. At 3 months, 65% of the patients were functionally independent (according to modified Rankin Scale scores of 0-2), and having a poor outcome was significantly related to moderate-to-severe supratentorial WML and large cerebellar infarction. CONCLUSIONS: Small cerebellar infarction accounted for one-third of the ischaemic strokes in this location, most often involved the posterior lobe, causing fewer clinical features, and had a better clinical outcome than large cerebellar infarction. Patients with small cerebellar infarction require appropriate vascular management including investigation for a cardioembolic source.

Relationship between intracranial pressure and phase contrast cine MRI derived measures of intracranial pulsations in idiopathic normal pressure hydrocephalus.

Phase contrast cine MRI with determination of pulsatile aqueductal cerebrospinal fluid (CSF) stroke volume and flow velocity has been suggested to assess intracranial pulsations in idiopathic normal pressure hydrocephalus (iNPH). We aimed to compare this non-invasive measure of pulsations to intracranial pressure (ICP) pulse wave amplitude from continuous ICP monitoring. We hypothesised that a significant correlation between these two markers of intracranial pulsations exists. Fifteen patients with suspected iNPH had continuous computerised ICP monitoring with calculation of mean ICP pulse wave amplitude (MWA) from time-domain analysis. MRI measured CSF aqueductal stroke volume and peak flow velocity. Mean MWA was 5.4mmHg (range 2.3-12.4mmHg). Mean CSF stroke volume and peak flow velocity were 65µl (range 3-195µl) and 9.31cm/s (range 1.68-15.0cm/s), respectively. No significant correlation between the invasive and non-invasive measures of pulsations existed (Spearman r=-0.30 and r=-0.27, respectively; p>0.05). We observed marked intra-individual fluctuation of MWA during continuous ICP monitoring of an average of 6.0mmHg (range 2.8-12.2mmHg). The results suggest a complex interplay between measures of pulsations derived from snapshot MRI measurements and continuous computerised ICP measurements, as no significant relationship existed in our data. Further study is needed to better understand the temporal profile of CSF MRI flow studies, as substantial variation in MWA over the course of several hours of ICP monitoring is common, suggesting that these physiologic fluctuations might obscure MRI snapshot measures of intracranial pulsations.

Delayed angiographic changes in postpartum cerebral angiopathy.

Postpartum cerebral angiopathy is a well-recognised subgroup of the reversible vasoconstriction syndromes. Increasingly described is a delay between clinical onset and angiographic changes. We report a patient who presented 19 days postpartum with severe thunderclap headaches, vertigo and a seizure. The clinical syndrome preceded evidence of extensive vasoconstriction on MRA imaging by 15 days; the changes were reversible at 3 months.

The prevalence and clinical significance of (18) F-2-fluoro-2-deoxy-D-glucose (FDG) uptake in the thyroid gland on PET or PET-CT in patients with lymphoma.

F(18) -2-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) has become a well established tool in staging and assessing therapy response in lymphoma. Incidental thyroid uptake on PET is not uncommon and can pose a diagnostic and management challenge. We retrospectively evaluate the prevalence and clinical significance of incidental FDG uptake in the thyroid gland in patients with lymphoma. 1868 lymphoma patients underwent PET and PET-CT between August 2002 and August 2008. 52 patients (2.8%) demonstrated FDG thyroid uptake (M = 17, F = 35; mean age 63 yr). Thyroid uptake was determined as focal or diffuse, maximum standardized uptake values (SUVmax) recorded as well as SUV max ratio compared to background mediastinum activity (SUVR). Corresponding CT findings on PET-CT were evaluated independently. Results were correlated with clinical, histopathological and imaging follow-up. 30 (1.6%) patients had focal thyroid uptake. 16 (53%) had histological confirmation either by surgery (n = 7) or FNA under USS (n = 9). The final diagnosis was benign in 12/30 patients and malignant in 9/30. The malignancy risk for focal thyroid uptake was 30%. Five patients had intercurrent thyroid cancer (four papillary, one microinvasive follicular) and four had lymphomatous involvement. There was no significant difference between the mean sizes of benign (23.7 mm, range 12-34) and malignant nodules (23.6 mm, range 8-48). The mean SUVmax of malignant and benign nodules was 4.4 (range 1.8-10.1) and 3.2 (range 1.8-6.9) respectively with no statistically significant difference. 22 (1.2%) patients had diffuse FDG uptake in thyroid and benign aetiology was found in all with adequate follow-up (15/22). Focal FDG thyroid uptake on PET or PET-CT in lymphoma patients warrants further investigations. The malignancy risk is 30% either due to intercurrent thyroid cancer or lymphomatous involvement. SUVmax, SUVR and CT attenuation characteristics are not helpful in distinguishing between benign and malignant aetiologies. Diffuse thyroid uptake has a benign aetiology.

Renal vein Doppler sonography of obstructive uropathy.

OBJECTIVE: Obstructive uropathy in the early stages can be difficult to diagnose using either standard sonography or the arterial resistive index. We tested the hypothesis that acute obstruction of the renal collecting system reduces the intraparenchymal renal compliance, which affects the intraparenchymal venous blood flow to a greater degree than the arterial flow. SUBJECTS AND METHODS: Twelve patients with clinical evidence of acute obstructive uropathy were referred for helical CT to confirm the diagnosis and to provide a gold standard by which we could evaluate the sonographic findings in the 12 test patients. Twelve patients without renal disease served as a control group. Doppler sonography of the interlobar arteries and veins of both kidneys then was performed, with the sonographer unaware of which kidney had an obstruction. Peak venous flow measurements and arterial resistive and venous impedance indexes were obtained. The impedance indexes of the obstructed and unobstructed kidney were compared for each patient. RESULTS: The mean arterial resistive indexes of the obstructed kidneys were larger than those of the unobstructed kidneys, 0.67 +/- 0.08 and 0.62 +/- 0.05, respectively (p = 0.05). The venous impedance indexes comparing obstructed and unobstructed sides were 0.38 +/- 0.25 and 0.80 +/- 0.25, respectively, a statistically significant result (p = 0.0002). On average, the peak venous flow signal in the obstructed kidney was 69% higher than that of the unobstructed kidney (p = 0.04) and 86% higher than that of the peak venous flow signal in the control group (p = 0.005). CONCLUSION: Renal obstruction alters the venous flow to a greater extent than the arterial flow, and a comparison between the venous flow in the obstructed and unobstructed kidneys may improve diagnostic accuracy.