Choong Kun Ha

Different uptake of 99mTc-ECD and 99mTc-HMPAO in the same brains : analysis by statistical parametric mapping

The purpose of this study was to investigate the differences between technetium-99m ethyl cysteinate dimer (99mTc-ECD) and technetium-99m hexamethylpropylene amine oxime (99mTc-HMPAO) uptake in the same brains by means of statistical parametric mapping (SPM) analysis. We examined 20 patients (9 male, 11 female, mean age 62±12 years) using 99mTc-ECD and 99mTc-HMPAO single-photon emission tomography (SPET) and magnetic resonance imaging (MRI) of the brain less than 7 days after onset of stroke. MRI showed no cortical infarctions. Infarctions in the pons (6 patients) and medulla (1), ischaemic periventricular white matter lesions (13) and lacunar infarction (7) were found on MRI. Split-dose and sequential SPET techniques were used for 99mTc-ECD and 99mTc-HMPAO brain SPET, without repositioning of the patient. All of the SPET images were spatially transformed to standard space, smoothed and globally normalized. The differences between the 99mTc-ECD and 99mTc-HMPAO SPET images were statistically analysed using statistical parametric mapping (SPM) 96 software. The difference between two groups was considered significant at a threshold of uncorrected P values less than 0.01. Visual analysis showed no hypoperfused areas on either 99mTc-ECD or 99mTc-HMPAO SPET images. SPM analysis revealed significantly different uptake of 99mTc-ECD and 99mTc-HMPAO in the same brains. On the 99mTc-ECD SPET images, relatively higher uptake was observed in the frontal, parietal and occipital lobes, in the left superior temporal lobe and in the superior region of the cerebellum. On the 99mTc-HMPAO SPET images, relatively higher uptake was observed in the medial temporal lobes, thalami, periventricular white matter and brain stem. These differences in uptake of the two tracers in the same brains on SPM analysis suggest that interpretation of cerebral perfusion is possible using SPET with 99mTc-ECD and 99mTc-HMPAO.

Primary leptomeningeal lymphoma with long-term survival : a case report

Primary leptomeningeal lymphoma (PLML) is a rare disease. The most common presentation is symptoms of increased intracranial pressure. Confusion, dysarthria, hearing loss, paraparesis and lumbosacral spinal root symptoms have also been reported. Chemotherapy and radiotherapy have been tried, but its prognosis is usually poor. We experienced a case of PLML with a relatively benign course in an 18-year-old girl. Initial diagnosis was made as idiopathic intracranial hypertension. Lumbosacral shunt was done with good response for 3 years. When headache recurred, she was reevaluated and was correctly diagnosed as PLML.

Plasma Total Homocysteine Levels are not Associated with Medial Temporal Lobe Atrophy, but with White Matter Changes in Alzheimer's Disease

Background and purpose Elevated plasma total homocysteine (tHcy) levels are reported to be associated with an increased risk of Alzheimers disease (AD). However, the mechanism by which homocysteine contributes to the pathogenesis of AD is as yet unknown. The aim of this study was to elucidate the relationship between white matter changes (WMC) and medial temporal lobe atrophy (MTA) on brain magnetic resonance imaging (MRI), and plasma levels of tHcy in AD patients. Methods Seventy-two patients with a clinical diagnosis of probable AD were recruited to the study. Plasma tHcy levels, vascular risk factors, and WMC and MTA on brain MRI were evaluated in all patients. The AD patients were classified into two groups: those with no or minimal WMC (69.2±8.8 years, mean±SD, n=36) and those with moderate-to-severe WMC (74.6±4.6 years, n=36) on brain MRI. Results In a univariate logistic regression analysis, the risk of moderate-to-severe WMC in AD was significantly associated with increasing age, female gender, lower education level, hypertension, high plasma tHcy levels, and lower Mini-Mental State Examination (MMSE) score. Multivariate logistic regression analysis revealed only high plasma tHcy as the independent and significant risk factor for moderate-to-severe WMC [odds ratio (OR; adjusted for age, gender, education level, MMSE score, and hypertension comparing the top tertile - tHcy levels ≥12.9 µmol/L - with the bottom tertile - tHcy levels ≤9.4 µmol/L)=7.35; 95% confidence interval, confidence interval=1.36-39.84; p=0.02], and age as a borderline significant risk factor (OR=1.08, 95% CI=0.99-1.19, p=0.09) in AD patients. Plasma tHcy levels were not correlated significantly with either right or left MTA. Conclusions Our results suggest that the vascular pathway mediates the association between elevated plasma tHcy levels and AD.

Hot Bath-Related Headache Controlled by Topiramate

Hot bath-related headache (HBRH) was described only recently, and has been rarely reported in the literature. Typical HBRH is provoked by pouring hot water on oneself or soaking in a hot bath, and lasts from 10 min to several hours (1–4). The headache develops rapidly and reaches a peak level of pain within a minute (1–4). HBRH has a bursting and pulsatile character and is seldom accompanied by nausea, vomiting, photophobia or phonophobia (2–4). Most HBRH attacks last up to several weeks and disappear spontaneously. Some HBRH is associated with cold stimulus headache (CSH), whereby headache is provoked by a cold stimulus (1, 3). The pathogenic mechanism of HBRH has not yet been established. Nimodipine, amitryptiline and valproate are known therapeutic options if symptoms do not improve spontaneously (2, 3, 5, 6). In this report, we describe the case of a woman who was relatively young at the onset of HBRH, with a long symptomatic period of typical HBRH accompanied by CSH and a dramatic response to oral topiramate.

Investigation of Common Mitochondrial Point Mutations in Korea

Abstract: Between 1997 and 2002, 65 patients with suspected mitochondrial diseases were screened for the mitochondrial point mutations A3243G, T3271C, A8344G, and T8356C. Among these patients, 15 were found to have one of these mutations: 12 with A3243G and 3 with A8344G. The phenotypes of A3243G and A8344G mutations were MELAS and MERRF, respectively. Many asymptomatic family members had the same mutations. In this report, detailed clinical and laboratory findings are presented.

A more basal approach in microvascular decompression for hemifacial spasm: the para-condylar fossa approach.

Summary Background. In view of the fact that a basal craniectomy in microvascular decompression (MVD) for hemifacial spasm (HFS) can minimize cerebellar retraction and expose the facial nerve root exit zone (FNREZ) directly from below without placement of tension on the seventh-eighth cranial nerve complex, we used a more basal approach in 32 patients with typical HFS. Method. A slightly curved skin incision 5 cm in length and 2 cm posterior and parallel to the mastoid notch was made. The basal lateral occipital plate including the lateral one-fourth of the condylar fossa and the posterior one-fourth of the jugular process were removed. For the early drainage of cerebrospinal fluid through a small dural hole, the basal occipital plate posteromedial to the condylar fossa was removed. With this basal craniectomy, minimum elevation of the cerebellar tonsil and flocculus could expose FNREZ safely. Findings. Thirty one of 32 patients displayed complete disappearance of spasm following surgery. One patient showed 70% decrease of spasm. Delayed transient facial weakness occurred in one patient. Audiometries showed no postoperative hearing decrease in any patient, even though no intra-operative monitoring of the cochlear function was undertaken. Interpretation. Although this basal approach, the para-condylar fossa approach, is a slightly basal modification of the conventional procedure, it may minimize complications.

Neuro-Behçet's Disease Presenting as Hypertrophic Pachymeningitis

A 25-year-old man presented with blurred vision and chronic headache. His brain MRI revealed bilateral frontal pachymeningeal enhancement with leptomeningeal enhancement. The patient had experienced recurrent oral ulcer and had anterior uveitis and papulopustules skin lesion. We diagnosed him with hypertrophic pachymeningitis (HP) associated with neuro-Behçets disease (NBD). There have been few reports describing HP in patients with NBD. We report a case of NBD presenting as HP.

Syndrome of inappropriate antidiuretic hormone secretion associated with pramipexole in a patient with Parkinson's disease.

The syndrome of inappropriate antidiuretic hormone secretion (SIADH) can be caused by a variety of drugs. Dopaminergic drugs might enhance the secretion of the antidiuretic hormone arginine vasopressin by reducing γ-amino butyric acid release through the dopaminergic receptor in supraoptic nucleus. A 75-year-old woman with Parkinson’s disease developed asthenia, delirium, aggravated parkinsonian symptoms, and hypotonic hyponatremia along with the diagnostic criteria for SIADH during dose escalation of pramipexole. After pramipexole withdrawal, these symptoms disappeared, and sodium levels returned to normal values. The serum sodium levels of patients receiving pramipexole should be monitored, especially during dose escalation.

A confusing case of multiple sclerosis and central nervous system graft versus host disease

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Acute chorea in posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome (PRES) is a characteristic syndrome caused by reversible cerebral edema due to dysfunction of the cerebrovascular blood– brain barrier unit [2]. The major symptoms are clinical seizures, encephalopathy, visual symptoms, and headache, in combination with a typical brain magnetic resonance imaging (MRI) pattern of high signal intensity on T2-weighted images predominantly in the posterior regions [5]. After the first case series described as posterior reversible leukoencephalopathy in 1996 [3], numerous case reports, series and imaging studies have been published, including those with atypical features. However, to our knowledge, acute chorea as the presenting symptom of PRES has not been reported so far. We describe a patient with end-stage renal disease (ESRD) on hemodialysis who presented with acute chorea associated with bilateral basal ganglia lesions as an atypical feature of PRES. A 50-year-old man visited to emergency department with an acute involuntary movement of ‘‘dance-like’’ feature after hemodialysis. He had a history of hypertension, diabetes and ESRD on dialysis for last 3 years (switched from peritoneal dialysis to hemodialysis 1 month ago). His initial blood pressure ranged from 178/91 to 210/110 mmHg, other vital signs were within normal range. He showed dysarthria and choreic movement of bilateral upper and lower extremities. He could not walk without assistance due to exaggerated steps with a dance-like quality. The rest of neurological examination was normal. His initial laboratory data were clinically insignificant except elevated levels of blood urea nitrogen (29.0 mg/dL) and serum creatinine (6.11 mg/dL). Cerebrospinal fluid analysis was normal, excluding mildly elevated protein level of 59 mg/dL. Electroencephalography did not display epileptiform discharges nor ictal rhythm. Brain computed tomography revealed symmetric low attenuation of bilateral basal ganglia. Brain MRI demonstrated symmetric hyperintensity of bilateral basal ganglia, frontal and occipito-parietal lobes on fluid-attenuated inversion recovery (FLAIR), T2and diffusionweighted images with high apparent diffusion coefficient (ADC) values, suggesting vasogenic edema (Fig. 1a–d). Gadolinium-enhanced MRI was not performed because of advanced renal failure. Brain magnetic resonance angiography was unremarkable. His neurologic symptoms improved gradually after conservative management and blood pressure control (target range of systolic blood pressure of 140–150 mmHg and diastolic blood pressure of 80–90 mmHg). Follow-up MRI after 10 days of onset of symptom, showed markedly decreased high signal intensity of the lesions (Fig. 1e–h). He discharged with only subtle dysarthria without chorea after 2 weeks of admission. Although there are reported cases of PRES with atypical neuroimaging features involving basal ganglia [5, 6], acute chorea as the presenting symptom with basal ganglia lesions in PRES has not been previously reported. Chorea is an irregular hyperkinetic involuntary movement originating from a dysfunction of neuronal networks interconnecting basal ganglia and the motor cortical area [1]. Among the various conditions related to chorea, there are rare case reports of acute chorea with bilateral basal ganglia lesions in patients with ESRD on dialysis, without an J.-J. Park J.-H. Ahn C.-K. Ha E.-K. Bae (&) Department of Neurology, Inha University Hostpital, 7-206, 3-Ga, Sinheung-Dong, Jung-Gu, Incheon 400-711, Republic of Korea e-mail: alchemist0210@gmail.com