Sun J. Chung

Syndrome of cerebral spinal fluid hypovolemia Clinical and imaging features and outcome

Objective: To investigate clinical, MRI, and radioisotope findings and therapeutic outcome of the syndrome of CSF hypovolemia. Methods: Retrospective review was performed of 30 consecutive patients (10 men, 20 women; mean age 37 years) with the syndrome of CSF hypovolemia. Results: All patients had an orthostatic headache, which was alleviated to a variable extent on recumbency. Additional clinical symptoms included nausea, dizziness, neck stiffness, blurring of vision, tinnitus, plugged ear, hearing difficulties and radicular pain of the arm. Eighty-two percent of the patients had CSF opening pressure less than 60 mm H2O, 59% had CSF pleocytosis, and 95% had increased CSF protein. Brain MRI showed diffuse pachymeningeal gadolinium enhancement on T1-weighted image in 83%, which was seen as hyperintense signals on T2-weighted imaging. Other features included subdural hematoma/hygroma in 17% and descent of the brain in 48% of the patients. Radioisotope cisternographic results identified CSF leakage sites in 52%, most often at the lumbar region. Also observed were limited ascent of the tracer to the cerebral convexity (91%), early appearance of radioisotope in the bladder (65%), and early soft tissue uptake of radioisotope (43%). Epidural blood patches were performed in 23 patients, which produced complete resolution of headaches in 70%. Two patients underwent drainage of subdural hematoma. None died or were disabled during hospitalization. Conclusions: Patients with CSF hypovolemia frequently have distinct MRI and radioisotope cisternographic abnormalities and often respond favorably to an epidural blood patch.

Intracranial Dural Arteriovenous Fistulas: Analysis of 60 Patients

Objective: To analyze and update the clinical symptomatology, CT and MRI findings, angiographic features, and therapeutic outcomes of patients with dural arteriovenous fistulas (DAVFs). Background: Studies of DAVFs in a large number of patients have seldom appeared in neurology literature. Methods: The authors investigated 60 consecutive patients with DAVFs who were admitted between January 1991 and January 2001. The DAVFs were graded into five types according to the classification of Cognard et al. [Radiology 1995;194:671–680]. Presumable etiologies, clinical features, imaging findings and therapeutic outcomes were evaluated on the basis of the location and type of DAVFs. Results: Sinus thrombosis, previous trauma, craniotomy, cerebral infarction and acupuncture were detected as possible etiologic factors of DAVFs. The cavernous sinus (57%) was the most common location of DAVFs. Although the neurological symptoms were closely related to the location of the DAVFs, in some patients, there were also symptoms that did not reflect the location. Although the women outnumbered the men, the men presented with aggressive neurological manifestations more often (p < 0.05). Ten out of 12 patients (83%) with DAVFs involving locations other than the large sinuses presented with aggressive neurological manifestations. 70% of brain CTs and 81% of brain MRIs showed abnormal findings suggestive of DAVFs. Of 33 patients who underwent only endovascular embolization, 29 patients (88%) were cured or improved. Radiosurgery and surgical excision done in some patients provided fair results. Patients with DAVFs involving large sinuses showed a better therapeutic outcome than those in whom locations other than the large sinuses were involved, while patients without venous ectasia had more a favorable outcome than those with it (each, p < 0.01). Conclusions: DAVFs result from various etiologic factors, show diverse manifestations usually reflecting the location and can be treated successfully in most patients. Factors related to poor clinical outcome include male sex, the presence of venous ectasia and involved locations other than the large sinuses.

Bilateral Effects of Unilateral Subthalamic Nucleus Deep Brain Stimulation in Advanced Parkinson’s Disease

To investigate the bilateral effects of unilateral subthalamic nucleus deep brain stimulation (STN-DBS), we prospectively studied 9 consecutive advanced Parkinson’s disease (PD) patients (2 men and 7 women) who underwent unilateral STN-DBS. Patients were evaluated preoperatively and at 3 and 6 months postoperatively with and without dopaminergic medications (‘on’ and ‘off’ medication, respectively). Postoperatively, patients were assessed with and without stimulation. We found that, when compared with baseline, the ‘off’ medication scores of the Unified Parkinson’s Disease Rating Scale motor part (UPDRS III) and activities of daily living (UPDRS II) were improved by 37% (p = 0.028) and 50% (p = 0.046) at 6 months after surgery, respectively. Stimulation while ‘off’ medication improved the total UPDRS score by 42% (p = 0.028) at 6 months. At 6 months after surgery, the subscore of UPDRS III of body parts contralateral to the DBS implantation had improved by 48% (p = 0.028), and the ipsilateral subscore of UPDRS III and the axial subscore of UPDRS III had improved by 20% (p = 0.027) and 39% (p = 0.028), respectively. Daily dosage of levodopa was reduced by 15% at 6 months. No patient exhibited permanent side effects. These findings indicate that unilateral STN-DBS may be a reasonable surgical procedure for selected PD patients who have markedly asymmetric parkinsonism.

Use of complementary and alternative medicine by Korean patients with Parkinson's disease.

OBJECTIVES Many patients with Parkinsons disease (PD) often utilize complementary and alternative medicine (CAM). We aimed to survey the prevalence, spectrum of use, and factors related to utilization of CAM in patients with PD in Korea. PATIENTS AND METHODS Between 15 December 2005 and 30 April 2006, we studied 123 patients with PD who volunteered to be interviewed using semi-structured questionnaires. RESULTS Ninety-four (76%) patients had used CAM. The mean cost of CAM paid by patients (out-of-pocket costs) was 102.3 US Dollars (USD) per month, while medical costs of treatment for PD paid by patients (out-of-pocket costs) averaged 72.8 USD per month. Patients using CAM sought to improve motor symptoms (57.6%), fatigue (19.6%), pain (4.3%), constipation (5.4%) or specified no single reason (13.0%). The spectrum of CAM use included oriental medicines (76.6%), traditional food (44.7%), non-prescribed drugs (31.9%), traditional therapies (7.4%), massage (7.4%) and behavioral therapy (7.4%). Factors related to current use of CAM were disease duration, degree of education, and daily levodopa equivalent dose. In a logistic regression analysis, the duration of PD was a significant factor for CAM use. CONCLUSIONS These results suggest that a high proportion of Korean PD patients employed CAM, associated with high costs and serious side effects in some patients.

Functional brain imaging in pure akinesia with gait freezing: [18F] FDG PET and [18F] FP‐CIT PET analyses

Pure akinesia with gait freezing (PAGF) has characteristic features, including freezing of gait and prominent speech disturbance without rigidity or tremor. The purpose of this study was to investigate changes in brain glucose metabolism and presynaptic dopaminergic function in PAGF. By using [18F] fluorodeoxyglucose (FDG) PET, 11 patients with PAGF were compared with 14 patients with probable progressive supranuclear palsy (PSP), 13 patients with Parkinsons disease (PD), and 11 normal controls. [18F] N‐(3‐fluoropropyl)‐2β‐carbon ethoxy‐3β‐(4‐iodophenyl) nortropane (FP‐CIT) PET was performed in 11 patients with PAGF and with 10 normal controls. The PAGF patients showed decreased glucose metabolism in the midbrain when compared with normal controls. PSP patients showed a similar topographic distribution of glucose hypometabolism with additional areas, including the frontal cortex, when compared with normal controls. The FP‐CIT PET findings in patients with PAGF revealed severely decreased uptake bilaterally in the basal ganglia. These findings suggest that both PAGF and PSP may be part of the same pathophysiologic spectrum of disease. However, the reason why PAGF manifests clinically in a different manner needs to be further elucidated.

Levosulpiride-induced movement disorders†

Levosulpiride is a substituted benzamide that is widely used for the management of dyspepsia and emesis. However, little is known about levosulpiride‐induced movement disorders (LIM). The aim of this study was to investigate the clinical characteristics of patients with LIM. Among 132 consecutive patients who were diagnosed with drug‐induced movement disorders between January 2002 and March 2008, 91 patients with LIM were identified and their medical records reviewed. Seventy‐eight (85.7%) patients were aged more than 60 years. The most common LIM was parkinsonism (LIP) (n = 85, 93.4%), followed by tardive dyskinesia (n = 9, 9.9%) and isolated tremor (n = 3, 3.3%). Twenty‐one (24.7%) of the 85 patients with LIP were rated as Hoehn and Yahr stage III–V. The oro‐lingual area was the only body part that was involved by tardive dyskinesia. LIM persisted after withdrawal of levosulpiride in 48.1% of patients with LIP, 66.7% with dyskinesia, and none with isolated tremor. None of clinical and MRI features predicted the reversibility of LIP. Levosulpiride frequently causes drug‐induced movement disorders, presenting mainly with LIP followed by lower face dyskinesia. The symptoms are often severe, and irreversible even after the withdrawal of levosulpiride. Physicians should be cautious in using levosulpiride, especially in elderly patients.

Short- and Long-Term Outcomes of Spontaneous CSF Hypovolemia

We undertook a study to investigate the short- and long-term outcomes of spontaneous CSF hypovolemia. Fifty-three consecutive patients with spontaneous CSF hypovolemia were included. Short-term outcome was assessed 4 weeks after the treatment in all patients. Long-term outcome after a mean follow-up of 61 months (range, 13–101 months) was evaluated in 26 patients. There were 18 men and 35 women (66%) with an age range of 22–64 years (mean 37 ± 9.5). In assessing the short-term outcome of CSF hypovolemia, we found that complete headache relief was significantly higher in 43 patients who received epidural blood patch (EBP) than in 10 patients treated with supportive measures (p < 0.05). After a mean follow-up of 61 months, 25 (96%) of the 26 patients evaluated for long-term outcome had complete headache relief. These results suggest that EBP is safe and effective, resulting in excellent short- and long-term outcomes of CSF hypovolemia.

Comparison of brain MRI and 18F-FDG PET in the differential diagnosis of multiple system atrophy from Parkinson's disease

To investigate the diagnostic value of brain magnetic resonance image (MRI) and 18F‐fluorodeoxyglucose positron emission tomography (18F‐FDG PET) in the differentiation of multiple system atrophy (MSA) from Parkinsons disease (PD). Thirty‐five patients with MSA (23 MSA‐P and 12 MSA‐C) and 17 patients with PD were included in this study. Overall correct diagnosis rates between clinical and imaging diagnosis among MSA‐P, MSA‐C, and PD patients were 80% for visual MRI analysis, 88.5% for visual 18F‐FDG PET analysis, and 84.3% for SPM‐supported analysis of 18F‐FDG PET. The sensitivity of brain MRI, and visual and SPM analysis of 18F‐FDG PET in differentiating MSA from PD was 72.7%, 90.9%, and 95.5%, respectively, the specificity was 100% for each imaging analysis, the positive predictive value was 100% for each imaging analysis, and the negative predictive value was 60%, 81.8%, and 90%, respectively. Our results suggest that brain MRI and 18F‐FDG PET are diagnostically useful in differentiating MSA (MSA‐P and MSA‐C) from PD, and indicate that 18F‐FDG PET has a tendency toward higher sensitivity compared to brain MRI, but a larger longitudinal study including pathological data will be required to confirm our findings.

Effect of trientine on manganese intoxication in a patient with acquired hepatocerebral degeneration.

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Focal hand dystonia in a patient with PANK2 mutation.

nevirapine, was started. One year later symptoms persisted, but were less frequent. Brain MRI scanning was normal. CSF showed 4 leuc/ L, normal glucose and protein levels and intrathecal synthesis of oligoclonal bands. By this time he had 331/mm CD4 cells. Every others studies were normal (electrolytes, thyroid function, serum/CSF serology, serum immunology, cupper, and ceruloplasmin). Surface electromyography performed with slow repetitive nerve stimulation (3 Hz) in facial nerve at rest, 30 s and 2 min after exercise showed no decrement in CMAP. Pyridostigmine was tried without any improvement. Larynx CT, laryngoscopy, and electroencephalogram were normal. Treatment with HAART was maintained. Symptoms persisted for 6 months then became less frequent and finally disappeared. He is still on HAART and asymptomatic. Apraxia of eyelid opening designates an intermittent inability to open the eyes voluntarily. Anatomical location of this lesion and pathophysiology are not clear but there is evidence that this condition is due to an abnormality in the supranuclear control of eyelid movement.3 Regarding the patient symptoms we have also considered the possibility of a neuromuscle junction disorder, but the clinical features did not support that: no relation to exercise or fatigability and he had no difficulty in keeping the lids open once they had been manually lifted up. EMG repetitive stimulation test and pyridostigmine trial were against that hypothesis. The presence of CSF inflammatory markers (intrathecal oligoclonal bands), improvement with antiretroviral therapy and absence of other etiology makes the correlation between HIV infection and patient symptoms reasonable. As far as we know this is the first report of a patient with apraxia of eyelid opening and AIDS. In a series of six HIV positive patients and dyskinesias, without opportunistic infections, two had normal MRI brain scanning and the other four had white matter changes or cerebral atrophy.2 Other two HIV positive patients presenting with chorea, without any other cause known besides HIV infection improved with HAART.4,5 There is another report of a patient that presented with similar features to Huntington disease, chorea, and behavioral changes, in which MRI scanning showed atrophy without signal changes, CSF had 18 cells/ L and two oligoclonal bands. She worsened progressively and a right temporal biopsy was performed showing the typical findings of HIVE.6 HIVE diagnosis is based on the evidence of multinucleated giant cells, microgliosis, and myelin pallor in frontotemporal cortex, hippocampus, basal ganglia, midbrain, and cerebellum, as well as substantial presence of HIV in the postmortem tissue.7 Like with other lentiviral infections such as feline immunodeficiency virus and simian immunodeficiency virus, selective vulnerability of neuronal subpopulations has been associated with HIV infection.8 There is also evidence that gp120, an envelope protein of HIV, is selectively toxic to dopaminergic neurons, leading to a reduction of dopamine in the brain and also inhibits dopamine uptake in mesencephalic neuronal cultures.9 We hypothesize that those symptoms could be due to HIVE that in an early stage was focused primarily in the mesencephalus.