Kl Tsang

Estrogen improves motor disability in parkinsonian postmenopausal women with motor fluctuations

Objective: To test the efficacy, tolerance, and safety of low-dose oral estrogen in postmenopausal women with PD associated with motor fluctuations. Background: Motor fluctuations in PD may be predictable or unpredictable, and eventually affect most patients after long-term levodopa therapy. Although estrogen can modulate nigrostriatal dopamine levels, its effects on PD are unclear. Methods: Patients were randomized to receive conjugated estrogen (oral Premarin 0.625 mg daily; n = 20) or placebo (n = 20) in a double-blind, parallel-group, prospective study over 8 weeks. Existing antiparkinsonian drug regimes were kept unchanged. Changes in “on” and “off” periods using patient diaries, Unified Parkinson’s Disease Rating Scale (UPDRS) score, timed tapping score, and Hamilton Depression Scale score were determined by one rater. Subgroup analyses were also performed on patients with only predictable motor fluctuations. Results: Both treatment groups were similar in age, duration of disease and menopause, antiparkinsonian medication, and compliance with test medication and diary assessments. “On” and “off” times, and motor score (UPDRS subscale III) improved with estrogen, using the Mann–Whitney U test (p < 0.05 after Bonferroni adjustment). Mean “on” time improved by 7% (9 hours/week of awake time) in estrogen-treated patients versus a deterioration of 0.5% (1.4 hours) in placebo-treated patients (95% confidence interval, [CI] of mean difference, 5.73 to 14.9). Mean “off” time improved by 4% (4.4 hours/week of awake time) in estrogen-treated patients versus no change in placebo-treated patients (95% CI, 1.54 to 7.16). Mean subscale III score improved by 3.5 points in estrogen-treated patients versus 0.4 in placebo-treated patients (95% CI, 1.02 to 5.18). No other significant changes were observed (p > 0.05). Subgroup analyses in patients with only predictable motor fluctuations showed similar results, except improvement in mean subscale III score was marginally not significant (p = 0.07; 95% CI, 1.06 to 6.24). Five patients on estrogen had facial flushing, three had lower abdominal discomfort, and two had mild withdrawal vaginal bleeding. The adverse events were mild and resolved without sequelae. Conclusion: Low-dose estrogen is a safe and effective adjunct therapy to existing antiparkinsonian treatment in reducing motor disability in postmenopausal women with PD associated with motor fluctuations.

Emotion recognition in patients with idiopathic Parkinson's disease

Emotion recognition (ER) was examined in 64 patients with idiopathic Parkinsons disease (PD; 56 bilateral and 8 right‐sided) and 64 matched healthy volunteers. Participants were administered an ER battery, consisting of the following subscores: overall ER (OER), overall facial ER, facial emotion identification (FEI) and discrimination, overall prosodic ER, and prosodic emotion identification (PEI) and discrimination. Measures of visuospatial functions, auditory attention, and depression were also administered. After controlling for visuospatial functions, auditory attention and depression, results indicated that patients with bilateral PD had poorer performance on all ER subscores, regardless of the modality and type of experimental task involved, relative to healthy volunteers. However, patients with right‐sided PD had difficulty on FEI and PEI only. Whereas none of the clinical variables examined in this study predicted any of the ER subscores, visual organization and auditory attention positively predicted OER in patients with PD. In addition, visual organization also positively predicted FEI in these patients. Implications are discussed in terms of the neural substrates underlying ER.

Postictal psychosis related regional cerebral hyperperfusion

Postictal psychosis is a known complication of complex partial seizure in particular temporal lobe epilepsy. It usually runs a benign and self limiting course. A postictal phenomenon with focal cerebral hypofunction (similar to Todds palsy), rather than ongoing seizure activity, has been postulated.1 2 Surface EEG is either normal or showing non-specific slow waves.3 Hence, antipsychotic medications are prescribed instead of antiepileptic drugs. Until recently, the pathogenic mechanisms have remained unknown. In this communication, we report on two patients with postictal psychosis, during which a cerebral SPECT study showed a hyperperfusion signal over the right temporal lobe and contralateral basal ganglion. As hyperperfusion in ictal cerebral SPECT is closely linked to epileptic activities,4 our findings support a contrary explanation for postictal psychosis. Interictal SPECT and SPECT performed during postictal psychosis. (Top) A SPECT study of patient 1 showing areas of relative hyperperfusion over the right temporal neocortex (red arrows) and the left basal ganglia (blue and yellow arrows) during postictal psychosis. (Bottom) SPECT study of patient 2 showing areas of hyperperfusion over the right temporal neocortex and the left basal ganglia. Arrows indicate areas of hyperperfusion. Prolonged video-EEG telemetry study was performed in patients who underwent presurgical evaluation for epilepsy surgery. Antiepileptic drugs were withdrawn …

A case-controlled MRI/MRA study of neurovascular contact in hemifacial spasm

BACKGROUND Neurovascular contact (NVC) with the root exit zone (REZ) of the ipsilateral facial nerve is associated with hemifacial spasm (HFS), but unresolved issues remain. OBJECTIVES To 1) determine the frequency of symptomatic and nonsymptomatic NVC, 2) determine the features of NVC associated with HFS, and 3) correlate severity of HFS to these features. METHODS Two independent, blinded, prospective assessments of high-resolution MR and MR angiography (MRA) images were performed on Chinese cases (HFS: n = 44; age-matched control subjects: n = 20). RESULTS Over 88% of 44 symptomatic sides in patients with HFS had NVC of the ipsilateral facial nerve. At least 80% of symptomatic sides involved NVC at the anterior aspect of the REZ [REZ(ant.)]. Although NVC was observed in approximately half of nonsymptomatic sides, at least 70% of them were not at REZ(ant.). NVC at the cisternal and intracanalicular portions of the facial nerve were not associated with HFS. Half of our patients with HFS had bilateral NVC, but none had bilateral symptoms. Most of our MR/MRA images showed that the size and position of the arterial branches of the vertebrobasilar system were markedly asymmetric. Of patients with bilateral NVC, over 83% had asymmetric NVC sites. The anterior inferior cerebellar artery was the most common vessel involved in NVC, but was not significantly associated with HFS. Most of the NVC involved one vessel at one contact point with no indentation. The development of HFS was significantly associated with nerve indentation in NVC. The development and severity of HFS were not associated with multiple contact points in NVC. No significant interobserver variability existed between the blinded assessments. CONCLUSIONS MRI/MR angiography are accurate, fast, and safe in characterizing neurovascular contact (NVC) at the brainstem. The site of NVC and ipsilateral facial nerve indentation in NVC are significant determinants for the development of hemifacial spasm (HFS). The lack of bilateral NVC at the anterior aspect of the root exit zone of the facial nerve could explain in part the lack of bilateral symptoms. The development and severity of HFS are not associated with a specific blood vessel or multiple contact points in NVC.

Translation and validation of the standard Chinese version of PDQ-39: A quality-of-life measure for patients with Parkinson's disease

PDQ‐39 has been widely used in the research and clinical management of Parkinsons disease. It has been translated into and validated in various non‐English languages. We report here on the validity and reliability results for the translated standard Chinese PDQ‐39 questionnaire. Fifty‐four patients were recruited from a movement disorder clinic and two regional patient groups, and data were collected by direct interview. Nineteen patients had the tests repeated 4 weeks later to assess the test–retest and interrater reliability. The standard Chinese version of PDQ‐39 demonstrated acceptable internal consistency (Cronbachs α = 0.54–0.90) and was comparable to versions in other languages. Further analysis showed good construct validity and test–retest reliability. Implications and limitations of the study are discussed.

Bath‐related headache

Bath-related headache (BRH) is a rare primary headache syndrome. We present our experience over seven years and review all reported cases of BRH. Thirteen patients, including six from our group, are described. BRH occurred exclusively in middle-aged or elderly Oriental women (mean age 51 years, range 32-67. Hong Kong 6 cases, Taiwan 4 cases, Japan 3 cases). The typical presentation was a uniphasic cluster of severe headache recurrently triggered by bathing or other activities involving contact with water. Each attack lasted 30 min to 30 h. Onset was hyperacute, consistent with that of thunderclap headache. Reversible multisegmental cerebral vasoconstriction was found in two patients. No underlying secondary causes were identified. Response to acute treatment was generally unsatisfactory, but headache could be prevented by avoiding the specific trigger(s). BRH runs a self-limiting course; all patients remitted within three months after onset. Nimodipine may shorten the duration of illness.

Idiopathic inflammatory demyelinating disorders after acute transverse myelitis

Acute transverse myelitis (ATM) is commonly para‐infectious. Recurrent ATM occurs in connective tissue diseases (CTD), infective myelitis and idiopathic inflammatory demyelinating disorders (IIDD) including multiple sclerosis (MS) and neuromyelitis optica (NMO). Previous studies might include NMO and idiopathic recurrent transverse myelitis (IRTM) as MS. The aim was to study the outcome of patients after a first attack of idiopathic ATM. Idiopathic ATM patients over a 6‐year period were retrospectively studied. Known causes of myelopathy were excluded. Among 32 patients studied, 20 (63%) had single ATM attack upon follow up for 39–93 months, three developed recurrent ATM related to CTD (two systemic lupus erythematosus and one anti‐Ro antibody positive) and nine (28.1%) developed recurrent neuroinflammation compatible with IIDD. Among IIDD patients, three had NMO, two restricted variant of NMO, three IRTM and one classical MS. NMO, its variant and IRTM had mean spinal MRI abnormality of 3.7, 2.1 and 3.9 vertebral segments respectively while non‐recurrent ATM had 1.6 vertebral segments. Four (80%) of the five patients with NMO or its variant had poor neurological prognosis versus only one (5%) of non‐recurrent ATM patients. IRTM patients had advanced mean onset age, 62 years vs. 43 years for non‐recurrent ATM patients. In IIDD patients presenting with ATM as first attack of neuroinflammation, NMO and its variant (56%) were most frequent, then IRTM (33%), with classical MS (11%) the rarest. As long‐term treatments for NMO are different from MS, early recognition of NMO and its variant is important for prevention of serious neurological deficits.

Clinical outcome of relapsing remitting multiple sclerosis among Hong Kong Chinese.

BACKGROUND Clinical outcome of Chinese relapsing remitting multiple sclerosis (RRMS) patients is uncertain. AIM To study the long-term clinical outcome of Chinese RRMS patients. METHOD RRMS patients with duration of 10 years or longer followed up in our hospital is retrospectively studied. RESULTS 61 RRMS patients (75% female) were studied. Their mean symptom onset age was 25.9 years and mean duration was 20.6 years (range 10-33); 36% patients had received β-interferon and 30% azathioprine. Their mean EDSS scores were 3.3 (range 1-7) and 4.7 (range 1-8) at 10 years and latest follow-up (mean duration 20.6 years) respectively. At 10 years, 30% patients had EDSS score ≤2, 34% EDSS 2.5-3.5, 20% EDSS 4.0-5.5 and 16% ≥6; 18% developed SPMS. At latest follow-up, 15% patients had EDSS ≤2, 20% EDSS 2.5-3.5, 19% EDSS 4.0-5.5 and 46% ≥6.0; 53% developed SPMS. The median time from symptom onset to EDSS 6 was 22 years. No differences were detected in demographic characteristics, presenting neurological features, number of attacks in first 2 years, neuroradiological findings and disease modifying therapies between patients with EDSS <6 and ≥6 at ten years. EDSS scores at 10 years and latest follow-up were similar for patients who had received β-interferon and those who had not. CONCLUSION Hong Kong Chinese RRMS patients may have worse long-term clinical outcome than Caucasian patients.

Sixty-Six-Year-Old woman with unexplained falls followed five years later by severe generalized myoclonus

In early 1988, a 66-year-old woman with a history of good health began to suffer from frequent falls. They were usually backwards and were not preceded by orthostatic or other cardiovascular symptoms. There was no suggestion of convulsive activity and she had never lost consciousness. These falls occurred about six or eight times a year and on one occasion she fractured her left femur, on another her wrist. She saw a neurologist for the first time 2 years into her illness, who diagnosed cryptogenic drop attacks. She was subsequently seen privately by another neurologist and was admitted under his care to the National Hospital for Neurology and Neurosurgery (NHNN) in January 1991. On examination, she was considered to have a spastic, shuffling gait with some weakness, spasticity, generalized hyperreflexia and equivocal plantars. A compressive spinal cord lesion or multiple sclerosis (MS) was considered. Computed tomography (CT) brain scan and chest X-ray were normal, and magnetic resonance imaging (MRI) of the cervical and thoracic spines showed advanced degenerative changes but no impingement on the spinal canal. Nerve conduction tests showed only a minor left ulnar neuropathy. Cerebral spinal fluid (CSF) showed leakage of oligoclonal IgG from a source located outside the central nervous system (CNS) but was otherwise normal. Visual and auditory evoked potentials (VEPs and AEPs) and standard blood test results were all normal. She left the hospital with a diagnosis of motor paraparesis of unknown cause, and was coded as “probable MS.” Her walking progressively deteriorated and she continued to have falls. Four years after onset, she was unable to walk, and a year later, at the age of 71 years, she noticed jerking movements of both legs and, a few months later, of the right arm, then left arm. These movements occurred at rest, were increased by action, and became so severe that she was precluded from performing basic daily activities; she became wheelchair-bound and was unable to transfer. She was catheterized because of urinary retention. She was admitted to NHNN for a second time in February 1994, under the same consultant. Examination revealed normal eye movements. In the limbs there was an apparent coarse tremor which worsened with action, and cogwheeling and slowness of alternating movements in the arms. There was diffuse weakness in all four limbs, and all reflexes were pathologically brisk, with ankle clonus bilaterally but flexor plantar responses. Sensation was normal. Autonomic function tests were essentially normal and a subcutaneous apomorphine test (3 mg) was negative. Selective bedside cognitive testing by a neuropsychologist showed some impairment in visuospatial skills and Kin-Lun Tsang’s current address: University Department of Medicine, Queen Mary Hospital, Pokfulam, Hong Kong SAR. *Correspondence to: Prof. N.P. Quinn, University Department of Clinical Neurology, Institute of Neurology, Queen Square, London, WC1N 3BG, United Kingdom. Received 16 April 2000; Revised 23 June 2001; Accepted 12 July 2001 Published online 18 December 2001. Movement Disorders Vol. 17, No. 1, 2002, pp. 174–180

MRI in vertebral artery dissection

In a recent report, Auer et al described the clinical and imaging findings in 19 cases of extracranial vertebral artery dissection retrospectively.1 We make the following comments. Firstly, the authors described the “sensitivity” and “specificity” of digital subtraction angiography (DSA), magnetic resonance imaging/angiography (MRI/A), and duplex sonography for diagnosing extracranial vertebral artery dissection.1These figures were based on the percentage of probable and definite features among the 19 patients. Nevertheless, sensitivity of a test is the number of cases with true positive results divided by the total number of positive results (including both true and false positives), and specificity is the number of cases with true negative results over the sum of true and false negatives. The authors misquoted the terms “sensitivity” and “specificity” in their report, as …