Successful intrathecal baclofen therapy for seronegative stiff-person syndrome: a case report.

We reported a 27-year-old man presenting with progressive muscle rigidity and intermittent spasms for six years. The diagnosis of stiff-person syndrome was based on the clinical features and neuroelectrophysiologic findings of continuous motor unit activities. Autoantibody against glutamic acid decarboxylase in our patient was absent either in serum or in cerebrospinal fluid. The patient was successfully treated with intrathecal baclofen therapy after a series of unsatisfactory traditional medical treatments. From our experience, we propose that intrathecal baclofen could be a considerable alternative treatment for patients with refractory stiff-person syndrome.

Epidural abscess of the cervical spine with atypical manifestations: a report of two cases.

BACKGROUND: Epidural abscess of the cervical spine is a medical and surgical emergency, especially when it is located at cervical area, considering this critical anatomic location. Although there is the so-called triad of epidural abscess (fever, local pain, and neurologic deficits), these are not sensitive enough to detect spinal abscess early and prevent significant morbidity and mortality. REVIEW SUMMARY: We report 2 fatal cases of cervical spine epidural abscess with atypical manifestations. Patient 1 presented as an ascending paraparesis without signs of upper motor neuron involvement. The initial presentation was only lumbago and paraparesis. Patient 2 presented as a pure motor monoparesis of the lower limbs with lumbago. However, both had no neck pain or percussion tenderness initially. There was also no fever in patient 1. The diagnosis was made on the fourth and eighth days, respectively. They both had longstanding diabetes mellitus (DM) and presumably were not well controlled. Diabetic neuropathy and an immunocompromised status resulted in delayed diagnosis. Neither received surgical intervention. Both patients died despite correct empiric antibiotic therapy. CONCLUSION: A high index of suspicion is most important in making a rapid, correct diagnosis of spinal epidural abscess (SEA) when a patient presents with local spinal pain and has risk factors like DM. The classic symptom triad of SEA is not sensitive enough for early detection, so a erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) and CRP can be used to improve the accurate diagnosis. Spinal MRI should be performed as soon as possible. When patients present with neurologic deficits, surgical intervention is essential if there is no contraindication.

Postmalaria neurologic syndrome: a case report.

Neurologic signs and symptoms are common in acute malarial infection. However, after the parasites have been cleared from the blood and patients recover full consciousness, neurologic or psychiatric symptoms may occur or recur within 2 months after the acute illness. This phenomenon is called "postmalaria neurologic syndrome" (PMNS). We present a 50-year-old man who returned from the Republic of Malawi and soon developed Plasmodium falciparum malaria. Cerebral malaria, renal failure, hepatic failure, diffuse intravascular coagulation with thrombocytopenia, and upper gastrointestinal bleeding were noted during the acute stage. He was admitted to the infectious diseases ward and treated for 3 weeks. He was free from clinical general symptoms and parasites in blood smear when discharged. However, 2 weeks after discharge, he began to experience severe headache, dizziness, diplopia, mild hand tremor, unsteady gait, and easy falling. When readmitted to the neurologic ward, he presented with irritability, delirium, visual hallucination, and strange behavior. Neurologic examination was normal except for mild general weakness and evident truncal ataxia when walking. Brain magnetic resonance imaging revealed no structural lesions, and electroencephalography showed diffuse cortical dysfunction. Cerebral spinal fluid profile exhibited cytoalbuminologic dissociation. Brain single photon emission computed tomography showed diffuse cerebral parenchymal disorder. Nerve conduction studies revealed early sensory predominant polyneuropathy. The unsteadiness persisted for the initial 2 weeks of hospitalization until corticosteroid was administered. Intravenous methylprednisolone (80 mg/day) was continued for 3 days, followed by oral prednisolone (45 mg/day). His unsteadiness improved gradually after medication, and he absconded from the hospital on the 9th day of corticosteroid treatment with clear consciousness and free ambulation. The manifestation of PMNS is diverse and may present as an acute confusional state or psychosis, generalized seizure, fine tremors, cerebellar syndromes, postural hypotension, or malarial polyneuritis. Although the neurologic syndrome is primarily self-limited in most cases, corticosteroid may be beneficial in reversing PMNS.

Successful treatment of rubral tremor by high-dose trihexyphenidyl: a case report.

A 24-year-old male suffered from acute-onset right-sided hemiparesis, dysarthria, and ophthalmoplegia in February 2001. Brain magnetic resonance imaging revealed a cavernous angioma with hemorrhage over the left thalamus. Moreover, some rhythmic, coarse, low-frequency (2-3 Hz) oscillation over the right wrist and elbow was noted 1 month later. Action tremor was more predominant than resting tremor. Rubral tremor was diagnosed on the basis of the clinical presentation and tremography analysis. Rubral tremor is not unusual, and pharmacotherapy is nearly always ineffective in clinical practice. Deep brain stimulation, thalamotomy, and pallidotomy are all considered effective according to recent research. However, they are either very expensive or invasive, and involve surgical risks. In our patient, we tried valproate, clonazepam, and verapamil one after another, but all in vain. Finally, titration of trihexyphenidyl provided significant benefit. The tremor was successfully controlled by a single high daily dose of trihexyphenidyl (38 mg) without severe or uncomfortable side effects. Here, we report a case of successful monotherapy of rubral tremor with high-dose trihexyphenidyl.

Effect of levetiracetam on truncal tic in neuroacanthocytosis.

We report on an uncommon manifestation of neuroacanthocytosis in a 31-year-old woman and the successful use of levetiracetam in the treatment of her neurological symptoms. Truncal tic is one of the major presenting features of this patient. We find that Levetiracetam, a new antiepileptic drug, was effective in eliminating this patient's truncal jerks and motor tic manifestations, such as eyelid blinking and head nodding. Levetiracetam can therefore be considered as a choice for the symptomatic therapy in neuroacanthocytosis.

Unilateral stereotactic posteroventral globus pallidus internus pallidotomy for Parkinson's disease: surgical techniques and 2-year follow-up.

With the advent of levodopa (L-dopa) and the recognition of its striking effect on Parkinson's disease (PD), virtually all surgical procedures for PD ceased from the mid 1960s. However, there has been a resurgence of pallidotomy and other stereotactic procedures in the last two decades as physicians realized that most PD patients eventually face medical failure after long-term treatment with L-dopa. Nine PD patients, three men and six women, with an average age of 62 years and disease duration of 13 years underwent unilateral globus pallidus internus (GPi) pallidotomy contralateral to the side with marked akinetic symptoms and drug-induced dyskinesia. All patients were evaluated using the Unified Parkinson's disease Rating Scale (UPDRS) after drug withdrawal and while taking their optimal medical regimen, preoperatively and 6, 12, and 24 months after surgery. There was significant improvement in activities of daily living and motor subscores as well as total UPDRS score in the "off" state at the 2-year follow-up, which mainly resulted from improvement in contralateral bradykinesia and rigidity. Significant improvements in contralateral akinetic symptoms and drug-induced dyskinesia were also observed in the "on" state and were sustained for at least 2 years. Ipsilateral and axial symptoms were not altered by unilateral GPi pallidotomy. The complications of surgery were generally well tolerated. One patient had a small postoperative asymptomatic hemorrhage identified by routine follow-up magnetic resonance imaging. Another two patients developed temporary sexual disinhibition and auditory hallucination, respectively, which resolved spontaneously 2 weeks after surgery. The effect of pallidotomy for alleviation of akinetic parkinsonism is modest but significant, and continues to be effective for at least 2 years. Further analytical studies, especially the correlation of clinical effects and lesion locations, are important not only to provide direct feedback for surgeons to examine the technical accuracy and but also to facilitate understanding of the pathophysiology of PD.

Cerebral hemosiderosis as a causative factor of vascular parkinsonism.

Secondary parkinsonism has comprised about 20 to 40 percent of all parkinsonism patients in movement disorders clinic. Most of them are induced by certain medications. About 5 to 10 percent of these patients are caused by sudden vascular events, who suffer from their parkinsonism abruptly, and usually of aged people with quite apparent stroke risk factors. Hemosiderosis was only occasionally reported as a causative factor in patients with parkinsonism, who presented with an insidious onset parkinsonism and a progressive supranuclear palsy-like clinical picture. We encountered two patients with stroke-related parkinsonism, whose high resolution MRI image showed apparent cortical or intraparenchymal hemosiderosis, although Binswanger type white matter change was also noted. Intracerebral hemosiderosis that manifests clinically as vascular parkinsonism might be much more frequent than we thought.