Outcome of Pallidal Deep Brain Stimulation in Meige Syndrome

OBJECTIVE: Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome. METHODS: Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was 23.1 +/- 6.4 months. The mean age at time of surgery was 58.0 +/- 7.8 years. The mean duration of symptoms was 8.7 +/- 7.6 years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After 2.4 +/- 1.3 days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). RESULTS: BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant. CONCLUSION: The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.

Pallidal Deep Brain Stimulation in Primary Cervical Dystonia with Phasic Type : Clinical Outcome and Postoperative Course

OBJECTIVE: The purpose of this study was to analyze in detail the relationship between outcome and time course of effect in medically refractory primary cervical dystonia (CD) with phasic type that was treated by bilateral globus pallidus internus (Gpi) deep brain stimulation (DBS). METHODS: Six patients underwent bilateral implantation of DBS into the Gpi under the guide of microelectrode recording and were followed for 18.7 +/- 11.1 months. The mean duration of the CD was 5.8 +/- 3.4 years. The mean age at time of surgery was 54.2 +/- 10.2 years. Patients were evaluated with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) and relief scale using patient self-reporting. RESULTS: The TWSTRS total scores improved by 64.5%, 65.5%, 75.8%, and 76.0% at 3, 6, 12 months, and at the last available follow-up after surgery, respectively. Statistically significant improvements in the TWSTRS scores were observed 3 months after surgery (p = 0.028) with gradual improvement up to 12 months after surgery, thereafter, the improvement was sustained. However, there was no statistically significant difference between the scores at 3 and 12 months. Subjective improvement reported averaged 81.7 +/- 6.8% at last follow-up. Mild dysarthria, the most frequent adverse event, occurred in 3 patients. Conclusions: Our results show that the bilateral Gpi-DBS can offer a significant therapeutic effect from 3 months postoperatively in patients with primary CD with phasic type, without significant side effects.

Electrophysiologic Study for Estimating the Clinical Severity of Hemifacial Spasm

BACKGROUND AND PURPOSE: Hemifacial spasm(HFS) is a chronic and often progressive disorder characterized by unilateral irregular clonic and tonic contractions of one or more muscles of facial expression. Many previous electrophysiologic studies showed characteristic features of HFS differentiating from other involuntary movements of the face. However, there has been no electrophysiologic study for estimating the clinical severity of HFS. This study was prospectively designed to evaluate the relationships between electrophysiologic findings and clinical severity of HFS. METHODS: The authors performed direct facial nerve stimulation, blink reflex, and lateral spread response in 62 patients with HFS, and compared the results of affected side with those of unaffected each other. Clinical severity was graded into seven groups (0-6) by questionnaire and confirmed by a neurologist. RESULTS: The results were as following. 1) The total number of subjects were 62, the number of patients in group 2 was 3 (4.8%), group 3 was 12 (19.3%), group 4 was 25 (40.3%), group 5 was 21 (33.9%), and group 6 was 1(1.6%). 2) The mean age was 54.5+/-9.1years old, the mean duration of the illness was 6.4+/-4.9years, male was 11(17.7%) and female was 51(82.3%), and involved sites were right in 26(41.9%) and left in 36(58.1%) patients. 3) Direct stimulation of facial nerve showed no differences between affected and unaffected sides in HFS. 4) The results of blink reflex showed more increased latencies and larger amplitudes of R1 & R2 responses in affected sides than in unaffected sides of HFS, but no differences among the groups. 5) The lateral spread responses were found in 45 of 62 (72.6%) patients by stimulation of zygomatic branch and recording in mentalis muscle, 26 of 62 (41.9%) patients by stimulation of mandibular branch and recording in orbicularis oculi muscle on affected side. 6) There was a linear correlation between the presence of lateral spread response with zygomatic or mandibular stimulation and the grade of clinical severity. CONCLUSION: We suspected that the lateral spread response was a significant electrophysiological test for estimating the clinical severity of HFS.

Reversible Parkinsonism in Idiopathic Hypoparathyroidism

BACKGROUND AND SIGNIFICANCE: Parkinsoinsm is characterized by clinical symptoms of igidity, bradykinesia, tremor, and gait disturbance. There are many causes of secondary parkinsonism. Hypocalcemia associated with hypoparathyrodism is a rare metabolic cause of secondary parkinsonism. We experienced a case presenting reversible secondary parkinsonism caused by hypocalcemia associated with idiopathic hypoparathyroidism. CASE: A 35-year-old woman was admitted to our hospital because of generalized edema. She had been diagnosed as nephrotic syndrome for 1 year. During admission, slurred speech suddenly developed and hypocalcemia was founded. With replacement of calcium and dialysis, these symptom were improving. However, she complained of slowly progressive gait disturbance. On examination, she had parkinsonian features including masked face, slurred speech, rigidity of all extremities, bradykinesia, gait disturbance with short step, and stooped posture. Laboratory studies showed hypocalcemia, primary hypoparathyroidism, normal thyroid and parathyroid scan. Brain CT and MRI revealed normal. She was diagnosed as secondary parkinsonism following hypocalcemia associated with idiopathic hypoparathyroidism. She was started on levodopa and bromocriptine, and parkinsonian features slowly improved. One month later, she was fully recovered from parkinsonian features, and then levodopa and bromocriptine were discontinued. COMMENT: We report a case of reversible secondary parkinsonism caused by hypocalcemia with idiopathic hypoparathyroidism.

A comparison between obstructive hydrocephalus and non-obstructive hydrocephalus on midsagittal brain MRI

Hydrocephalus refers to distension of the cerebral ventricles, usually as the result of obstruction somewhere along the pathway of the cerebrospinal fluid circulation. Hydrocephalus ex vacuo means ventricular enlargement in the presence of cerebral atrophy or irreversible loss of brain parenchyma. According to morphologic change of ventricular enlargement, the mechanism and results of treatment are quite different. MRI improves diagnostic accuracy in patients with hydrocephalus because of its ability to show small obstructing lesion and anatomical changes. To evaluate differential points, we compared patients with obstructive hydrocephalus and non-obstructive hydrocephalus. T1-weighted sagittal images were retrospectively analyzed in 19 patients with obstructive hydrocephalus, 22 non-obstructive hydrocephalus, and 36 normal subjects. On midsagittal section, we evaluated mamillopontine distance, thickness and shape and position of the corpus callosum, and antero-inferior portion of the 3rd ventricle. The average mamillopontine distance was 11.5mm for normal subjects, 8.2mm for obstructive hydrocephalus group, and 13.6mm for non-obstructive hydrocephalus group(p < 0.01). Dilatation of the anterior inferior portion of the 3rd ventricle was seen only in 11 patients of obstructive hydrocephalus group. Average thickness of the corpus callosum at the level of the foramen of Monro was 7.5mm for normal subjects, 5.1mm for obstructive hydrocephalus group, and 4.4mm for non-obstructive hydrocephalus group(p < 0.01). There was a difference in the shape of corpus callosum between obstructive hydrocephalus and non-obstructive hydrocephalus group. It is concluded that mamillopontine distance, thickness and shape of the corpus callosum, dilatation of the antero-inferior 3rd ventricle could be good differential points between obstructive hydrocephalus and non-obstructive hydrocephalus.

Bilateral spontaneous carotid-cavernous fistula(CCF) presenting as painful ophthalmoplegia: Two cases

Carotidcavernous fistula(CCF) is a syndrome in which arteriovenous sinus shunts exist between the carotid artery and the cavernous sinus. These shunts have very wide spectrums in pathogenesis, angiografic findings, hemodynamics and teatment. Spontaneous dural CCF is dural vascular malformations that usually runs a benign course with variable clinical findings. We experienced two case of bilateral spontaneous CCF presenting as painful ophthalmoplegia. In the first case, a 53-years old hypertensive, diabetic woman had previous history of unilateral painful ophthalmoplegia eight months before admission and as partially recovered. On examination, she had slowly progressive bilateral chemosis, exophthalmos, and sixth nerve palsy. MRI showed abnormal vessels in the cavernous sinus from prominent superior ophthalmic vein. Angiographic findings showed fistulas of both external carotid and left internal carotid arteries draining into the cavernous sinus. We had multiple trials of transarterial and transvenous embolization with gelfom material or platinum coils with partial improvement of chemosis and exophthalmos. In the second case, a 45-years old woman noted slowly progressive headache, ptosis, dilatation of pupil in the left side, and diplopia suggesting paralysis of the left inferior rectus muscle. MRI showed signal voids in the cavernous sinus. Angiographic findings showed fistulars of the right and left internal carotid arteries draining into the cavernous sinus. CCF should be consider as a underlying cause in patient presenting as painful ophthalmoplegia. We report two cases of bilateral spontaneous CCF presenting as painful ophthalmoplegia.

A case of acute disseminated encephalomyelitis(ADEM) following rubella infection

Acute disseminated encepahalomyelitis(ADEM) is acute monophasic infammatory demyelinating disease of the central nervous systems occurring several days to two weeks after viral illness or vaccination. ADEM following rubella infection is very rare. We experienced a case of 24-years old man who developed confusion, nysmgmus, paraparesis, and voiding difficulty three days after the recovery form rubellar infection expressing skin rash and fever. CSF study showed pleocytosis(119cells/mm3), increased titer of [gM and IgG for rubella,, and myelin basic protein, whereas the level of the glucose and protein is normal. MRI of brain, cervical and thoracic spinal cord showed multiple high signa] intensities in the deep white matter of brain, entire cervical and upper thoracic spinal cord. He had been treated with high dose solumedrol intravenously for six days, and then oral prednisolone for ninteen days. Follow-up CSF study after seven days showed normal profiles and MRI after three weeks demonstrated remarkably decreased size of the previous lesions. He showed much improvement of neurologic symptoms except mild voiding difficulty. We report a case diagnosed as ADEM with good prognosis following rubella infection.