Tc-99m-HMPAO SPECT Findings in Clinically Suspected HSE

Herpes simplex virus type I (HSV-I) causes an acute necrotizing encephalitis that selectively affects tempioral and frontal lobes. The sequelae and mortality of herpes simplex encephalitis(HSE) may be reduced by available antiviral therapy and therefore early diagnosis of HSE is essential. We have assessed the potential of brain perfusion scintigraphy using Tc-99m-HMPAO single photon emission computed tomoglaphy(SPECT) in HSE. Nine Tc-99m-HMPAO SPECTs were performed in eight patients with clinically suspected herpes simplex encephalitis. The examinations were made between 18 days and 10 months after onset of encephalitic symptoms The SPECT images in seven patients revealed decreased accumulation ol radioactivity in the affected temporal, frontal or parietal lobes. In a 30 yr-old female patient, two SPECT images were obtained on 18th day after the onset of symptoms and 3 months later. Her initial SPECT showed increased accumulation of radioactivity in the affected temporal and inferior frontal lobes. 3 month later follow-up SPECT image revealed the decreased radioactivity in the affected temporal, inferior frontal, and inferior parietal lobes. We found that the SPECT done at early stage of HSE may show increased cerebral perfusion due to inflammatory or other pathologic mechanism and the later stage SPECT may show decreased cerebral perfusion due to decreased cerebral metabolism caused by neuronal death We suggest that Tc-99m-HMPAO SPECT may support the clinical diagnosis of HSE.

Effect of Nimodipine on Ischemic Brain Edema of Gerbil

The mechanisms involved in brain neuronal damage in ischemia are related to the elevation of cytosolic calcium concentration and calcium antagonist is considered as a promising drug that may alleviate ischemic neuronal damage. Using transient global ischemia model of Mongolian gerbil, we studied the effect of nimodipine, a cerebroselective calcium antagonist, on ischemic brain edema. We treated each gerbil intraperitoneally with nimodipine (lmg/kg) or the same amount of saline 30 minutes prior to ischemia, and transient global ischemia was induced by means of clipping both common carotid arteries either for 10 minutes or for 45 minutes. Three hours after reperfusion, the animals were decapitated and the water content of the bain was determined by oven dry method. With 10 minute ischemia the brain water content in nimodipine pretreatment group (78.6 +/- 0.2%) was lower than that in saline pretreatment group (79.1 +/- 0.4%) significantly (p<0.05). But with 45 minute ischemia nimodipine pretreatment did not reduce the postischemic increase of water content compared with saline pretreatment (79.8 +/- 0.4% and 79 6 +/- 0.4%, respectively; not significant). Our results suggest that nimodipine pretreatment may suppress the development of ischemic brain edema and its effect depends largely on the extent of brain ischemia.

A Clinical Study of 21 Patients with Lateral Medullary Syndrome

We reviewed clinicoradiologic findings of twenty-one patients with lateral medullary(Wallenberg) syndrome who admitted at Seoul National University Hospital from March, 1983 to February, 1989. The results are as follows: 1. Lateral medullary syndrome was 14.6% of ischemic stroke in vertebrobasilar territory during the same period. The ages ranged from 35 to 69 years and most of them were in 6th or 7th decades. Z. Detected risk factors were hypertension in 17, previous stroke in 8, hyperlipidemia in 7, and diabetes mellitus in 4. 3. The interval which developed maximal characteristic symptoms after onset were within 5 days in all patients and 2 days in 13 patients. 4. Frequent complaints were dizziness in 21, vertigo in 17, nausea or vomiting in 18, swallowrng difficulty in 14, dysarthria in 12, headache in 11, hoarseness in 7, hiccup in 7, and facial pain in 3, 5. Frequent signs were Horner's syndrome in 21, ataxia in 21, crossed sensory change in 19, soft palate sagging in 15, ipsilateral facial weakness in 9, DTR increase in the same side in 7, tongue deviation in 6, transient diplopia in 5, and transient altered consciousness in 4, 6. Disease courses were considerable improvement in 18, recurrence in 1, and discharge against medical advice in 1. Frequent disturbing complaints during the follow-up period were paresthesia or sensory loss in 16 and ataxia in 11. 7 Among the radiologic studies, MRI could define the lesion at corresponding site in 8 of 9, CT in 3 of 21, and TFCA in 3 of 6. These results suggest that MRI is the best rdiologic study to yeild the lesion in lateral medullary syndrome.

A Clinical Study of 38 Cases of Olivopontocerebellar Atrophy

Olivopontocerebellar atrophy (OPCA) is a form of degenerative disease associated with neuronal degeneration in the cerebellar cortex, pons, and inferior olives. The authors have reviewed 38 cases, clinically diagnosed as OPCA, admitted in department of neurology, Seoul National University Hospital From January, 1985 to August, 1989. Seven cases of familial and 31 cases of sporadic forms have been gathered. Gait ataxia and dizziness are the most frequent initial manifestations and the cerebellar ataxia and pyramidal tract signs are frequent neurologic findings in both familial and sporadic forms. Age of onset is earlier in the familial form (mean 31.4y) than in the sporadic form (mean 47.5y). The duration of the disease is longer in the former(6.8y) than in the latter (2.9y). Abnormal ocular movement and nystagmus are more frequent in the familial form, while autonomic changes and parkinsonian features in the sporadic form.

Multiple Sclerosis in Korea: Its Clinical Features and Estimated Prevalence Rate

It is well-known that multiple sclerosis (MS) has a higher prevalence rate in occidental countries than in Asia and Africa. In addition to their lower prevalence rate of MS, oriental countries may have different clinical characteristics. It has been shown in the previous studies performed in Japan that there is more frequent involvement of optic nerve and spinal cord during the course of disease. These results were also confirmed by the study of MS in Hawaii which compared orientals with caucasians in their clinical features. In Korea, there has been no estimate for clinical features and prevalence rate of MS except two studies. We performed this study to evaluate the clinical features and estimated prevalence rate based on a series of 50 consecutive cases of MS seen over 8 and a half year-period in the Seoul National University Hospital.

Current Status of Cerebrovascular Disease in Korea

To evaluate the current status of cerebrovascular disease in Korea, we performed a retrospective study on 1260 cases of stroke who had been admitted to or had visited Seoul National University Hospital, Choongnam National University Hospital, Hanyang University Hosipital, and Chung Ang University Hospital in the period from Jul 1, 1986 to Jun 30, 1987. The incidence was highest in sixth and seventh decades. Ischemic stroke (51.1%) was more common thar, hemorrhagic stroke (47.6%), and thrombotic infarction was the leading type (37.7%) of all kinds of strokes. Middle cerebral arterial territory was the most commonly involved site in thrombotic and embolic stroke. Among intracerebral hemorrhage, basal ganglia hemorrhage was found in 47.7%, and was followed by thalamic(23.8%), lobar (15.3%) and pontine hemorrhage (9.4%). Primary intraventricular hemorrhage (IVH) was relatively rare (8 cases, 7.5% of all IVH), and hemorrhages in basal ganglia or thalamus frequently induced secondary IVH. 16 cases (10.7%) of subarachnoid hamorrhage were accompanied by secondary IVH. This study suggests changing trends of stroke in Korea, and multicenter prospective study using stroke registry is requird for confirmation.

Two Cases of Unverricht-Lundborg Disease

Unverricht-Lundborg disease(Baltic myoclonus) is one of the major causes of progressive myoclonus epilepsy. It is characterized by stimulus sensitive myoclonic seizure, generaized tonic-clonic seizure, generally synchronous polyspike and wave discharges on EEG and absence of severe or early dementia. It has usually been described in the countries around the Baltic area. But recently, it is regarded as the most common form of progressive myoclonus epilepsy in the other countries as well. We report, with the review of the literature, two patients who showed the typical features of this disorder.

A Case of Down Syndrome with Atlanto-axial Dislocation

Atlanto-axial instability in children with Down syndrome(trisomy 21) has become a wel! Known entity and it has been reported frequently since it was first reported over 25 years ago. However, symtomatic atlanto-axial dislocation associated with cord compression is relatively rare and it had seldom been reported. We recently experienced a 14-year-old mongoloid female with progressive quadriparesis, who have no prior cervical inflammation and we detected atlanto-axial dislocation with ossculum terminale and cord compression through radiological evaluations. We report a case of symptomatic atlanto-axial dislocation in Down syndrome with review.

A Case of Podophyllin Induced Peripheral Neuropathy

Podophyllin is known as a neurotoxin, We recently observed a 34-year-old woman with podophyllin induced peripheral neuropathy. She was treated with topical podophyllin for perianal multiple condyloma acuminata. One day after application, she complained of G-I symptoms ( abdominal pain, nausea, vomiting, diarrhea), followed by tingling sensation on all extremities. We confirmed peripheral neuropathy with electrophysiologic study and sural nerve biopsy. She has improved on follow-up.

Primary Intraventricular Hemorrhage

Nine adult patients with non-traumatic primary intraventricular hemorrhage (PIVH) were reviewed. Lack of lateralizing sign could be one of the differential points between PIVH and secondary intraventricular hemorrhage (SIVH). In three cases, vascular malformations were identified. Bleeding diathesis was present in one. The pathogenesis of hemorrhage in the remaining five cases was speculated. According to the mainly involved ventricle, prognosis was presumed to be variable. From this study, we conclude that for PIVH investigation including angiography and/or magnetic resonance imaging should be done to identify the etiology and to conduct appropriate management. Survival was more common than SIVH.

A Clinical Study on Wernicke Disease

No abstract available.

3 Case of Shy-Drager Syndrome

Shy-Drager syndrome is a multisystem degenerative disorder of the central nervous system in which progressive autonomic failure such as orthostatic hypotension, urinary or fecal incontinence, impotence and anhidrosis is a main feature. The nonautonomic neurological abnormalities include parkinsonia, cerebellar and pyramidal signs. In 1984 we met 3 such cases at Seoul National University Hospital and present them here with a brief review of literatures.

A Study on Cerebellar Hemorrhage

We analysed 20 cases of cerebellar hemorrhage which were diagnosed with the brain CT scan at Seoul National University Hospital from 1982 to 1984. The most frequent presumptive cause of the hemorrhage was hypertension (65% of our cases). Most of the ictus occurred during their normal daily activities. Common symptoms at the onset were vomiting, headache, vertigo, other cerebellar dysfunctions and subsquent alteration of consciousness. The patients who showed rapid deterioration of neurologic condition were fatal in outcome. The poorer the consciousness at arrival and the larger the size of hematoma or hydrocephalus at first brain CT scan, the graver the outcome. So we suggest that suboccipital craniotomy is inevitable in cases of rapid deterioration within several hours, hematoma larger than 4 cm in diameter and marked hydrocephalus on their first brain CT scan. And neurosurgical treatment will be considered in cases of aggravating tendency over 24 hrs with hematoma larger than 3 cm and/or moderate hydrocephalus on the brain CT scan.

A Clinical Study on Spontaneous Subarachnoid Hemorrhage: with a special reference to intracranial complications

The authors reviewed the medical records of 94 patients who met the strict diagnostic criteria of spontaneous subarachnoid hemorrhage (SAH). They were diagnosed and treated at Seoul National University Hospital from January 1983 to June 1984. The interrelationships among the age, clinical status on admission, findings on brain computerized tomography (CT), site of the ruptured aneurysm and the intracranial complicaions (ventricular dilatation, vasospasm, rebleeding) and their outcome were studied. Conclusions obtained are as follows: 1) The local clot on CT (especially associated with ICH and /or IVH) was more frequently found in clinically poor patients than in good ones. 2) Cerebral angiography (TFCAs, 53 cases) revealed the anterior communicating artery (A-COM) to be the most common site of aneurysm in 21 cases (39.6%) and multiple aneurysms in 5 cases (9.4% : two in 4 cases, three in 1 case) and no aneurysm in 9 cases (17%). Vasospasm on angiography was found in 27 cases (50.9%). 3) Ventricular dilatation, which was measured on the first CT after SAH, was detected in 43 cases (45.7%). High incidence was found in the cases showing a local clot on CT (53.4%), especially associated with intraventricular hemorrhage (IVH; 83.3%), and in the cases of A-COM aneurysm rupture (52.4%; probably due to IVH). 4) The clinical vasospasm was edtected in 41 cases (43.6%). The interval between SAH and the development of clinical vasospasm ranged from from 3 to 35 days (mean 9.8 days). Thirty-five cases (85.4%) of them suffered from clinical vasospasm within 14 days. The incidence of clinical vasospasm steeply increased in the elderly patients (Fifties: 60%, Sixties: 66.7%) but that of findings of vasospasm on angiography (angiographic vasospasm) slowly increased with age. The local clot on CT seemed to be the most important factor of vasospasm, both clinically (51.7%) and angiographically (75%). The site of vasospasm on angiography was closely related with that of the ruptured aneurysm. 5) Rebleeding occurred in 21 cases (22.3%) with the interval ranging from 2 hours to 38 days (mean 10.1 days) after SAH and 14 cases (66.7%) of them rebled within 10 days. The shorter the interval after SAH, the higher the incidence. There were no definitely related factors affecting the rebleeding but it seemed to be affected to some extent by ICH and/or IVH on CT (31.3%). 6) Thirty-five cases (37.2%) diedof various causes. Mortality seemed to be most closely related with the clinical status on admission. Other related iactros were the aging, the local clot on CT (53.4%), especially associated with ICH and/or IVH (69.7%), and the development of various systemic or intracranial complications. Mortality seemed to be more closely related with ventricuar dilstation and rebleeding than with vasospasm. But it was suggested that vasospasm should be regrarded as a major cause of disabilities and an indirect cause of death.

Pseudohypoparathyroidism and Basal Ganglia Calcification: Case Report

No abstract available.

Two Cases of Oculopharyngeal Muscular Dystrophy in One Family

The authors encountered 2 siblings who showed progressive ophthalmoplegia and bulbar palsy. The patients were studied with various laboratory aids and thought to be as oculopharyngeal muscular dystrophy. The oculopharyngeal muscular dystrophy is a rare form of muscular dystrophy. The authors present the cases as familial oculopharyngeal muscular dystrophy.

Three Cases of Olivopontocerebellar Atrophies

No abstract available.