Co-occurrence of Myasthenia Gravis in a Patient with Systemic Sclerosis-Sjogren's Syndrome without D-penicillamine Therapy / 대한류마티스학회지

Systemic sclerosis is an autoimmune disease characterized by progressive fibrosis of the skin and visceral organs. Myasthenia gravis is also an autoimmune disease characterized by weakness and fatigue of skeletal muscles. The symptoms of systemic sclerosis and myasthenia gravis overlap clinically, so the recognition of disease co-occurrence may be delayed. Co-occurrence of myasthenia gravis and systemic sclerosis is very uncommon and usually diagnosed after use of D-penicillamine for treating the systemic sclerosis. We report a case of a 49-year-old female patient who complained of general weakness and was diagnosed with myasthenia gravis. Four months earlier she was diagnosed with systemic sclerosis with Sjogren's syndrome and her medications did not include D-penicillamine.

Dopamine Receptors and Agonists in Parkinson's Disease

No abstract available.

A Case of the Os Odontoideum Associated Neurological Syndromes

The Os odontoideum is a rare congenital disorder which is due to non fusion of axis and odontoid process so that seperation of odontoid process occurs. It causes various neurological syndrome because of atlantoaxial dislocation due to bony defect. To our knowledge, this type of malformation in a narrow sense has been reported only three cases in the world literature, and there are no reports of this malformation in Korea. With reviewing some of literatures, We present a case of the Os odontoideum which was confirmed by operation.

Three Cases of Meige's Syndrome (Blepharospasm-Orofacial Dystonia Syndrome)

In 1910, Henri Meige described "spasme facial median", which a disorder characterized chiefly by symmetric dystonic spasm of the facial muscles. This dyskinesia is most commonly seen in middle aged or elderly women and consists primarily of blepharospasm with marked overflow into other facial movements. The cause of this disorder is obscure and there has been no consistent response to any particular treatment. With reviewing some of literatures, we present 3 cases of Meige's syndrome which is improved by pharmacotheraphy.

A Clinical Study of Brain Tuberculomas Based Upon 20 Case Records

The authors reviewed the medical records of 20 patients who were diagnosed as brain tuberculoma at Severance Hospital from 1980 to 1984. The 20 patients were presumptive cases based on clinical, roentgenological or laboratory findings without histologic proof were used, exept one. All cases were Korean, onset was between 3 to 59 years of age and there was no sexual preponderance. The initial clinical features were seizure, symptoms and signs of increased intracranial pressure, focal neurological signs, etc. Although a definite diagnosis is often not possible on CT scan, the method is of value in determining number, location and extent of lesions and in evaluating the effectiveness of antituberculous therapy. Fourteen of our 20 patients had single tuberculoma, six had mutliple or miliary tuberculoma and 18 cases were located on the cerebrum, two on the brainstem.

Increased Plasma B-Endorphin in Patients with Cerebral Thrombosis

We measured plasma B-Endorphin in 10 patients with cerebral thrombosis, with comparing normal control group and obtained the following results. 1. The mean value of plasma B-Endorphin was 6.173+0.335 pmol/l in normal control male group and 4.55+0.443 pmol/l in normal control female group. 2. The level of plasma B-Endorphin was increased as mean value was 29.2 pmol/o in acute phase of cerebral thrombosis and it showed significant statistical difference. 3. Measurement on convalescent phase in patient with cerebral thrombosis also increased significantly compared with normal control group, but reduced significantly compared with patients in acute phase.

Ataxic Hemiparesis Due to Brainstem Tuberculosis

No abstract available.

Roussy-Levy Syndrome: A Case Report

This is a case report of the Roussy-Levy syndrome. The patient is a 22year-old male with with gait ataxia, pes cavus, tremor on both hands, dysequilibrium and peroneal muscular atrophy. On family history, his grandfather, father, aunt and younger brother were affected similar symptoms but slightly milder. Abnormal signs on neurologic examinotion were atropy by of both legs, high arched feet, decreased position and vibration sensation in both lower extremities, absent DTRs in all extremities, positive Romberg test on closed eyes and intention tremor of both hands. On EMG study, there is denervated potentials in tested muscles. On sensory nerve conduction velocity study, no potentials was appeared and on motor NCV, prolonged terminal latency (6.2-8.6 m/sec)and delayed NCV (31-36m/sec) were appeared. His symptoms have been slowly progressed and condition is relatively stable at present.

Chorea as a Clinical Manifestation of Delayed Neurologic Sequelae in Carbon Monoxide Poisoning: Case Report

No abstract available.

A Clinical Study of Cryptococcal Infection

A clinical study was carried out in 15 patients with cryptococcosis who were seen at Severance Hospital from June, 1974 to September, 1983 and the following results were obtained. Age of patients ranged from 13/12 to 72 years (mean 23.9) with the peak incidence in child and adolescence. Common symptoms at admission were headache, fever, chilling etc. Initial impression at admission were Tbc. Meningitis, Aseptic Meningitis, Cryptococcal Meningitis, lymphoma and hepatitis etc. in that order. Involved organs were meninges in 14 patients (93.3%), lymphnodes 4 (26.6%), lung 2, liver 2, skin 1, Brain 1, adrenal gland 1. Variable findings were obtained in initial CSF study, but all cases except two were positive in initial CSF india ink preparation. In our study, the prognosis was better in patients without coexisting disease than with coexisting disease.

Seizure Disorder Associated with Focal Low Density on Computed Tomography

During last 3 years, we experienced 24 cases of acute seizure disorder, showing localized low density on computed tomographic (C-T) scan and having the clinical characteristics, and have analyzed clinically. The results were followings: 1. It was commoner in men, with a ratio of 2:1, and their ages ranged from 4 to 54 years (mean 20.9 years), with the peak incidence in 2nd & 3rd decades. 2. It occurred in any season, but commoner in summer and autumn. 3. This disorder was characterized by sudden onset of convulsion, focal or generalized, in health man, and had no other associated symptoms. 4. C-T brain scan showed decreased density without mass effect on parietal or frontal area, which had usually constrast enhancement such as small ring or nodular type, and disappeared between 3 to 5 months. 5. Other laboratory tests were normal except leukocytosis (11 of 18 cases) and abnormal EEG (9 of 24 cases). 6. This disorder was self limited. The cause was unknown, but it had the possibility of viral infection.

A Case of Adynamia Episodica Hereditaria

Four members of a family suffering from frequent attacks of flaccid paralysis with asymptomatic myotonia were evaluated. There was an autosomal dominant pattern of inheritance and their earliest symptoms were noticed between 1-3 years of age. The plasma potassium level rised during the attack and an EMG demonstrated myotonic discharge. Treatment with acetazolamide was beneficial. These findings were consistent with adynamia episodica hereditaria. The clinical features and pathogenesis were also described.

A Case of Miller Fisher Syndrome (Variant of Guillain Barr'e Syndrome-Ophthalmoplegia, Ataxia, Areflexia)

Miller Fisher syndrome is a syndrome of acute external ophthalmoplegia, ataxia and areflexia without significant motor or sensory deficit in the limbs and usually results in complete recovery without specific treatment. It's accurate anatomic lesion sites and pathogeneiss is still unknown. Recently we experienced a 57 year old man who had the sudden onset of ophthalmoplegia, ataxia, areflexia and albuminocytologic dissociation in CSF and completely recovered 2 1/2 months later.

A Case of Miller Fisher Syndrome (Variant of Guillain Barr'e Syndrome-Ophthalmoplegia, Ataxia, Areflexia)

Miller Fisher syndrome is a syndrome of acute external ophthalmoplegia, ataxia and areflexia without significant motor or sensory deficit in the limbs and usually results in complete recovery without specific treatment. It's accurate anatomic lesion sites and pathogeneiss is still unknown. Recently we experienced a 57 year old man who had the sudden onset of ophthalmoplegia, ataxia, areflexia and albuminocytologic dissociation in CSF and completely recovered 2 1/2 months later.