Two Cases of Holmes-Adie Syndrome

BACKGROUND: Adie's tonic pupil consists of mydriasis, accommodation defect, decreased light reflex, and tonic near reflex. When tonic pupil is combined with hypo- or areflexia, it is called Holmes-Adie syndrome. CASE: Fifty-two-year-old and 37-year-old female patients showed unilateral dilated irregular pupil, decreased light reflex, and decreased or absent DTR. When 0.125% pilocarpine was dropped on the dilated pupil, the pupil was constricted after pilocarpine application. Somatosensory evoked potentials were within normal ranges, and H-reflex responses were absent or delayed.

A case of wilson's disease showing palialia as an initial symptom

Wilson's disease is an autosomal recessive disorder resulting from an excessive accumulation of copper in the liver, cornea, kidneys, and in the basal ganglia of the brain. The prominent speech disturbances of Wilson's disease include monopitch, monoloudness, slow rate, low pitch, delayed in initiating speech and rarely palilalia. A19-year-old woman developed palilalia which was characterized by compulsive repetition of a phrase with increasing rapidity and with a decrescendo of voice volume. Although she had been suffering from liver cirrhosis for the past 8 months, the palilalia was the only neurological sign at initial examination. She showed a low serum ceruloplasmin, low serum copper, increased urinary copper excretion, and Kayser-Fleischer rings. Brain magnetic resonance images showed high signals in the bilateral basal ganglia in T2-weighted images, and slight cortical atrophy.

Cognitive Function in Non-Insulin Dependent Diabetic Patients : P300 Event-related Potentials and Neuropsychological Tests

BACKGROUND AND OBJECTIVE: Several recent studies have demonstrated that cognitive function is impaired in non-insulin dependent diabetes mellitus (NIDDM) patients compared with age-matched non-diabetic controls. However, the underlying pathophysiological mechanism of this diabetic central nervous involvement remains obscure. The present study was designed to explore further whether cognitive deficits are present in NIIDDM patients who do not show apparent symptoms and signs of central nervous system damage and to evaluate the influence of clinical parameters and diabetic complications on cognitive function. METHODS: Thirty patients over 50 years of age with NIDDM and 30 age-, sex- and education-matched normal controls were recruited for the study. P300 event-related potential test and neuropsychological tests (Mini-Mental State Examination, MMSE; Benton Visual Retention test; Digit Span Forward and Backward) were perfomed. P300 latencies were recorded according to the conventional technique using Counterpointer MK2. RESULTS: Compared with controls, NIDDM patients showed significant impairment in MMSE (P<0.05), Benton Visual Retention (P<0.05), and Digit Span Backward (P

Automatic respiratory failure after medullary infarcts

Ondine's curse refers to the failure of automatic breathing during sleep associated with preservation of voluntary respiratory controls. This syndrome of Ondine's curse usually result from both bilateral and unilateral medullary tegmental infarcts. We report a case of a 56-year old hypertensive man who showed automatic respiratory failure during sleep and bilateral medullary infarcts in brain MRI.

Occipital lobe seizure due to hypertensive encephalopathy in youth

BACKGROUND & OBJECTIVES: Hypertensive encephalopathy is an acute neurologic syndrome characterized by abrupt and marked elevation of blood pressure, headache, vomiting, seizure, visual disturbance, and altered mental status. This syndrome may occur as a complication of toxemia, renal artery stenosis and acute glomerulonephritis. We report 4 young patients with occipital lobe seizure, as a presenting sign of hypertensive encephatopathy, whose brain MRI and perfusion scans showed lesions on bilateral occipital lobes. Case : Four young patients experienced moderate to severe headache, visual illusion, generalized seizure, and loss of sight for a few days. Their systolic blood pressure was 150-170mmHg and diastolic blood pressure 100-120mmHg. Three patients had proteinuria and hematuria as the results of acute glomerulonephritis. CSF findings were within normal limits. EEG showed intermittent generali,ed or bilateral temporoparietooccipital slowings in all patients and continuous parietooccipital slowings in one patient. Brain MRI man showed hyperintensity signal in T2WI and hypointensity signal in TlWl on bilateral occipital lobes and SPECT scan showed normal perfusion three patients and increased perfusion on occipital areas in one patient. They were treated with antihypertemsive agents and anticonvulsants (phenobarbital or valporic acid) for 2-10 months. Seizure was well controlled and didn't recur though discontinuing anticonvulsant. CONCLUSION: In young age, acute hypertension may cause clinically occipital lobe seizure and radiologically bilateral occipital lobe lesions.

A Case of Bilateral Cavernous Sinus Mucormycosis

Rhinocerebral mucormycosis, which is a fulminant and progressive disease often seen in debilitated hosts! Requires a high degree of clinical suspicion to promptly diagnose and an aggressive medical and surgical therapy. The prognosis is grave, especially in case of bilateral cavernous sinus thrombosis. We report a case of 69-year-old female patient with bilateral cavernous sinus thrombosis caused by rhinocerebral mucormycosis, who showed orbital swelling and lateral bulging of bilateral cavernous sinus in orbital CT, and large branching nonseptate hypae in biopsed specimen of the left periorbital necrotic tissue.

Expression of c-fos mRNA in the Hippocampus of Pentylenetetrazol Kindling Rat

Kindling, induced by repeated subconvulsive electrical or chemical stimulations, leads to progressive and permanent amplification of seizure activity, resulting in permanent brain changes. It is a good animal model of epilepsy and neural plasticity. C-fos has been proposed as the gene responsible for turning on molecular events for these permanent brain changes underlying epilepsy and neural plasticity. But the role of c-fos in the development of kindling is controversial. This study was undertaken to investigate the role of c-fos mRNA in the plastic changes underlying kindling. Among 66 adult male Sprague-Dawley rats, 29 rats were kindled by repeated administrations of subconvulsive doses (IS-25 mg/kg) of pentylenetetrazol (PTZ), 25 rats experienced convulsions induced by a single injection of convulsive dose(30-60 mg/kg) of PTZ, and 12 rats experienced convulsions by a single electroconvulsive shock (ECS), Twelve control rats received normal saline only. Animals were sacrificed at various seizure stages. C-fos mRNA levels in the hippocampus were quantified using slot-blot hybridization analysis. In the experiment of PTZ kindling, c-fos mRNA expression 30 min after convulsion was elevated about 2-4 times at stage 1, 2 and 5, but wasn't increased at stage 3 and 4, compared with controls. C-fos mRNA expression 60 min after convulsion was elevated about 2 times at stage 1 and 5, but wasn't increased at stage 2, 3 and 4. In the experiment of PTZ-induced seizures, c-fos mRNA expression 30 min after convulsion was elevated 2.5, 2.2 and 6 times stage 1-2, 3-4, and 5, respectively. C-fos mRNA expression 60 min after convulsion was elevated 3.6 times at stage 3-4, but wasn't increased at stage 2 and 5. In the experiment of ECB-induced seizures, c-fos mRNA expression 1 min after mild convulsion was elevated 3,3 times, but wasn't increased generalized tonic-clonic seizure. C-fos mRNA expression 60 min after convulsion wasn't increased at any stage of convulsion. These results show that c-fos mRNA levels have no meaningful relationship with the stages of PTZ kindling, and PTZ or ECS-induced seizures, and that c-fos mRNA does not seem to play the crucial role in turning on a molecular program underlying kindling.

A Study on F Wave in Amyotrophic Lateral Sclerosis

F waves are motor responses produced by antidromic activation of motoneurons following stimulation of motor axons peripherally. The F wave is useful in evaluating proximal motor nerve conduction, in assessing the integrity of the ventral root, and in measuring the motor neuron excitability. This study was undertaken to assess the integrity of the lower motor neurons in amyotrophic lateral sclerosis(ALS). F-wave latency(FWL), F-wave conduction velocity(FWCV), F ratio, F-response frequency, and distal MNCV were determined in the median and tibial nerves of 9 patients with ALS. The results were compared with 32 healthy adult volunteers as controls. In patients with ALS, in contrast,to the control subjects, the mean FWL was prolonged, the mean FWCV and distal MNCV slowed, and the mean F-response frequency reduced. These results are thought to reflect some degree of loss of the lower motor neurons.

Polymorphonuclear CSF Pleocytosis during the Treatment of Tuberculous Meningitis

Among 73 patients with possible and definite tuberculous meningitis, 14 cases showed a sudden unexpected polymorphonuclear (PMN) CSF pleocytosis during treatment. Patients with superimposed bacterial meningitis were excluded. Eleven patients(15. 1%) matched inclusion criteria. The intervals between the onset of the treatment and the onset of the PMN CSF pleocytosis were 7-54 days(mean 17.2+ 14.4 days). The mean duration of PMN CSF pleocytosis was 14.2+12.4 days. A PMN CSF pleocytosis may develop occasionally weeks or months after the start of the treatment for tuberculous meningitis. Though the cause is uncertain, we suggest that probably its cause is superimposed acute meningeal inflanunation by the release of Mycobacterium from tuberculomas or.delayed Jarisch-Herxheimer reaction.

A Case of Thrombotic Thrombocytopenic Purpura Associated with Large Intracerebral Hemorrhage and Peri-pheral Neuropathy

Thrombotic thrombocytopenic purpura(TTP) is characterized by fluctuating neurologic signs, microangiopathic hemolytic anemia, thrombocytopenia, fever and renal abnormalities. Neurologic involvement occurs in over 90% of patients with TTP. TTP is rarely associated with large intracerebral hemorrhage and peripheral neuropadiy. The authors report a case of TTP associated with large intracerebral hemorrhage confirmed by brain MRI and peripheral neuropathy diagnosed electrophysiologic findings and sural nerve biopsy.

A Case of Stiff-Man Syndrome Associated with Panhypopituitarism

Stiff-man syndrome is a rare disorder characterized by intermittent spasms and stiffness of the axial and limb muscles, associated with an electromyographic pattern of continuous motor unit activity in affected muscles. The cause of this disorder is unknown, but it has been associated with autoimmune disease and with endocrine disorder. We present a 49 year old female patient with clinical and electrophysiologic features of stiff-man syndrome and postoperative panhypopituitarism, whose muscle stiffness and spasm resolved with diazepam, clonazepam and prednisolonereplacement therapy.

Effects of monoclonal anti-IL-4 antbody(11B11) and interferons onIgE production in vivo and hypersensitivity reactions I. inductionof systemic anaphylaxis in mice by antigen-specific IgG and IgG subclasses monoclonal antibodies / 대한면역학회지

No abstract available.

A Case of Myositis Ossificans Progressiva

A case of myositis ossificans progressiva is reported. The patient, a 22-year -old progressive ossification for 16 years with severe motion limitation. Bone CT and MRI, bone scanning and muscle biopsy showed multiple ossifying lesions in the connective tissue. All labolatory findings are normal. But serum alkaline pbosphatase become worse with disodium etidronate(EHDP) therapy. The case is presented with the review of literatures.

A Case of Acute Paraplegia in Hypereosinophilic Syndrome

Hypereosinophilic syndrome (HES) is characterized by a persistent eosinophilia of l,500/mm3 lasting for at least 6 months associated with multi-system involvement and no other identifiable cause. A 45-year-old man had hypereosinophilia, cardiopathy, and acute paraplegia. Following corticosteriod with antipletelet agent therapy, paraplegia was improved rapidly and circulating eosinophils were normalized. We postulated that embolic phenomenon was due to hypercoagulable state by eosinophil-derived proteins and/or to direct infiltration by eosinophils.

The Usefulness of Color-Coded Phase Image for the Evaluation of Regional Wall Motion Abnormalities in Coronary Artery Disease

Phase and Amplitude Image were developed to overcome the subjective nature of interpretation for cinematic endless loop display of radionuclide gated blood pool scan. To evaluate the usefulness of color coded phase image in coronary artery disease, we conducted a clinical study with coronary angiography and phase image in 23 patients, and compared the results of those studies. 1) The results of coronary angiography revealed normal coronary artery in 6, 1 vessel disease in 11, 2 vessel disease in 4, and 3 vessel disease in 2 patients. 2) Color-coded Phase Image revealed abnormal phase area in 2/2(100%) of 3 vessel disease patients and in 3/4(75%) of 2 vessel disease patients, in 5/11(45%) of 1 vessel disease patients, and only 1/6(16%) of normal coronary artery. 3) Among the patients who disclosed abnormal phase image, 5 patients of 2-or3-vessl disease revealed abnormal phase through whole left ventricular area, but 3 patients with right coronary artery lesion and 2 patients with left antrior descending artery lesion showed localized abnormal phase area, infero-apical and anterior portion respectively, which were in accord with each coronary lesion. We concluded that the phase image may be useful in evaluation of the regional wall motion abnormalities in the patients of coronary artery disease. Further studies seem to be neccessary to clarify the sensitivity and specificity of the phase image in the each disease entity group.

Postictal Serum Prolactin Alterations in Various Type of Seizures

Postictal sequential serum prolactin concentrations were observed in 28 patients with various type of seizures who were hospitalized at Keimyung University Hospital. Of 28 epileptic patients, 18 male and 10 female, age ranged from 18 years to 79 years (mean 42 years), 15 with generalized seizures, 13 with focal seizures (5 with simple partial seizures and 8 with partial seizures with generalization). Symptomatic epilepsy and epileptic patients with factors which may affect serum prolactin concentrations were excluded in this study. Serum prolactin concentrations were measure at 15, 30 and 60 minutes after convulsion and 24 hours after convulsion for the base line serum prolactin level. Patients with generalized seizsures and significant elevation of serum prolactin concentrations at 15, 30 and 60 minutes after ictus (Table 1). Same results were obtained in patients with simple partial seizures at 30 minutes and 60 minutes after ictus, and 15 minutes and 30 minutes after ictus in patients with partial seizures with secondary generalization (Table 2 and 3). Level of serum prolactin concentrations were higher in patients with generalized seizure than the other type of seizures. Therefore, measuring serum prolactin level could help differentiate postictal state and other causes of mental change, and also differentiative various type of seizures.

Clinical Studies on Thalamic Hemorrhage: Factors Affecting the Prognosis

Clinical studies were made on 37 cases with thalamic hemorrhage diagnosed by computed tomographic scan and only localized on the thalamic area, were admitted to the Keimyung University Dongsan hospital from January 1981, to December 1985. The age and sex distribution, symptoms and neurologic signs on admission, relationship between the hospital course and many factors affecting the prognosis such as age, side of lesion, level of consciousness, volume of the hematoma, degree of hydrocephalus and ventricular hemorrhage were analysed. The results were summarized as follows; 1. The most prevalent age group was above 51 years of age with 41-50 years, 21-30 years and 31-40 years of age in the order of frequency. Male to female ratio was 15:22. 2. The clinical symptoms on admission in the order of frequency were the impairment of consciousness, headache, nausea and vomiting, speech disturbance and hemiparesis, dizziness and voiding difficulties. The neurologic signs on admission showed hemiparesis, hyperreflexia, and positive Babinski sign, impaired consciousness, hemisensory deficit, central facial nerve palsies, dysarthria, sluggish and unreactive light reflex, small pupil and absent gag reflex, impaired vertical gaze, papilledema, aphasia and abducens nerve palsies in that order. 3. The hospital course had no significant relationship with the age of the patients and the side of hematoma but there was a significant relationship between the alert-drowsy group and the stuporcoma group (P<0.01). 4. The hospital course had no significant relationship with the degree of volume of hematoma and hydrocephalus. The hospital course had a significant relationship with ventricular hemorrhage (P<0.05) but the hospital course of the improved group had no significant tendency to the rate of improvement.