A Case of Lemierre's Syndrome with Cranial Nerve Palsies

Lemierre's syndrome is a rare acute medical condition characterized by anaerobic oropharyngeal infections leading to septic thrombophlebitis of the internal jugular vein. The illness is often complicated by septic pulmonary emboli and distant metastatic infections. Treatment consists of long-term intravenous antibiotic therapy and surgical drainage of purulent collections. Although Lemierre's syndrome is rare, it is potentially fatal and remains an important entity for clinicians to recognize and treat appropriately. We report a 19-year-old female of Lemierre`s syndrome complicated with bacterial meningitis and multiple cranial neuropathies. (J Korean Neurol Assoc 19(5):529~532, 2001)

A Case of Spinocerebellar Ataxia Type 2 / 대한소아신경학회지

This is a case of a boy with autosomal dominant cerebellar ataxia (SCA type 2), which was confirmed by DNA analysis. A 9-year-old boy had been suffering from tremor in both arm and leg for 5 years. After traffic accident, a year ago, the symtoms were more aggravated, and he visted our hospital. The boy showed slow saccades, decreased DTR, ataxic gait and limb ataxia. We analyzed DNA repetition, which revealed positive for the SCA2 expanded repeat.

A Case of Spontaneous Intracranial Hypotension: Detection of Cerebrospinal Fluid Leakage by Early Dynamic Radionuclide Cisternography / 대한핵의학회잡지

Although cerebrospinal fluid leakage is suggested as one of the causes of spontaneous intracranial hypotension, on]y a few cases with direct evidence of cerebrospinal fluid leakage on radionuclide cisternography have been reported in the literature Indirect evidences of cerebrospinal fluid leakage such as early visualization of the soft tissue and bladder or delayed migration of radiotracer have been observed in most patients with spontaneous intracranial hypotension. We report a case of spontaneous intracranial hypotension in which cerebrospinal fluid leakage was directly demonstrated by early dynamic imaging of spine on radionuclide cisternography. We suggest that early dynamic imaging of spine is an important adjunctive procedure in detecting cerebrospinal fluid leakage in patients with spontaneous intracranial hypotension.

Postrenal Transplantation Cyclosporine Associated Hemolytic Uremic Syndrome in End Stage Renal Failure Patient Caused by Systemic Lupus Erythematosus / 대한이식학회지

Although the outcome and the possibility of renal recurrence of disease in systemic lupus erythematosus is still a matter of controversy, kidney transplantation is generally regarded as a proper indication for the treatment of patients with end stage renal failure caused by systemic lupus erythematosus. Hemolytic uremic syndrome is characterized by the symptoms of sudden onset of hemolytic anemia, thrombocytopenia, and deteriorating renal function. Many patients with postrenal transplantation hemolytic uremic syndrome have lost their grafts because of no known established treatment modality. Although the substitution of cyclosporine to FK506 is reported as a successful strategy for the treatment of cyclosporine associated hemolytic uremic syndrome in many reported cases, we cannot find the constant reports because FK506 or even OKT3 is also known as the cause of postrenal transplantation hemolytic uremic syndrome. But cyclosporine associated hemolytic uremic syndrome can be treated by the proper choice of immunosuppressant and conservative treatment. In this report, a patient with end stage renal failure caused by systemic lupus erythematosus experienced cyclosporine associated postrenal transplantation hemolytic uremic syndrome. He has recovered from the symptoms by withdrawal of cyclosporine and reduced dose of FK506 and at the same time, conservative treatment. We report this case with literature review.

Combined Facial and Abducens Nerve Palsy in Pontine Infarction

Isolated cranial neuropathies involving the facial nerve are very rare manifestations of pontine infarction, and have not been described in paramedian pontine infarction. We report a 67-year-old woman who developed sudden and long-lasting right facial nerve paralysis accompanied by transient ipsilateral abducens nerve palsy. Brain magnetic resonance imaging showed two discrete simultaneous infarctions in the right inferior paramedian pontine area, which seemed to correspond to the facial nerve fascicle and the abducens nerve fascicle, respectively. Paramedian pontine infarction can be one of the causes of facial nerve palsy and present as an isolated or predominant sign.

Dentatorubropallidoluysian atrophy in a korean family

Dentatorubropallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder usually inherited with autosomal dominant pattern, which has been mostly described in reports from Japan. Recently, DRPLA proved to be associated with an expanded CAG nucleotide report in a gene on chromosome 12p. We report the first Korean family with this mutation, which was confirmed by genetic analysis. Case History : A 34 year-old man present ad with a 5 year history of clumsiness, seizures, and gait ataxia. He had dysarthria, clumsiness of hands, gait ataxia and intermittent choreic movements in both arms. There was mild cognitive impairment. EEG showed intermittent generalized slowing, and brain MRI revealed diffuse cerebral and cerebellar atrophy with enlarged 4th ventricle. There were three other affected family members; his 37-year old sister presented with choreiform movements developed at the age of 31. His father and uncle were reported to have been ataxic during the late period of their life, who died at age of 65 and 40 respectively. DNA studies of the prebend and his sister confirmed CAG repeat expansiom in the DRPLA gene, the size of which was 64 and 66, respectively. CONCLUSION: This is the first Korean family with DRPLA, and it should be considered in any patients with inherited neurodegenerative disorder with the above-mentioned clinical features

Factors determining the volume of the spontaneous intracerebral hemorrhage

BACKGROUND AND PURPOSE: Hypertension and alcohol consumption are well known independent risk factors of spontaneous intracerebral hemorrhage (ICH). However, it is uncertain whether controlling of them reduces the volume of ICH. Furthermore, it remains also unkown whether other factors such as age, sex, education level, smoking, diabetes mellitus, and previous history of stroke are related to the volume of ICH. This study was designed to evaluate the relations of those things with the volume of ICH. METHODS: We prospectively assessed above items in 103 consecutive patients with ICH aged 33 to 87 years (mean age : 64 years, 50 men and 53 women ). We divided the volume of ICH with the use of CT scan into small (=l0cc, n=32) groups. RESULTS: The patients with previous history of stroke significantly have a smaller volume of ICH compared to those without (p=0.04). However, the other factors (age, sex, education level, antihypertensive medication, alcohol consumption, smoking, and diabetes mellitus) were not significantly correlated with the volume of ICH to multivariate analysis. The patients with previous history of stroke tended to receive more regular antihypertensive medication than these without (p-0.06). CONCLUSION: The patients with previous history of stroke have a smaller volume of ICH possibly due to adequate antihypertensive treatment. However, the other factors which we evaluated were not significantly correlated with the volume of ICH.

A case of CNS Cryptococcosis with internucleat ophthalomoplegia

Ocular disturbances related to cranial nerve lesion or increased intracranial pressure are well known in cryptococcal meningitis, but internuclear ophthaloplegia is very rare and only two cases have been reported to our knowledge. We report the third patient of internuclear ophthalmoplegia in cryptococal meningitis. The internuclear ophthalmoplegia in our case persisted for one year with a demonstrable lesion in brain MRI, in contrast to the other cases in which intranuclear ophthalmoplegia was transient and no responsible lesion was observed.

Cerebral Vasculitis and Intracranial Hemorrhage Associated with Methamphetamine Abuse

Intracranial hemorrhage is a rare complication of amphethamine abuse, and cerebral vasculitis as a cause of the hemorrhage has been demonstrated by arteriography or autopsy findings. We report a 22-year-old female, an occasional methamphetamine abuser over 1 to 2 months, who presented with the sudden onset of bifrontal headache, nausea and vomiting immediately after an intravenous injection of methamphetamine. Imaging studies demonstrated intracerebral hemorrhage and subdural hemorrhage associated with cerebral vasculitis. To our knowledge, this entity has not been previously reported in Korea.

Cerebral Vasculitis and Intracranial Hemorrhage Associated with Methamphetamine Abuse

Intracranial hemorrhage is a rare complication of amphethamine abuse, and cerebral vasculitis as a cause of the hemorrhage has been demonstrated by arteriography or autopsy findings. We report a 22-year-old female, an occasional methamphetamine abuser over 1 to 2 months, who presented with the sudden onset of bifrontal headache, nausea and vomiting immediately after an intravenous injection of methamphetamine. Imaging studies demonstrated intracerebral hemorrhage and subdural hemorrhage associated with cerebral vasculitis. To our knowledge, this entity has not been previously reported in Korea.

Transesophageal Echocardiographic Findings in Stroke Subtypes

Although transesophageal echocardiography (TEE) is known to be useful in detecting a possible embolic source in patients with ischemic stroke, its usefulness in various stroke subtypes remains still uncertain. We reviewed the clinical findings and TEE records of 191 patients with various stroke subtypes. We classified them into four stroke subtypes- cardiac embolism, lacunar infarct, large artery disease and stroke of unclear etiology. TEE parameters such as left atrial thrombus, spontaneous echo contrast, atrial septal aneurysm, interatrial shunts, ventricular thrombus or aneurysm, myxomatous mitral valve or mitral valve prolapse and atheromatous plaque on ascending aorta or aortic arch were compared among different stroke subtypes. The yield of TEE was subtantial in all stroke subtypes. Left atrial thrombi and spontaneous echo contrast were frequent in patients with high-risk cardiac embolism-21.4% and 42.9%, respectively, whereas only one patient had spontaneous echo contrast in other subtypes. Interatrial shunt was common in all stroke subtypes and was found more frequently in stroke of unclear etiology (42. 9%) compared to other subtypes (27. 2%) (P=O.02, X2). Other parameters were detected in only a few patients. TEE seems to be a useful procedure in patients with high-risk cardiac embolism, and the clinical significance of interatrial shunt in patients with stroke of unclear etiology remains to be determined.