ABSTRACT
No abstract available.
Subject(s)
Humans , Antipsychotic Agents , Dystonia , HIV , HIV Infections , SchizophreniaABSTRACT
Tolosa-Hunt syndrome is a painful ophthalmoplegia due to nonspecific granulomatous inflammation in the cavernous sinus. A 16-year-old man developed Tolosa-Hunt syndrome accompanied by facial nerve palsy. MRI showed an inflammatory lesion in the cavernous sinus with gadolinium enhancement. This rare presentation suggests that Tolosa-Hunt syndrome is a localized form of idiopathic hypertrophic pachymeningitis.
Subject(s)
Adolescent , Humans , Cavernous Sinus , Facial Nerve , Gadolinium , Inflammation , Ophthalmoplegia , Paralysis , Tolosa-Hunt SyndromeABSTRACT
Carbamazepine (CBZ) may alter the secretion of prolactin (PRL), however, there is no report about hyperprolactinemia with galactorrhea induced by CBZ medication. A 30-year-old woman presented with galactorrhea. She had been treated with CBZ for three years and hormonal study revealed increased serum PRL levels. Sella MRI showed no evidence of pituitary lesion. Two months after changing CBZ to oxcarbazepine, galatorrhea was improved and the serum PRL level was normalized.
Subject(s)
Adult , Female , Humans , Pregnancy , Carbamazepine , Galactorrhea , Hyperprolactinemia , Magnetic Resonance Imaging , ProlactinABSTRACT
A 58-years-old man came to the hospital because of progressive intellectual deterioration, gait disturbance and postural instability for 6 months. Neuropsychological tests showed severe impairment of memory and executive functions, and dural arteriovenous fistula of the superior sagittal sinus (dAVF of SSS) was found on brain MRI and angiography. The symptoms were markedly improved after selective transvascular embolization treatments. Even though the dAVF of SSS is a rare cause of dementia, we suggest that it can be one of the treatable causes of dementia.
Subject(s)
Angiography , Brain , Central Nervous System Vascular Malformations , Dementia , Executive Function , Gait , Magnetic Resonance Imaging , Memory , Neuropsychological Tests , Superior Sagittal SinusABSTRACT
Leigh syndrome (LS) is a genetically and clinically heterogeneous disorder caused by metabolic defects affecting lactate/pyruvate metabolism. The consequence of the metabolic defects are decreased amounts of APT and basic cell energy productions of the nervous system. In LS, several mutations have been reported in both the nuclear and the mitochondrial genome. Here, we report a 26-year-old woman clinically diagnosed with LS having characteristic brain MR and MRS abnormalities but without known definite pathogenetic mitochondrial DNA mutations.
Subject(s)
Adult , Female , Humans , Brain , DNA, Mitochondrial , Genome, Mitochondrial , Leigh Disease , Magnetic Resonance Imaging , Metabolism , Mitochondrial Diseases , Nervous SystemABSTRACT
Segmental myoclonus can be seen in variable lesions of the brainstem or spinal cord, but the pathophysiology of the segmental myoclonus is not fully defined yet. We describe three patients with delayed developed and chronically persisted involuntary movement restricted to one arm after mild cervical injury. Myoclonus developed 1 month later in 2 patients and the other 5 months later after the injury. They suffered from myoclonus for more than 2 months, 1 year, and 25 years, respectively. Clonazepam and phenytoin were tried, but not satisfactory.
Subject(s)
Humans , Arm , Brain Stem , Clonazepam , Dyskinesias , Myoclonus , Phenytoin , Spinal CordABSTRACT
Segmental myoclonus can be seen in variable lesions of the brainstem or spinal cord, but the pathophysiology of the segmental myoclonus is not fully defined yet. We describe three patients with delayed developed and chronically persisted involuntary movement restricted to one arm after mild cervical injury. Myoclonus developed 1 month later in 2 patients and the other 5 months later after the injury. They suffered from myoclonus for more than 2 months, 1 year, and 25 years, respectively. Clonazepam and phenytoin were tried, but not satisfactory.
Subject(s)
Humans , Arm , Brain Stem , Clonazepam , Dyskinesias , Myoclonus , Phenytoin , Spinal CordABSTRACT
The human rabies is a fatal infectious disease invading the central nervous system. There are many cases of clinically suspicious human rabies encephalitis in Korea since 1906. A 53-year old man was admitted to the Department of Neurology because of rapidly progressive dysphagia. His sputum was examined and the rabies virus was found by the polymerase chain reaction technique. So we report a case of human rabies encephalitis confirmed by detection of the rabies virus from his saliva.
Subject(s)
Humans , Middle Aged , Central Nervous System , Communicable Diseases , Deglutition Disorders , Encephalitis , Korea , Neurology , Polymerase Chain Reaction , Rabies virus , Rabies , Saliva , SputumABSTRACT
A 31-year-old male patient was admitted to the department of neurology for progressive spastic paraparesis and sen-sory deficit in bilateral lower legs. On review of system, he had decreased libido and mild urinary frequency. On neuro-logic examination, he showed paraparesis, lower limb hypesthesia, bilateral hyperactive knee jerk and bilateral positive Babinski sign. The nerve conduction studies were sensori-motor demyelinating polyneuropathy. For spastic paraparesis and peripheral neuropathy, we approached adrenomyeloneuropathy and ascertained elevated serum very long chain fatty acids (VLCFA) level. (J Korean Neurol Assoc 19(4):427~430, 2001)
Subject(s)
Adult , Humans , Male , Adrenoleukodystrophy , Fatty Acids , Hypesthesia , Knee , Leg , Libido , Lower Extremity , Neural Conduction , Neurology , Paraparesis , Paraparesis, Spastic , Peripheral Nervous System Diseases , Polyneuropathies , Reflex, BabinskiABSTRACT
Tuberculomas in the spine are estimated to be 15 to 50 times less common than those occurring in the cranium. We experienced a case of intramedullary spinal tuberculoma and brain tuberculoma associated with pulmonary tuberculosis. A 39-year-old male was referred to the National Medical Center via emergency room because of urinary difficulty and lower limb weakness for 3 days. He had been treated with anti-tuberculosis regimens against pulmonary tuberculosis for 20 days. Spinal MRI revealed intradural intramedullary tuberculoma at T5. On the 21st day at the hospital, a generalized seizure attacked him. Brain MRI revealed multiple tuberculoma in both hemispheres, brainstem and cerebellum. He was treated anti-tuberculosis regimens and corticosteroids for 9 months. His condition improved clinically and radiologically. We report this case with a review of the literature.
Subject(s)
Adult , Humans , Male , Adrenal Cortex Hormones , Brain Stem , Brain , Cerebellum , Emergency Service, Hospital , Lower Extremity , Magnetic Resonance Imaging , Seizures , Skull , Spine , Tuberculoma , Tuberculosis, PulmonaryABSTRACT
Myasthenia gravis has been associated with a variety of autoimmune diseases, but the association of myasthenia gravis with idiopathic thrombocytopenic purpura has been rarely reported. A previously healthy 33-year-old female presented with a 10-month history of easy fatigability, motor weakness, diplopia and menorrhage. Clinical and labora-tory findings supported the diagnosis of myasthenia gravis and idiopathic thrombocytopenic purpura. After a thymecto-my, there were surprising improvements not only in muscle strength, but also of thrombocytopenia. Though this associ ?ation may be incidental, the patient's progress suggests that idiopathic thrombocytopenic purpura may be closely relat-ed to myasthenia gravis immunologically because both diseases developed simultaneously and showed good therapeu-tic responses after a thymectomy.
Subject(s)
Adult , Female , Humans , Autoimmune Diseases , Diagnosis , Diplopia , Muscle Strength , Myasthenia Gravis , Purpura, Thrombocytopenic, Idiopathic , Thrombocytopenia , ThymectomyABSTRACT
A 7 year-old girl presented with generalized muscle weakness and delayed motor development. She was able to stand up at 15 months and began to walk at 4 years of age. A nerve conduction study showed severe demyelinating neuropa-thy .There was no family history of peripheral neuropathy, and her parents and younger brother were clinically and electrophysiologically normal. A sural nerve biopsy showed moderate loss of myelinated fibers with onion-bulb forma-tions. Many teased nerve fibers revealed typical tomaculous changes. However the molecular genetic study of the patient confirms the duplication of 17p11.2-p22 on a polymerase chain reaction using D17S261 as a primer but not in her parents.
Subject(s)
Child , Female , Humans , Biopsy , Charcot-Marie-Tooth Disease , Genotype , Molecular Biology , Muscle Weakness , Myelin Sheath , Nerve Fibers , Neural Conduction , Parents , Peripheral Nervous System Diseases , Phenotype , Polymerase Chain Reaction , Siblings , Sural NerveABSTRACT
Subacute sensory neuropathies associated with primary Sjogren's syndrome have been reported rarely. We describe a woman with primary Sjogren's syndrome who developed a widespread, pure sensory neuropathy with a subacute onset. An electrophysiological study showed the typical absence or decreased amplitude of sensory nerve action potentials (SNAPs). A sural nerve biopsy showed a loss of large myelinated fibers and axonal degeneration without inflammation. The clinical course of long-standing subacute sensory neuropathy, the biopsy-documented axonal degeneration, and the neurophysiological findings suggest involvement of the dorsal root ganglia.
Subject(s)
Female , Humans , Action Potentials , Axons , Biopsy , Ganglia, Spinal , Inflammation , Myelin Sheath , Peripheral Nervous System Diseases , Sjogren's Syndrome , Sural NerveABSTRACT
We report a case of decreased cerebrospinal fluid glucose with lymphocytic pleocytoisis in a patient with herpes zoster meningoencephalitis. The finding was likely to be confused with that of tuberculous meningoencephalitis. The concentration of CSF glucose is a critical point in the differential diagosis of various causes of nervous system infection. Although the herpes zoster meningoencephalitis isa well recognized, cases with markedly low, level of CSF glucose has been rare. We reviewed such unusual cases in the literature that were accompanied by hypoglycorrhachia. The duration of hypoglycorrhachia was transient. This suggests a differential point from that of tuberculous meningitis.
Subject(s)
Humans , Cerebrospinal Fluid , Encephalitis, Varicella Zoster , Glucose , Herpes Zoster , Meningoencephalitis , Nervous System , Tuberculosis, MeningealABSTRACT
Rhus has been known to one of the most frequent plants which produce allergic contact dermatitis. Allergenic component of thus is urushiol which is composed of catechols with a 15-carbon-atom side-chain. Systemic administration can produced hematogenous contact dermatitis and also severe systemic reactions such as drowsiness, convulsion, nausea, vomiting, glomerulonephritis and urticaria. We experienced a case of right anterior and posterior watershed cerebral infarction, generalized urticaria and allergic contact dermatitis following ingestion of urushiol.
Subject(s)
Catechols , Cerebral Infarction , Dermatitis, Allergic Contact , Dermatitis, Contact , Eating , Glomerulonephritis , Nausea , Rhus , Seizures , Sleep Stages , Urticaria , VomitingABSTRACT
In nonsystemic vasculitic neuropathy, clinically only nerves are affected : there are no or few constitutional symptoms or serological abnormalities. The clinical and pathological features are those of an ischemic neuropathy caused by a necrotizing vasculitis of small arterioles. This is a case report of nonsystemic vasculitic neuropathy in a 22-year-old male who has the distal symmetric poIyneuropathy as a symptom of localized vsaculitis. The sural nerve biopsy showed a few myelin digestion chamber suggesting axonal degeneration. The patient was treated with steroid daily with gradual clinical and electorophysiological improvement.
Subject(s)
Humans , Male , Young Adult , Arterioles , Axons , Biopsy , Digestion , Myelin Sheath , Sural Nerve , VasculitisABSTRACT
Adrenoleukodystrophy is an inborn error of metabolism characterized by adrenal insufficiency and progressive demyelmation of brain white matter and peripheral nerves. Authors experienced three cases of adrenoleukodystrophy in a 7 year old boy, a 17 and a 210 year old males that were diagnosed by increased plasma content of very long chain fatty acid(VLCFA). The clinical symptoms include seizure, visual impairment, and hemiparesis. Two cases showed typical radiological findings and sural nerve biopsy was performed in one.
Subject(s)
Child , Humans , Male , Adrenal Insufficiency , Adrenoleukodystrophy , Biopsy , Brain , Metabolism , Paresis , Peripheral Nerves , Plasma , Seizures , Sural NerveABSTRACT
Solitary plasmacytoma, in contrast to the disseminated neoplastic proliferation of plasma cells with marked infiltration of multiple organ system in multiple myeloma, is plasma cell neoplasm of a single focus occuring either in bone or soft tissue. The association between a solitary plasmacytoma and peripheral neuropathy is rare, and it is a progressive sensorimotorneuropathy, with a raised CSF protein and mixed demyelination and axonal loss in nerve biopsy. Localized radiotherapy indeed proves to be effective of not only arresting the progress of the neuropathy but also allowing a degree of recovery. We experienced a 55-year-old male with a solitary plasmacytoma and peripheral neuropathy confirmed by the radiologic studies, immunohistochemical stain of nasopharyngeal mucosa biopsy and sural nerve biopsy, which has loss of myelinated fiber and axonal degeneration. Until now, the reported cases are very rare in Korea, so we presented a case of solitary plasmacytoma associated with peripheral neuropathy.