Polymorphism and disorder of poly(dA).poly(dT) in fibers

OBJECTIVE: The purpose of this report is to describe the clinical history, treatment, pathology, and imaging in two cases of rare CNS infection caused by free-living amebas. The Naegleria fowleri and Acanthamoeba species cause primary amebic meningoencephalitis and granulomatous amebic encephalitis, respectively. We describe the neuroimaging findings of a case involving a nonspecific cerebral edema pattern in primary amebic meningoencephalitis and a case involving focal enhancing lesions in granulomatous amebic encephalitis. CONCLUSION: Primary amebic meningoencephalitis and granulomatous amebic encephalitis have a grave prognosis and, although rare, should be considered in the differential diagnosis for patients who present with appropriate histories and imaging findings, including nonspecific brain edema on CT in primary amebic meningoencephalitis and focal punctate enhancing lesions in the posterior cranial fossa on T1-weighted MR imaging in granulomatous amebic encephalitis.     

PCR on cerebrospinal fluid to show influenza-associated acute encephalopathy or encephalitis

BACKGROUND: Except for Reye's syndrome, influenza-associated acute encephalopathy or encephalitis is not universally recognised. We did a multicentre study of laboratory and clinical data for patients with influenza-associated acute encephalopathy or encephalitis. METHODS: In Nagoya, Japan, ten patients with acute encephalopathy or encephalitis associated with influenza-like illness were admitted to our hospitals between April, 1996, and March, 1997. We collected clinical, laboratory and serological data and assessed cerebrospinal fluid samples by PCR for influenza A and B. FINDINGS: Seven patients, aged 22 months to 4 years, had evidence of recent influenza infection, six with type-A/Hong Kong (H3N2) and one with type B. The first sign in the central nervous system appeared within 2 days of fever in all but one patient. The first sign of involvement of the central nervous system was generalised convulsions in all patients. Two patients died, one had sequelae, and four survived without sequelae. PCR for influenza type A was positive for five patients. INTERPRETATION: The results of PCR suggest that at least part of the influenza type A genome existed in the central nervous system. Influenza-associated acute encephalopathy or encephalitis in young children deserves wider recognition.     

Glomus tumor of the temporal bone

A case story describing the typical symptoms and course of a glomus tumour of the temporal bone is presented. The most frequent symptoms are pulsatile tinnitus, unilateral hearing loss, aural fullness and paresis of the vagal nerve or other lower cranial nerves. The tumour is frequently visible by otoscopy. Despite being histologically benign, the tumour is infiltrative and may affect the surrounding cranial nerves or spread into the cranial cavity. The early signs and findings are vague. Since the sequelae are fewer when the tumour is treated while it is small, an increased awareness will be of benefit to the patients.     

Clinical features of anterior inferior cerebellar artery territory infarcts--a study of ten patients

Clinical features of the anterior inferior cerebellar artery (AICA) territory infarcts were investigated in ten patients, ranging in age from 38 to 76 years. In all patients, there were MR images of infarction located in the area supplied by the AICA. The lesion was on the left side in 6 patients and right side in 4. The lesion of brain stem including the middle cerebellar peduncle was found in 7 patients and that extended to the cerebellum was in 3 patients. The main ipsilateral neurological signs were the VII and VIII cranial nerves palsy and cerebellar ataxia. The V and VI cranial nerves palsy. Horner's syndrome, and dysphagia were also present. The main contralateral sign was superficial sensory disturbance, but no hemiplegia. The underlying pathology included chiefly hyperlipidemia, hypertension, and diabetes mellitus. Cerebral angiography was performed in 8 patients, most of which was observed severe arteriosclerosis suggesting poor hemodynamics in the vertebral and basilar arteries. The prognosis was relatively good, but the VII, VIII, and V cranial nerves palsy and contralateral superficial sensory disturbance remained as the sequelae. As mentioned above, there were various neurological findings and MR images in AICA territory infarcts. Especially there were some patients whose lesion extended to the upper medulla and neurological findings were similar to the Wallenberg syndrome. It is important that one investigates not only axial slices but also coronal slices of MR image to estimate the extension of AICA territory infarct.     

Single-photon emission CT findings in acute Japanese encephalitis

PURPOSE: To determine the usefulness of single-photon emission CT (SPECT) in the diagnosis of acute Japanese encephalitis (JE). METHODS: We examined 10 patients (six men and four women; mean age, 69 years) with viral encephalitis. We divided the cases into two groups: the JE group (n = 4) and the non-JE group (n = 6; two with herpes simplex encephalitis and four with encephalitis of unknown origin). All cases were investigated with 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT within 15 days after the onset of symptoms. Two patients in the JE group were also examinated by SPECT at a later stage. In all cases MR imaging was performed after the SPECT study. RESULTS: In the acute stage, all patients in the JE group showed a marked increase of HMPAO uptake that matched the hyperintense area observed on MR images in the thalami and putamina bilaterally. Follow-up SPECT studies of two patients with JE revealed a decrease of HMPAO deposition in the areas of high uptake. None of the patients in the non-JE group had an increased accumulation of HMPAO in the thalami or the putamina. CONCLUSIONS: SPECT is helpful in differentiating JE from herpes simplex encephalitis and other types of encephalitis. SPECT may be useful as a diagnostic tool in the early stages of JE.     

Delineation of small nerves and blood vessels with three-dimensional fast spin-echo MR imaging: comparison of presurgical and surgical findings in patients with hemifacial spasm

BACKGROUND AND PURPOSE: We applied a 3D fast spin-echo (3D-FSE) MR imaging technique to the preoperative and postoperative evaluation of patients with hemifacial spasm. METHODS: The study group comprised 20 patients. All images were acquired on a 1.5-T MR system with a 3D-FSE sequence. RESULTS: In all 20 patients, the courses of the seventh and eighth cranial nerves were depicted separately, and the arteries presumed to be responsible for the hemifacial spasm were seen to be in contact with the facial nerves at the root exit zone (REZ). Eight patients underwent neurovascular decompression. In all patients, the presumed responsible blood vessels depicted by 3D-FSE MR imaging corresponded to intraoperative findings. In addition, postoperative 3D-FSE images confirmed the separation of the facial nerve from a contiguous vessel at the REZ. DISCUSSION: The 3D-FSE technique makes it possible to obtain extremely high-quality images of microstructures in the cerebellopontine cistern, and it has several advantages over conventional angiography: it is noninvasive and able to depict the cranial nerves and surrounding vessels in the same image without contrast material, and it may be useful for postoperative evaluation of the decompression procedure. This imaging technique is expected to prove useful for the clinical evaluation of hemifacial spasm.     

Prognostic factors of early sequelae and fatal outcome of Japanese encephalitis

A clinical case control study to identify prognostic factors present at hospital admission associated with early sequelae and fatal outcome of acute Japanese encephalitis (JE) was carried out in Gusi county, Henan Province, central China from June to September 1991. A total of 70 patients with laboratory-confirmed acute JE were studied, of whom 3 cases died and 33 cases had neurological or psychiatric sequelae at the end of three months follow-up. The results showed that acute JE at younger age, with higher body temperature, high white cell count in CSF, and deep coma present at hospital admission were markers for unfavorable outcomes (sequelae or fatal). A history of the vaccination was not correlated with the early sequelae and fatal outcome of the disease. The paper suggests that early diagnosis and treatment and universal JE vaccination for all susceptible populations are keys for decreasing incidence of sequelae and fatal outcome of acute JE.     

Breast implants

We describe the case - to our knowledge unique - of an 8-year-old boy who presented with acute onset of lower cranial nerve palsy and tetraparesis caused by a hematoma in a dorsal exophytic pilocytic astrocytoma of the medulla oblongata. The boy showed near-complete recovery after neurosurgical management in two stages: first, emergency evacuation of the hematoma with tumor biopsy, and second, complete tumor removal 5 months after the initial event. Intraoperative electrophysiological monitoring techniques for the lower cranial nerves are of value in preserving their functional integrity. Ultrasonography is helpful in assessing the extent of tumor removal. Although the pathological diagnosis of a pilocytic astrocytoma would not justify radiotherapy, local field radiotherapy was added mainly because of the unexpectedly rapid tumor progression during the interval between the two surgical procedures. The literature on brainstem and tumor hematoma in children is reviewed.     

Diethylstilbestrol in beef production: what is the risk to consumers?

Acute measles encephalitis with severe sequelae in a 25-year-old man was studied. A transient appearance of oligoclonal IgG in cerebrospinal fluid and of intrathecally produced measles antibodies was found during 2 months after the onset of the disease. On the basis of this finding of local hyperimmunization it is proposed that in the case studied the measles virus infection may have been directly responsible for the disease process in the central nervous system.     

Intensity of MR contrast enhancement does not correspond to clinical and electroneurographic findings in acute inflammatory facial nerve palsy

PURPOSE: To determine the value of MR contrast enhancement in predicting the course of acute inflammatory facial nerve palsy and in selecting patients for surgical decompression. METHODS: Six patients with an acute inflammatory incomplete or complete peripheral facial nerve palsy (five idiopathic and one herpetic in origin) had repeated MR imaging studies with and without contrast enhancement, electroneurography, and clinical examinations to establish a connection between the intensity of contrast enhancement on MR images, the clinical condition, and the electrophysiological data. The examinations were performed every second day starting on the first day of admission until clinical recovery was proved by clinical deblockage (spontaneous clinical improvement). The last examination was performed 3 months after the onset of the facial nerve palsy. RESULTS: An abnormal, very intense contrast enhancement of the facial nerve was always present in the distal intrameatal and proximal tympanic segments and in the geniculate ganglion. The labyrinthine segment exhibited a mild to moderate enhancement, and the distal tympanic and mastoid segments showed a moderate to intense enhancement. The intensity of contrast enhancement did not correspond to the severity, duration, or course of the facial nerve palsy, and the electroneurographic data had no predictive value in indicating the severity of the inflammatory process. Three months after clinical recovery, a persistent and more or less unchanged or even slightly more intense contrast enhancement was observed. CONCLUSION: The long-lasting intense contrast enhancement seen in the facial nerve segments of patients who have acute peripheral inflammatory facial nerve palsy is explained by a two-phase breakdown of the blood-nerve barrier.     

Myelitis and optic neuritis caused by varicella

INTRODUCTION: Varicella mainly affects children between 1 and 14 years old. It is the initial infection caused by the Varicella-Zoster virus. It is characterized by a vesicular cutaneous eruption, fever and generally good prognosis. The neurological complications caused by the Varicella-Zoster virus are infrequent and include: meningitis, encephalitis, cerebellar ataxia, Reye's syndrome, myelitis, optic neuritis, mononeuropathy, polyneuropathy, necrosis of the retina and cerebral arteritis. CLINICAL CASE: We present the unusual case of a woman patient aged 18 who presented with myelitis 15 days after having a varicella rash. Analysis of the cerebrospinal fluid showed intrathecal production of antibodies against the Varicella-Zoster virus. Fourteen days after resolution of the myelitis, she presented with unilateral optic neuritis which remitted without sequelae, (as did the myelitis). Cerebral and medullary MR showed no alterations. CONCLUSIONS: The pathogenesis leading to involvement of the nervous system is still not well defined. Direct invasion by the virus has been postulated, particularly in Herpes-Zoster (reinfection by Varicella-Zoster), as immunological phenomena which may be more frequent with Varicella (initial infection by Varicella-Zoster virus). In our case there were two short episodes of neurological involvement: optic neuritis and myelitis, with a satisfactory clinical course after giving corticosteroids. This makes one think of immunological mechanisms rather than direct invasion of the central nervous system by the Varicella-Zoster virus.     

Spontaneous spinal extradural hematomas: report of two cases

INTRODUCTION AND CLINICAL CASES: We present two cases of spontaneous spinal extradural hematoma, which occurred in 1996 and were admitted to and operated on by our Department. Both patients were women. No cause for the bleeding was found in either case. Both presented with the site of the lesion in the thoracic spine, although at different levels. There was acute onset of the clinical condition. The best diagnostic procedure was MR. The treatment of choice was surgical and the results related to the rate of onset of the clinical picture and the time elapsed before operation. CONCLUSION: The form of presentation is discussed, together with the importance of early diagnosis and the relation to emergency surgical treatment.     

Delayed sequelae after acute overdoses or poisonings: cranial neuropathy related to ethylene glycol ingestion

A 31-year-old woman came to the hospital with breathlessness, confusion, and a refractory anion gap metabolic acidosis; acute renal failure subsequently developed. Her blood ethylene glycol concentration was 390 mg/L, and she was treated with an intravenous ethanol infusion and hemodialysis. During the tenth and eleventh day after admission bilateral seventh cranial nerve paralysis developed, as well as bilateral dysfunction of cranial nerves II, V, VIII, IX, X and XII. Magnetic resonance imaging of her head showed gadolinium enhancement of the fifth cranial nerve bilaterally and a communicating hydrocephalus. Over the subsequent 11 months she recovered full function of her cranial nerves V, VII, IX, X, and XII, and she had subjective clinical improvement to baseline function in cranial nerves II and VIII. This case serves to introduce a discussion of agents that cause delayed complications after their acute toxic ingestion.     

Acute encephalitis and bacterial meningitis in children in Aichi Prefecture (1984-1993)

A survey was performed of acute encephalitis and bacterial meningitis in infancy and childhood from 1984 to 1993 using a questionnaire directed to departments of pediatrics in large hospitals in Aichi prefecture. The case records for 391 patients with acute encephalitis including related diseases and 328 patients with bacterial meningitis were obtained from 63 hospitals. Of 391 patients with acute encephalitis, 224 were male and 167 were female. Of 328 patients with bacterial meningitis, 200 were male and 128 were female. Sex ratio were 1.3, 1.6 respectively. Of the patients, 52.4% of encephalitis and 84.8% of meningitis were under 4 years of age and 0 year olds made up 53.7% of the latter. The causes of these diseases were confirmed in 38.7% and 82.9% of the encephalitis and meningitis patients, respectively, etiologically. In encephalitis, rubella virus was the most frequent with 29 cases, followed by measles virus (27 cases), herpes simplex virus (24) and varicella-zostervirus (19). In meningitis H. influenzae (95 cases), S. pneumoniae (56), Group B streptococcus (41) and E. coli (27) were frequently diagnosed. These diseases showed respective patterns of age distribution and clinical course, and moreover, the increases in their onset were clearly related to the prevalence of causal infections. Therefore, the results of this study should be utilized in the development of administrative measures for prevention of these diseases.     

Third cranial nerve palsy caused by gummatous neurosyphilis: MR findings

The clinical and MR findings in an unusual case of gummatous neurosyphilis are reported. A 44-year-old woman suffering from diplopia and right-sided headaches was admitted. Physical examination and routine laboratory parameters were normal except for a third-nerve palsy. MR images revealed a contrast-enhancing lesion of the upper brain stem and third cranial nerve. Differential diagnosis included neuroma of the third cranial nerve, as well as neurosarcoidosis and other inflammatory processes. Serologic tests and lumbar puncture revealed the presence of active syphilis. After intravenous treatment with penicillin G, follow-up MR examinations showed diminishing size of the lesion with its complete resolution within 3 months.     

Fusiform P1 segment artery aneurysm in a pediatric patient: technical case report

We report a case of cavernous sinus thrombosis in an 8-year-old boy, following a neglected and untreated nasal furuncle. This rare entity is manifested by hyperthermia, with headache, vomiting and involvement of the III-IV & VI cranial nerves. It is usually a complication of a facial infection. The diagnosis is established by CT-Scan or MRI. Without appropriate and early treatment, the prognosis is poor, with a fatal outcome or severe sequelae.     

Long-term astigmatic changes after clear corneal cataract surgery

Ramsay Hunt's syndrome is an infectious cranial polyneuropathy caused by varicella zoster, the herpetic virus that also causes chickenpox and shingles. Its symptoms include facial paralysis, ear pain, and an auricular rash. Oral lesions are also present in most cases. This syndrome can affect any cranial nerve and usually affects multiple nerves, causing central, cervical, and peripheral effects. This article reports the case of a 35-year-old white female who was treated by the oral surgery service of a large urban hospital, after first reporting to the emergency clinic. Her reported symptoms of unilateral left-side facial paralysis, auricular pain, and trigeminal hyperesthesia were confirmed by clinical examination. An initial short low-dose steroid regimen was unsuccessful. A second daily dosage of 50 mg of prednisone was successful in 21 days. No permanent sequelae were evident or reported after treatment.     

Measles and its complications in Hamburg from 1960 to 1973 (author's transl)

A study of measles was undertaken from the case histories of 3 093 children under 15 years of age, treated in the children's clinics of Hamburg between 1960 to 1973. 562 children were suffering from pneumonia (18,2%), 230 had otitis (7,4%) and 229 (7,4%) showed neural affections (143 cases with fits and 86 patients with encephalopathy or encephalitis), Various parameters were analysed: age, sex, seasonal distribution, fatality, age disposition to convulsions and measles encephalitis, "incubation period" of neural affections, sequelae. Permanent sequelae due to measles encephalitis were seen in 14% of the cases. A plea is made for an universal immunisation against measles.     

Biodistribution study of the anaesthetic sodium phenobarbital labelled with technetium-99m in Swiss mice infected with Schistosoma mansoni Sambon, 1907

The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.     

Jugulotympanic paraganglioma: therapy concepts under development

BACKGROUND: The operative treatment and radiation therapy of jugulotympanic paragangliomas (JTP) are still a matter of controversial discussion. In spite of various improvements during the last 50 years, selecting the appropriate treatment modality (surgery, radiation, or observation) is still a challenge. PATIENTS: During a 16-year period, 44 patients with 45 JTP (10 at level A/B and 35 at level C/D according to Fisch) were seen at the ENT-department in Fulda. Forty-one cases were treated surgically. RESULTS: Complete resection was possible for level A/B in 100% of the patients (n = 10). Residual tumor was demonstrated for level C in 23% of the patients (5/22) and for level D in 40% (4/10) with a median follow-up time of 69 months. In two cases residual tumor was treated by radiation. Six patients with residual paraganglioma tissue were maintained under observation without any evidence of tumor progression (median follow-up time 39 months). We report one death after the attempt to resect a large residual paraganglioma that had already caused brain stem compression. A sufficient duraplasty could not be achieved following radiation therapy. CONCLUSIONS: Complete tumor resection of jugulotympanic paragangliomas of levels A and B is often possible without injury to the cranial nerves. Extensive tumors present difficulties in complete tumor resection and increase the risk of cranial nerve injuries. Advanced paragangliomas therefore require an individualized therapeutic regime including surgery, radiation therapy, and observation of tumor growth.