Isolated third nerve palsy due to sarcoidosis

We present a 28-year-old man with isolated third nerve palsy as the initial manifestation of sarcoidosis. MRI demonstrated abnormal enhancement of the cisternal portion of the right third nerve. One month after steroid therapy, he showed almost complete recovery with resolution of the contrast enhancement on MRI. This patient illustrates the usefulness of enhanced MRI for detection of cranial nerve lesions of sarcoidosis.     

Semistructural allografting in bone defects after curettage

Amongst the focal and multifocal neuropathies that are associated with diabetes mellitus one of the most common is a proximal predominantly motor lower limb neuropathy. Recent evidence has indicated that, at least in a proportion of cases, this may have an inflammatory basis. We have examined a consecutive series of 15 cases of proximal diabetic neuropathy (diabetic amyotrophy). These were characterized by proximal pain and asymmetric proximal or generalized lower limb muscle weakness, associated in some cases with radicular sensory involvement. Two-thirds of the patients had an accompanying distal symmetric sensory polyneuropathy. Biopsy of the intermediate cutaneous nerve of the thigh, a sensory branch of the femoral nerve, showed epineurial microvasculitis in 3 patients and nonvasculitic epineurial inflammatory infiltrates in another case. In a further case, microvasculitis was found in both in the sural nerve and a quadriceps muscle biopsy specimen. The detection of inflammatory changes appeared to be correlated with the occurrence of sensory radicular involvement. Whether similar changes are present in muscle nerves in this predominantly motor syndrome requires further study. Nevertheless, the present observations confirm the view that secondary vasculitic or other inflammatory reactions may contribute to some forms of diabetic neuropathy.     

MRI findings in patients with acute autonomic and sensory neuropathy

Acute autonomic and sensory neuropathy (AASN), characterized by acute onset of extensive autonomic dysfunction and severe sensory deficits, was first described by Colan et al. (1978). We present two female patients with AASN in whom magnetic resonance imaging (MRI) confirmed such findings in the posterior column of the spinal cord. One patient was a 44-year-old woman who developed an upper respiratory tract infection followed in 2 weeks by numbness of the limbs and gait disturbance. There was orthostatic hypotension with syncope, paretic ileus, anhidrosis and urinary retention. There was a loss of sensation over the entire body, including the face, and deep tendon reflexes were generally absent. Neurophysiologic studies showed that sensory nerve action potentials and SSEPs were not evoked in the nerves examined. Sural nerve biopsy demonstrated severe axonal degeneration of the myelinated and unmyelinated fibers. Our second patient, a 27-year-old woman, exhibited similar clinical and laboratory features. The autonomic dysfunction in both patients improved gradually without drug treatment, but the sensory deficits--predominantly a loss of deep sensation--persisted for several years. In both patients, MRI revealed the T2*-weighted high intensity area in the fasciculus gracilis of the posterior column of the spinal cord. Such high intensity areas were present in all spinal segments. The severe and persistent sensory disturbance in these patients may have been caused by a lesion of the posterior column of the spinal cord following the involvement of the dorsal root ganglion cells, or ganglioneuronopathy, as demonstrated by MRI.     

The clinical significance of gadolinium enhancement of lumbar disc herniations and nerve roots on preoperative MRI

The clinical significance of preoperative gadolinium DPTA enhancement around disc herniations and in the epidural space on MRI is not clear. The relation of nerve root enhancement to dysfunction also remains controversial. To investigate the clinical significance of contrast enhancement we looked at the symptoms and signs and gadolinium DPTA-enhanced images of 71 consecutive surgical candidates in a standardised fashion. Annular rupture and disc enhancement were found to be closely related, and correlated with sensory loss and paresis, respectively. Nerve root enhancement was found to be related to neurological deficits in general and sensory impairment in particular.     

F-18-FDG uptake in tuberculosis

We report a 7-year-old boy with neuroborreliosis presenting with headache and bilateral facial nerve palsy. MRI demonstrated tentorial and bilateral facial and trigeminal nerve enhancement.     

Ki-67 immunostaining and prognosis in laryngeal cancer

A 7-year-old male Husky dog developed atrophy of the right masseter muscle and pruritus of the right side of the face. A myogenic origin was excluded using muscular biopsy. Electrophysiologically, there was involvement of the motor and sensory fibers of the trigeminal nerve, suggesting a lesion located between the brainstem and the trigeminal ganglion. On MRI examination, a nodular mass was detected in the right caudal fossa. This mass was characterized by intense enhancement after injection of contrast medium. Because of the progressive clinical signs, electrophysiology, and MRI results, a presumptive diagnosis of a trigeminal nerve schwannoma was made. The animal's condition improved slightly with corticosteroids. The dog underwent euthanasia 3 months after initial presentation. Necropsy was not performed.     

Neuritis of multiple cranial nerves in idiopathic focal pachymeningitis

A 76 years old patient suffering from insulin dependent diabetes presents a multiple, bilateral and asymmetric cranial neuropathy involving on the left side the cranial nerves VIII, IX with a participation of the efferent autonomic fibers of the nerves VII and IX (xerostomia) and the nerve XII, and involving on both sides the nerves VII and X. Like it is known from literature the nerve XI was spared, probably because of the more caudal emergence from the brainstem. The disease was preceded by a violent otalgia on the left side which was initially interpretated as a malignant external otitis. This affection was associated with an inflammatory syndrome that was easily monitored by the blood sedimentation rate. The course was favorable but marked by fluctuations of the neurological deficits. The MRI shows a pachymeningitis localised at the beginning in the medial fossa and on the cerebellar tentorium, later on the left parieto-occipital convexity. The diagnosis of a focal idiopathic pachymeningitis was confirmed by excluding different other affections like inflammatory, tumoral and infectious diseases, by using MRI examinations, CSF analysis and especially meningeal biopsy. It is a rare inflammatory disease of unknown origin with potentially persistent neurological deficits. We discuss the differential diagnosis and the therapeutical possibilities, which consist in a long term immunosuppression with corticosteroids and azathioprine.     

Gd-DPTA enhanced MRI in Bell's palsy and herpes zoster oticus: an overview and implications for future studies

Magnetic resonance imaging (MRI) is a new and important tool for use in diagnosing and investigating diseases affecting the facial nerve. In recent gadolinium-DTPA enhanced MRI (Gd-MRI) studies it has unequivocally been demonstrated that ipsilateral facial nerve contrast enhancement, predominantly in the meatal portion, is present in both Bell's palsy and herpes zoster oticus. In this overview, the results of MRI studies performed on patients with acute peripheral facial palsy, especially Bell's palsy and herpes zoster oticus, are discussed. The Gd-MRI pattern in Bell's palsy is very similar to that seen in herpes zoster oticus, and the findings reported so far support the theory that an inflammation may be the cause of the nerve injury in both cases. So far, however, Gd-MRI has not been helpful in evaluating the severity and/or prognosis of the facial palsy. Further studies employing improved techniques, including three-dimensional fast (or turbo) spin echo (3DFSE) MRI with heavily T2-weighted sections and high resolution three-dimensional Fourier transform (3DFT) MRI, need to be conducted in order to determine whether it is possible to follow the course of the disease and whether MRI and/or Gd-MRI are useful prognostic tools in the early stages of palsy.     

Radiologic manifestation of spinal accessory neurinoma: a case report

Spinal accessory nerve neurinoma is very rare; only 12 cases have been reported in the literature. We describe a 54-year-old woman who had a cisterna magna tumor expanding extracranially to the C1 cervical body. The tumor originated from the spinal root of the right accessory nerve. The radiological features including CT, MRI, and angiography are discussed. There have been reports on cranial MRI studies of spinal accessory neurinoma. MRI suggested an extra-axial tumor. The histological diagnosis was mixed Antoni A and B neurinoma. The neuroradiological findings of 12 cases of neurinoma of the accessory nerve reported in the literature are reviewed.     

Facial nerve enhancement on gadolinium-DTPA in a case with neurosarcoidosis

In a case of neurosarcoidosis with bilateral facial nerve palsy and hydrocephalus, contrast-enhanced magnetic resonance imaging (MRI) study and angiotensin converting enzyme (ACE) activities in cerebrospinal fluid (CSF) were valuable for the diagnosis and the follow up. Facial nerve lesions were demonstrated on gadolinium-DTPA enhanced MRI. The disappearance of enhancement was concomitant with the amelioration of facial nerve palsy after corticosteroid therapy.     

MRI findings of VIIth cranial nerve involvement in sarcoidosis

Right facial nerve palsy in a 58-year-old woman was due to sarcoidosis demonstrated by Gd-DTPA enhanced MRI. Abnormal enhancement of the right VIIth cranial nerve in the distal internal acoustic canal was seen on MRI. The enhancing lesion was smaller after 1 month of prednisolone 50 mg day-1. This is the first report on facial nerve involvement in neurosarcoidosis examined by Gd-DTPA enhanced MRI. The use of Gd-DTPA enhanced MRI with thin slicing, e.g. 3 mm slice thickness and 1 mm interslice gap, is effective in detecting small extramedullary lesions.     

Improvement of myelopathy in Sj?gren's syndrome with chlorambucil and prednisone therapy

The authors present a patient with Sj?gren's syndrome with a fluctuating and then progressive myelopathic syndrome, and optic nerve involvement. Treatment with chlorambucil and prednisone improved the patient's function from being wheelchair bound to walking unaided. Spinal MRI showed multiple, extensive intraparenchymal areas of abnormal T2-weighted signal intensity, gadolinium enhancement, and cord swelling, which also improved during the period of treatment.     

Quality assurance in palliative care: some of the problems

Twenty four patients with pure motor neuropathy are reported. The chronic motor involvement associated with fasciculations and cramps, mainly in the arms, led, in most patients, to an initial diagnosis of motor neuron disease. In some patients (nine of 24), there was no appreciable muscle atrophy. Tendon reflexes were often absent or weak. The finding of persistent multifocal conduction block confined to motor nerve fibres raises questions about the nature and the importance of this syndrome. Segmental reduction of motor conduction velocity occurred at the site of the block, but significant slowing of motor nerve conduction was not found outside this site. The response to intravenous IVIg treatment seems to be correlated with the absence of amyotrophy. Patients with little or no amyotrophy had an initial and sustained response to IVIg, and did not develop amyotrophy during the follow up study. They could be considered to have a variant of chronic inflammatory demyelinating polyneuropathy. Patients with pronounced amyotrophy independent of the disease duration did not respond as well to IVIg treatment, suggesting the existence of a distinct entity. Among the patients treated about two thirds who had an initial good response to IVIg had high or significant antiganglioside GM1 (anti-GM1) antibody titres, but there was no correlation between the high titres before treatment and long lasting response to IVIg treatment.     

An unusual skin lesion in a pediatric patient with Wegener's granulomatosis

Cutaneous manifestations occur in a significant number of patients with Wegener's granulomatosis (WG); however, the presentation and histopathology of these lesions are highly variable and may present problems in diagnosis. We report the presentation of a single large skin lesion in a pediatric patient with a history of WG and the characterization of this lesion by magnetic resonance imaging (MRI) and histopathology. MRI was helpful in delineating the extent of the lesion, although a skin biopsy was necessary to confirm the diagnosis of the vasculitic nature of the lesion.     

Intraoperative electron beam radiotherapy for previously irradiated advanced head and neck malignancies

In patients with malignant astrocytomas or metastatic brain disease treated with high-dose radiotherapy, conventional imaging methods may not adequately distinguish recurrent tumor from radiation change. We used a fast spoiled gradient refocusing technique in the open-configuration intraoperative MR system to assess the rate of regional enhancement of the treated tumor bed and to localize specific sites for pathologic sampling to determine whether gadolinium uptake correlated with histologic data. Twenty-four patients were studied. Fourteen of 15 patients with areas of early enhancement had recurrent tumor present in histologic samples, and 8 of the remaining 9 patients had only reactive changes. Dynamic MRI was predictive of recurrent tumor (P < .0005, Fisher exact test and P < .002, Student t test). We conclude that dynamic MRI in the open-bore magnet is a promising method for localizing potential sites of active tumor growth in patients treated for malignant astrocytomas and metastatic brain lesions.     

Epidemiology of soil-transmitted nematode infections in Ha Nam Province, Vietnam

Brain-stem gliomas occur mainly in childhood and are localized in the mesencephalon, pons and medulla oblongata. Diagnosis is a domain of MRI, requiring T2, T1 and KM. CT shows hemorrhage and calcification well. The criteria are the primary site, size, tumor growth, brain-stem enlargement, delineation, intralesional structure, exophytic components and enhancement. Secondary criteria are herniation, hydrocephalus and liquorgenic seeding. In CT glioma are hypodense, in MRI hyperintense in T2 and hypointense in T1. Enhancement is seen in 25-60% and does not allow differentiation of tumor vs nontumor or gradings. Factors influencing poor outcome are high grade, a short history, cranial nerve involvement, severe brain-stem enlargement, pontine site, diffuse growth and recurrency. The 5-year-survival rate is 30% (after radiation: focal tumors 85%, diffuse 20%). Most frequent are symptoms of brain pressure, cerebellum, cranial nerves and pyramidal tract. There is no agreement on whether biopsy is necessary or not. A diagnosis of tumor is highly suggestive if classical MRI findings fit the clinical history.     

Intradural lumbar disc herniations: report of three cases

Intradural lumbar disc herniation is a rare pathological entity. Three new cases among a series of 5000 lumbar spine operations are added to the about 60 previous case reports in the literature. None of our patients had undergone lumbar spine surgery before. In one patient the location of the free disc fragment was medial within the dural sac; in two patients the free disc fragment had penetrated the dural sac from the axilla of the nerve root. Pathogenesis is discussed (congenital adhesions of the dura mater to the posterior longitudinal ligament at the lower lumbar spine; weakness of the dura mater ventrally and at the axilla of the nerve root). By multiplanar MRI, the free disc hernia within the dural sac may be shown preoperatively.     

Tumour blood flow and partition coefficients: correlation with grade of cerebral gliomas using xenon-enhanced computed tomography

It is possible to underestimate the grade of nonenhancing cerebral tumours on conventional contrast-enhanced MRI or CT. Differentiation of high- and low-grade gliomas by measurement of the brain-blood partition coefficient lambda (T lambda) with Xe-enhanced CT (XeCT) has been reported. We assessed the practical applications of XeCT in suspected low-grade astrocytomas. We examined 15 patients with tumours which showed no contrast enhancement on conventional MRI and CT, using XeCT. Tumour blood flow (TBF) and T lambda were calculated. Fourteen patients underwent surgery, one patient had a biopsy. We recognized three histological groups. While T lambda differed significantly between them, TBF did not. Group 1 contained grade II-III astrocytomas and T lambda was 0.77; group 2 contained grade I-II astrocytomas with T lambda 1.14, and group 3 four oligodendrogliomas in which a T lambda of 1.50 was found.     

Stimulation of IL-2 production and CD2R expression by splenopentin analogs

A multiplicity of peripheral nerve syndromes may develop in patients with diabetes mellitus, the commonest of which is a chronic symmetric sensory polyneuropathy, often associated with autonomic neuropathy. Once established, it is largely irreversible. Acute painful diabetic sensory neuropathy is a separate entity with a favorable prognosis. It now seems likely that chronic inflammatory demyelinating polyneuropathy occurs with greater frequency in diabetic subjects than in the general population and is one explanation for the occurrence of a predominantly motor polyneuropathy. Focal and multifocal peripheral nerve lesions are seen mainly in older diabetic patients and comprise cranial, thoracoabdominal and limb nerve lesions, the last including proximal lower limb diabetic motor neuropathy (diabetic amyotrophy). With this wide array of disorders and the frequency of diabetes, it is important to distinguish those that are directly or indirectly related to diabetes from those that have a coincidental relationship.