Neurobrucellosis: clinical and neuroimaging correlation.

BACKGROUND AND PURPOSE: Manifestation of nervous system involvement by neurobrucellosis, a treatable infection, is not well documented. We investigated patterns of nervous system involvement and determined if neuroimaging abnormalities correlated with clinical manifestations of neurobrucellosis. METHODS: We reviewed 23 MR imaging studies (17 of brain, six of spine) and seven CT scans of brain in 23 patients (14 male and nine female patients; age range 17-71 years) with positive Brucella titers in their serum and CSF. RESULTS: Twelve patients had central nervous system (CNS) involvement, four had peripheral nervous system (PNS) involvement, two had combined PNS and CNS involvement, and five had isolated hearing loss. Imaging findings were variable: five of seven brain CT studies were normal, and 10 of 23 MR studies were normal (eight brain, one thoracic, one lumbar). One brain CT study showed subthalamic hemorrhage, mild perivascular enhancement, left caudate lacunae, and diffuse white matter changes. One other brain CT study showed enhancement of the tentorium in addition to white matter changes. Abnormal MR findings were basal meningeal enhancement (n = 3), lumbar nerve root enhancement (n = 3), granuloma of the suprasellar region (n = 1), diffuse white matter changes (n = 7), and spinal cord atrophy (n = 1). All patients improved after treatment with three antimicrobial drugs for 3-12 months. Seven patients had follow-up imaging; the enhancement disappeared but the white matter and ischemic changes persisted despite almost complete clinical recovery. CONCLUSION: Clinical-radiologic correlation in neurobrucellosis varies from a normal imaging study despite positive clinical findings, to a variety of imaging abnormalities that reflect either an inflammatory process, an immune-mediated process, or a vascular insult.

Polyradiculopathy. A rare complication of neurobrucellosis.

Neurobrucellosis is chronic brucellosis affecting the nervous system. It may mimic many neurological diseases but it rarely presents as polyradiculopathy. Brucellar radiculopathy was diagnosed in 6 patients who presented with weakness of the lower extremities. Five patients had lumbar puncture, 4 had magnetic resonance imaging of lumbar spine and 4 had nerve conduction studies. Five patients had areflexia and weakness; one had areflexia with proprioceptive ataxia. All patients had positive Brucella serology; cerebrospinal fluid showed lymphocytic pleocytosis, elevated protein, normal-low glucose; brucella serology was positive in all specimens. Nerve conduction studies showed absent F-wave in 2 patients and polyradiculopathy with secondary motor axonopathy in 2 patients; motor conduction velocity was normal in all. Magnetic resonance imaging with gadolinium injection showed enhancement of lumbar nerve root in 3 patients, and no enhancement in one. All patients improved after treatment with antibiotics and lumbar root enhancement disappeared. Symptoms of myelopathy were unmasked after radiculopathy had resolved in one patient. In endemic areas, brucella infection should be considered in the differential diagnosis of radiculopathy. Radiculopathy is probably due to inflammation of the meninges and the intrathecal portion of the roots. The pathogenesis of myelopathy may involve demyelination as spasticity persists or worsens after radiculopathy improves.

Transcranial magnetic stimulation in Behçet's disease: a cross-sectional and longitudinal study with 44 patients comparing clinical, neuroradiological, somatosensory and brain-stem auditory evoked potential findings.

OBJECTIVES: To compare neurological involvement in Behçet's disease as documented by transcranial magnetic stimulation (TMS) with clinical, neuroradiological, somatosensory (SEP) and auditory evoked potential (BAEP) findings. METHODS: Forty-four patients were studied over an 8 year period. Nine patients had follow-up studies done. TMS central motor conduction (CMC) studies to upper and lower limb muscles, brain magnetic resonance imaging (MRI), SEP, and BAEP testing were conducted. RESULTS: Thirty-nine patients had CMC slowing, decreased amplitude or absent motor evoked potentials (MEP); 5 of these patients were neurologically normal. Concordance of TMS results, clinical deficits, and MRI findings occurred in 36 of the 39 patients. SEP and BAEP testing proved non-complementary to MEP. Generally, follow-up studies revealed faster CMC and higher MEP amplitude. However, in two patients the CMC time to one target muscle became prolonged with diminished MEP amplitude over a period of 1.5-3 years. CONCLUSIONS: TMS can be useful in detecting and quantifying motor tract dysfunction in Behçet's disease and provides functional information complementary to imaging studies. TMS is more sensitive than either SEP or BAEP. Our longitudinal studies suggest that TMS studies may be valuable in monitoring disease activity or therapeutic response.

Craniocervical junction tuberculosis: a rare but dangerous disease.

BACKGROUND: Mycobacterium tuberculosis of the cervical spine is a rare but dangerous manifestation of extra-pulmonary tuberculosis. The clinical picture ranges from early, nonspecific, insidious symptoms to severe neurological complications and death, attributed to craniocervical junction instability and cervicomedullary compression. The different lines of management include antituberculous medication with traction and external fixation or adjunctive surgery (debridement and stabilization) in patients with severe or persistent neurological complications and/or vertebral instability. METHODS: We describe two patients with advanced craniocervical junction tuberculosis. The early clinical picture was nonspecific in Case 1 and obscured by psychiatric illness in Case 2. The detailed clinical and radiological findings, and the management, will be described. Involvement of the occipital condyles and foramen magnum, which has not been reported previously, will be demonstrated. RESULTS: Both cases underwent transoral biopsy, aspiration, and debridement of retropharyngeal abscess (granuloma). Histological and tissue culture studies proved the abscesses were tuberculous and anti-tuberculous medications were started. Case 1 showed complete resolution of the clinical and radiological findings. Case 2 developed cardiorespiratory arrest while in a halo jacket. He was resuscitated but remained quadriplegic and semiconscious; he developed nosocomial gram negative pneumonia. He was referred back to his local hospital where he died 1 year later. CONCLUSIONS: Tuberculosis is an infrequent but notable cause of cervicomedullary compression. It should be suspected in patients with infective spondylitis who are immunocompromised or reside in an area highly endemic for tuberculosis. Management strategies include antituberculosis medication, transoral biopsy and drainage of the abscess, traction and external fixation, posterior decompression, and internal fixation, according to the clinical and radiological findings.

Sildenafil (Viagra) A breakthrough in the management of an old problem or a risky drug.

Full text is available as a scanned copy of the original print version.

Sildenafi (Viagra).

Full text is available as a scanned copy of the original print version.

Biotin-responsive basal ganglia disease: a novel entity.

We describe a novel, biotin-responsive basal ganglia disease in 10 patients. At onset, it appears as a subacute encephalopathy, with confusion, dysarthria and dysphagia with occasional supranuclear facial nerve palsy or external ophthalmoplegia, and progresses to severe cogwheel rigidity, dystonia and quadriparesis. These symptoms disappear within a few days if biotin (5-10 mg/kg/day) is administered, and there are no neurological sequelae. They reappear within 1 month if biotin is discontinued. Patients diagnosed late, or who have had repeated episodes, suffer from residual symptoms such as paraparesis, mild mental retardation or dystonia. The numerous biochemical studies of intermediary metabolism, like the autoimmune and toxicological studies, enzyme assays including biotinidase, carboxylase and lysosomal activities, and bacterial and viral studies were all normal. The aetiology may be related to a defect in the transporter of biotin across the blood-brain barrier. The only consistent radiological abnormality was central necrosis of the head of the caudate bilaterally and complete, or partial, involvement of the putamen on brain MRI. This was present during the initial acute encephalopathy and remained unchanged during follow-up of 3-10 years. Although its aetiology is unknown, it is important to recognize this disease, since its symptoms may be reversed and the progression of its clinical course prevented simply by providing biotin.

Sildenafil (Viagra) A breakthrough in the management of an old problem or a risky drug.

Full text is available as a scanned copy of the original print version.

Respiratory arrest: a complication of Arnold-Chiari malformation in adults.

We describe the cases of 2 adult patients who developed respiratory arrest and were found to have Arnold-Chiari malformation on magnetic resonance imaging. Following posterior fossa decompression both patients improved, though one of them subsequently died during sleep. Imaging of the craniocervical junction should be part of the workup of patients with unexplained apneic episodes.

Acute Wernicke's encephalopathy associated with hyperemesis gravidarum: magnetic resonance imaging findings.

A 25-year-old woman with hyperemesis gravidarum developed acute Wernicke's encephalopathy during prolonged intravenous fluid therapy without vitamin supplements. Delay in diagnosis led to a persistent severe neurological deficit, including coma. Gadolinium-diethylenetriaminepentaacetic acid-enhanced magnetic resonance imaging revealed symmetrical lesions around the aqueduct and fourth ventricle, which resolved after treatment with thiamine. She did not regain consciousness. This report demonstrates the diagnostic value of enhanced magnetic resonance imaging in acute Wernicke's encephalopathy.

Hemichorea in systemic lupus erythematosus: significance of MRI findings.

A young man with systemic lupus (SLE) developed hemichorea 13 years after the onset of his illness. For the first time in the course of his illness he had a positive test for anticardiolipin antibodies (aCL). Magnetic resonance imaging (MRI) of his brain showed lesions of presumed vascular cause in the ipsilateral basal ganglia. The findings support the contention that an immune phenomenon, invisible on proton imaging by MRI, is responsible for the striatal neuronal activation. Chorea, the clinical expression of this activation, was probably blocked on the side previously affected by vascular pathology.

Familial tremulous and myoclonic dystonia with white matter changes in brain magnetic resonance imaging.

We report two families with a disorder, probably autosomal recessive, characterized by tremor of juvenile onset, dystonia, and myoclonus with preserved cognitive, cerebellar, and peripheral nervous system functions. During 4 years' follow-up, mild spasticity appeared. Magnetic resonance imaging (MRI) revealed mild diffuse changes in the white matter. Central conduction times for visual, motor, and sensory systems were all prolonged. Extensive metabolic work-up failed to reveal lysosomal, peroxisomal, mitochondrial, or other metabolic abnormalities.

Evoked potential findings in Behçet's disease. Brain-stem auditory, visual, and somatosensory evoked potentials in 44 patients.

We studied 54 patients with Behçet's disease, 41 males and 13 females, mean age 28 years. Forty-four patients had auditory brain-stem evoked potential (BAEP) recordings, 39 had pattern reversal visual evoked potentials (VEP), 27 had median nerve somatosensory evoked potential (SEP) recordings, and 25 tibial nerve SEPs. BAEPs were abnormal in 16 patients (52%) with neurological manifestations and in 4 (31%) without, because of decreased amplitude of wave V, prolonged I-III or III-V interpeak latencies, or uncertain/absent waves III and/or V. Eleven patients (40%) with neurological symptoms and 3 patients (25%) without, had abnormal VEPs. Absent potentials, decreased amplitude, with or without prolonged P100 latency, were found in 75% of the cases, the rest had prolonged P100 latency only. Median SEPs were abnormal in 8 patients (38%) with neurological manifestations. Four patients (21%) had abnormal tibial SEPs. Decreased amplitude with or without mild slowing in central conduction was the predominant SEP abnormality. SEPs were normal in all patients without neurological symptoms. In total, 84% of patients with, and 38% of patients without, neurological symptoms had abnormalities of one or more EP modality. When used cautiously, EP studies in Behçet's disease might be helpful to separate neuro-Behçet from other disorders with similar symptomatology, to disclose subclinical CNS involvement, to evaluate and monitor CNS disease activity, and to provide objective measures of treatment response.

Myelopathy after intrathecal chemotherapy. A case report with unique magnetic resonance imaging changes.

BACKGROUND: Paraplegia caused by intrathecal chemotherapy has no known pathognomonic features and is a diagnosis of exclusion. METHODS: The authors reported the clinical and neuroimaging findings in one patient with this syndrome. RESULTS: The patient had severe paraplegia with urinary retention and impaired pain and touch sensation below T-10 with sparing of proprioception and vibration sense. Magnetic resonance imaging (MRI) scan showed diminished intensity throughout the central cervical spinal cord. Post-gadopentetate dimeglumine enhancement was scattered throughout the cervical spinal cord and in two areas of the dorsal spinal cord. Axial views of the cervical spinal cord showed that this enhancement was limited to the lateral columns. CONCLUSIONS: The MRI in myelopathy due to intrathecal chemotherapy may show a unique pattern of postgadopentetate dimeglumine enhancement limited to the lateral columns of the spinal cord. However, two recently encountered patients with the same syndrome did not show similar changes.

Subacute sclerosing panencephalitis. Imaging and clinical correlation.

Twenty-one patients at different clinical stages of subacute sclerosing panencephalitis were studied by magnetic resonance imaging and computed tomography. In patients in the early clinical stage, the imaging studies appeared normal. Within 6 months, diffuse or focal areas of high signal on T2-weighted magnetic resonance images were evident. Progressive hemispheric, cerebellar, and brainstem atrophy was seen later. Lesions of the deep nuclei were noted frequently in patients in the early and intermediate stages, with the lentiform involved more than the caudate. Magnetic resonance imaging was better than computed tomography in illustrating white matter and basal ganglia abnormalities. Radiographic progression occurred regardless of clinical course.

Fibrinolysis in pseudotumor cerebri.

Pseudotumor cerebri is a syndrome associated with diverse putative etiological factors that include chemicals such as vitamin A, tetracycline and estrogens or venous circulatory disturbances like sagittal or transverse sinus thrombosis. Diseases predisposing to thrombosis, such as polycythemia vera and essential thrombocythemia, were reported to cause sinus thrombosis and pseudotumor cerebri. This is a pilot study to investigate the possible role of hemostatic factors in the pathogenesis of pseudotumor cerebri. We studied nine patients with severe, recurrent, or refractory pseudotumor cerebri causing visual impairment and found abnormal euglobulin clot lysis time (prestress in all of them and post stress in seven). Digital subtraction angiography was suggestive of recanalized sinus thrombosis in only three patients. We conclude that abnormalities in the fibrinolytic system are present in a subset of patients with severe pseudotumor cerebri, which calls for further studies on venous circulatory pathogenesis of pseudotumor cerebri and the possible role of anticoagulants in such cases.