Jolt Accentuation of Headache: The Most Sensitive Sign of CSF Pleocytosis

We prospectively examined the clinical signs of 54 febrile patients associated with recent-onset headache. They underwent lumbar puncture (LP) on suspicion of meningitis. The relation of each sign to cerebrospinal fluid (CSF) pleocytosis was estimated. Among 34 patients with pleocytosis, 33 had jolt accentuation (sensitivity: 97.1%), while only 5 of them had neck stiffness or Kernig's sign. Among 20 patients without pleocytosis, 12 had no jolt accentuation (specificity: 60%). We found jolt accentuation to be the most sensitive sign of CSF pleocytosis. If jolt accentuation is noted in a febrile patient associated with recent onset headache, the CSF should be examined even in the absence of neck stiffness or Kernig's sign.     

Recognizable inherited syndrome of progressive central nervous system degeneration and generalized intracranial calcification with overlapping phenotype of the syndrome of Aicardi and Goutières

Five boys and two girls from a large consanguineous British Muslim family of Pakistani origin are described. All presented from infancy to early childhood with progressive moderate to severe developmental delay, postnatal microcephaly, spastic quadriplegia, refractory seizures, and visual handicap. Cerebrospinal fluid (CSF) pleocytosis was present in three children. Neuroimaging with computerized tomography on three boys and a girl showed generalized cortical atrophy, dilatation of the lateral, third, and fourth ventricles, widening of the surface CSF spaces, hypoplasia of the posterior fossa structures, and multiple and solitary calcifications in the cerebral cortex and punctate calcifications involving basal ganglia, cerebellum, and the Sylvian fissure. Histopathological examination of the brain from three boys and one girl confirmed generalized cortical and cerebellar atrophy with widespread calcifications within the cortical grey and white matter, the basal ganglia, the cerebellum, and in some areas along the capillaries. Investigations excluded a possible nongenetic cause. Parental consanguinity favor autosomal recessive inheritance. This appears to be a recognizable syndrome overlapping the syndrome of Aicardi and Goutières (MIM 225750). Am. J. Med. Genet. 75:508–515, 1998. © 1998 Wiley-Liss, Inc.     

CSF pleocytosis and expansion of spinal lesions in Japanese Multiple sclerosis with special reference to the new diagnostic criteria

New diagnostic criteria for multiple sclerosis (MS) were recently proposed from the international panel on the diagnosis of MS, and they include exclusion criteria, such as lesions extending over more than two vertebral segments on spinal MRI and CSF pleocytosis of more than 50/mm³. We reviewed the clinical features of 158 patients who satisfied the diagnostic criteria for MS except for having the above atypical paraclinical findings. All patients exhibited two or more clinical attacks and objective clinical evidence of multiple lesions without any evidence of other disorders. Thirty–three (20.9%) patients had one or both atypical paraclinical findings. Twenty–one out of the 33 patients were classified as having optico–spinal MS (OSMS), and the other 12 as non–OSMS patients with atypical large expanding or destructive cerebral, cerebellar or brainstem lesions on MRI as well as one or both atypical paraclinical findings. Based on this heterogeneity in clinical findings in MS, there is an urgent need to develop a common general concept of the “MS” syndromes, and the ethnic–related heterogeneity should be considered in the revised criteria for the diagnosis of MS.     

Regression of white matter hypodensities with age in Aicardi–Goutierés syndrome: a case report

Background Aicardi–Goutierés syndrome (AGS) is a severe and progressive familial encephalopathy that is characterized by acquired microcephaly, intracranial calcification, white matter lesions, and chronic lymphocytosis with elevated levels of interferon-α in the cerebrospinal fluid. Although the degree of calcification and the severity of brain atrophy are variable, typically, the brain lesions appear to progress on successive examinations.Case report We report a 7-year-old male patient who showed relative regression of white matter lesions with nonprogression of basal ganglia calcification and atrophy on follow-up magnetic resonance imaging and computed tomography scans.Results Magnetic resonance spectroscopy findings were normal. This, to our knowledge, is the first case report, which describes relative regression of the white matter changes in AGS.     

Cerebral blood flow changes in pseudomigraine with pleocytosis analyzed by single photon emission computed tomography. A spreading depression mechanism?

Pseudomigraine with pleocytosis is a benign and autolimited syndrome. The etiology has been related to viral infection, but its pathophysiology is not yet well identified. To investigate this point, and to see if there were changes in cerebral blood flow (as in migraine), we performed single photon emission computed tomography (SPECT) studies in four patients who fulfilled the diagnostic criteria for this syndrome. This was done during the acute phase and we repeated SPECT after resolution of the syndrome in two of them. We found a reduction in brain blood flow on the side of origin of the neurological deficits during the acute phase. This normalized after recovery of the syndrome. The finding suggests that the neurological deficits in this syndrome could be produced by a spreading depression-like mechanism similar to that proposed for migraine with aura.     

Lymphocytic Meningitis in Patients with Sympathetic Ophthalmia

Purpose: This study aimed at reporting lymphocytic meningitis in patients diagnosed with sympathetic ophthalmia (SO).

Methods: In this single-center retrospective observational case series, we reviewed cases diagnosed with SO. We analyzed the patients’ inciting injuries, the characteristics of uveitis and the cerebrospinal fluid (CSF) analyses.

Results: Nine patients were diagnosed with SO and CSF analyses were available in all cases. Four cases had lymphocytic pleocytosis, 3 of which showed marked CSF inflammation with more than 300 lymphocytes/mm³. The inciting event in these 3 patients was a globe perforation injury, whereas 4 patients without meningitis had SO following a surgical intervention.

Conclusions: In this case series of patients with SO, lymphocytic meningitis was a common finding. The prevalence of meningitis in patients with SO and its value for the diagnosis of the disease needs to be further studied.     

C6-peptide serology as diagnostic tool in neuroborreliosis

The aim of this study was to evaluate the usefulness of borrelia serology (Quick ELISA C6 Borrelia assay kit) as a diagnostic tool in cases of suspected neuroborreliosis. A retrospective patient material consisting of 124 paired serum and cerebrospinal fluid samples with a positive anti-borrelia antibody index (AI) using the IDEIA Lyme Neuroborreliosis test was compared with 124 AI-negative matched control subjects. The patients were divided into four groups based on presence of pleocytosis and age above or below 12 years. The presence of positive C6 serology in AI-positive patients with pleocytosis was 89% (83/93), significantly different (p<0.01) from in patients without pleocytosis (58%, 18/31). In AI-positive patients aged > or =12 years with pleocytosis, 94% (51/54) had a positive C6 serology. Of AI-positive patients with a symptom duration of more than 30 days, 93% (27/29) were positive by the C6 test. We conclude that the C6 serum test, together with clinical evaluation, is a powerful diagnostic tool in adult (> or =12 years) European patients with suspected neuroborreliosis with a symptom duration of more than 30 days. Patients with suspected neuroborreliosis and positive C6 results should be further investigated by lumbar puncture for definite diagnosis.     

Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination

To evaluate the effect of interferon beta-1b (IFNB-1b) on multiple sclerosis (MS) with severe optic nerve and spinal cord demyelination, we examined the relationship between IFNB-1b treatment outcome and the clinical and genetic characteristics of three types of demyelinating diseases of the central nervous system, i.e., neuromyelitis optica (NMO), MS and MS with severe optic-spinal demyelination. Japanese MS frequently carried HLA DPB1*0501, which is associated with NMO. MS with DPB1*0501 showed severe optic-spinal demyelination represented by longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis. IFNB-1b treatment did not succeed in these patients because of the increase of optic nerve and spinal cord relapse and other severe side effects. IFNB-1b should not be administered to demyelinating patients with genetic and clinical characteristics mimicking NMO such as HLA DPB1*0501 allele, longitudinally extensive spinal cord lesion, blindness and CSF pleocytosis even if they have symptomatic cerebral lesions as typically seen in MS. The present study strongly suggests that these patients should be diagnosed as having NMO.     

Common Symptoms – Different Diseases: Coexistence of Neurosyphilis and Non-Hodgkin's Lymphoma

We describe the case of a 32-year-old man with generalized lymphadenopathy who was diagnosed with a low-grade follicular small-cleaved cell lymphoma. The patient developed hearing loss, tinnitus and cerebrospinal fluid (CSF) pleocytosis attributed to central nervous system (CNS) infiltration by his malignancy, while receiving chemotherapy with vincristine, cyclophosphamide and prednisone. Despite intrathecal chemotherapy with methotrexate, the CSF pleocytosis persisted. Neurosyphilis was suspected because of prior history of gonorrhea and was confirmed with serologic studies of blood and CSF and from the decline of the anti-treponemal antibody titers with appropriate antibiotic therapy. Syphilis should be considered in the differential diagnosis of patients with generalized lymphadenopathy and neurologic signs or symptoms. Received: March 16, 2001 · Revision accepted: October 4, 2001