Pressure-related torticollis: an unusual manifestation of pseudotumor cerebri

A 9-year-old boy with pseudotumor cerebri who presented with neck pain and an accompanying torticollis is described. The patient exhibited bony abnormalities of the upper cervical spine and facial asymmetry that suggested a congenital torticollis. Reduction of the increased cerebrospinal fluid pressure by lumbar puncture resulted in a prompt and dramatic resolution of the cervical symptoms and signs. Increased cerebrospinal fluid pressure should be added to the list of those disorders that may give rise to reversible torticollis.     

Reactivation of varicella presenting as pseudotumor cerebri: three cases and a review of the literature

The possible association of pseudotumor cerebri and varicella infection was previously mentioned in a few case reports. In those cases, the history and clinical features of active varicella were obvious, and signs were directly related to the varicella infection. We describe three immunocompetent children with pseudotumor cerebri as the only manifestation of Varicella zoster virus reactivation, with a review of the literature. We suggest considering Varicella zoster virus in children with pseudotumor cerebri, even in the absence of a history of recent varicella infection.     

Pseudotumor cerebri secondary to subacute sclerosing panencephalitis

Pseudotumor cerebri is characterized by signs of raised intracranial pressure occurring in the absence of obvious brain pathology. We present an 8-year-old boy with pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The reported patient suggests a possible association between subacute sclerosing panencephalitis and pseudotumor cerebri and that subacute sclerosing panencephalitis should be added to the list of disorders known to be associated with pseudotumor cerebri. Patients with subacute sclerosing panencephalitis might benefit from treatment aimed at increased intracranial pressure.     

Pseudotumor Cerebri in Lyme Disease: A Case Report and Literature Review

Pseudotumor cerebri is an unusual presentation of Lyme disease. The case of an 8-year-old girl with pseudotumor cerebri secondary to acute neuroborreliosis is reported. She presented with acute onset of headache, papilledema, sixth nerve palsy, increased intracranial pressure, and cerebrospinal fluid pleocytosis. Serum and cerebrospinal fluid Lyme antibodies were positive. Twelve reported cases that mostly presented with systemic findings and signs of Lyme disease before development of pseudotumor cerebri were reviewed. We conclude that acute neuroborreliosis can present with pseudotumor cerebri as an initial manifestation. It is important to include Lyme disease in the differential diagnosis of pseudotumor cerebri in an area endemic for Lyme disease.     

Idiopathic intracranial hypertension in the pediatric population

The aim of this study was to determine the features of pseudotumor cerebri or idiopathic intracranial hypertension in prepubertal and pubertal children. We retrospectively reviewed patient charts of those 16 years and younger, diagnosed with pseudotumor cerebri/idiopathic intracranial hypertension. Our study group consisted of 27 patients; the mean age was 10.9 years, and there was a male-to-female ratio of 13 to 14. In the prepubertal group (n = 13), the male-to-female ratio was 8 to 5; in the pubertal group, (n = 14), the ratio was 5 to 9. Overweight or obesity was found in 16 (59%) patients. Outcome was favorable except for one who remained symptomatic. Pseudotumor cerebri/idiopathic intracranial hypertension in children is rare. Its characteristics differ from adults. We found the prepubertal group to be a distinct group since pseudotumor cerebri/idiopathic intracranial hypertension did not occur predominantly in females and was not associated with obesity.     

MAGNETIC RESONANCE IMAGING IN PSEUDOTUMOR CEREBRI

The pathophysiology of pseudotumor cerebri is unclear, but may relate to an abnormality in water transport in the brain. The authors performed MR imaging in seven children with pseudotumor cerebri; the signal intensity in the white matter was normal in all patients. These data suggest that periventricular brain water content is not increased markedly in children with pseudotumor cerebri. The authors speculate that this may relate to the establishment of an equilibrium between increased resistance to cerebrospinal fluid outflow and increased brain stiffness, occurring as a consequence of increased cerebral blood-volume and/or interstitial pressure.     

A case of pseudotumor cerebri associated with colon cancer

A case of pseudotumor cerebri associated with iron deficiency anemia due to colon cancer is reported in a 37-year-old woman. Her initial symptoms were vomiting and severe headache. On physical examination, no lymph nodes and abdominal mass were palpable but marked anemia was noted in her skin and conjunctiva . Neurological examination revealed papilledema in her both eyes and stiff neck. There was no abnormal findings on CT scan on admission. Spinal puncture revealed CSF pressure as high as 620 mmH2O with normal cells, protein, sugar and chloride levels. Hematological examination revealed iron deficiency anemia and thrombocytosis. Angiography at third day revealed no sinus occlusion, but retention of contrast media was seen on the cortical vein of parietal lobe and right transverse sinus. Brain scintigram at sixth day revealed mild accumulation in left parietal lobe, so small venous infarction was suggested. There were two circumscribed stenotic lesions of right ascending colon in the barium enema, and right hemicolectomy was achieved. The pathological diagnosis was adenocarcinoma. The symptoms of pseudotumor cerebri was completely disappeared soon after the surgery together with resolution of anemia. She lives with no deficits now 1 year 3 months after surgery. In conclusion much attention is necessary to a patient of pseudotumor cerebri with iron deficiency anemia for the presence of cancer, because not only this central nervous system lesion is reversible and curable but also the cancer itself may be curable by surgery.     

Panuveitis in association with pseudotumor cerebri

We report a case of an 11-year-old girl with bilateral panuveitis in association with pseudotumor cerebri. The patient underwent complete ophthalmologic, neurologic, and laboratory evaluations and was treated with therapy for pseudotumor cerebri. The patient met the diagnostic criteria for pseudotumor cerebri and also had panuveitis. Symptoms and findings of pseudotumor cerebri and panuveitis improved significantly after combination therapy of oral acetazolamide and weight reduction. The index case illustrates that pseudotumor cerebri can be associated with panuveitis. Therapy for pseudotumor cerebri might also help with the resolution of uveitis.     

Pseudotumor cerebri associated with obstructive nephropathy

We report two infants with pseudotumor cerebri associated with renal disease. The pathogenesis of increased intracranial pressure in this clinical setting is unclear, but may be mediated by one or more of the conditions commonly associated with pseudotumor cerebri, including sinus thrombosis, increased intravascular fluid volume, anemia, and endocrine disturbances resulting in abnormal calcium and phosphorus metabolism. The onset of pseudotumor cerebri also may be related to changes in vasopressin levels that affect brain water permeability.     

Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension

In patients presenting with intracranial hypertension without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition (pseudotumor cerebri syndrome), the diagnosis of intracranial sinus venous thrombosis (ISVT) has crucial etiological, therapeutic and prognostic implications. Utilizing two well-defined groups of pseudotumor cerebri patients, one with magnetic resonance imaging (MRI) or angiography confirmed ISVT (17 patients) and the other in whom ISVT has been excluded (idiopathic intracranial hypertension [IIH], 27 patients), we investigated the characteristics that might be helpful in distinguishing them. No clinical or auxiliary findings differed between the ISVT and IIH groups except for female gender and lower CSF protein level, which were significantly associated with the latter. While the syndrome pseudotumor cerebri could be due to multiple causes including ISVT, the term IIH should be restricted for patients with isolated intracranial hypertension attributable to no other neurological or systemic disease. Since CT frequently misses ISVT, patients with pseudotumor cerebri syndrome should undergo MRI and MR venography before being labeled as IIH. We conclude that Modified Dandy's Diagnostic Criteria of pseudotumor cerebri, formulated prior to MRI era, can no longer be applied for the diagnosis of IIH.     

Pseudotumor Cerebri associated with Hypovitaminos is A and Hyperthyroidism

Pseudotumor cerebri, or benign intracranial hypertension, is relatively rare in children. A 12-year-old girl with Hashimoto hyperthyroidism and hypovitaminosis A is described, who fulfilled the diagnostic criteria for pseudotumor cerebri.     

Pseudotumor cerebri in a boy with 11-β-hydroxylase deficiency — a possible relation to rapid steroid withdrawal

A boy who was known to suffer from adrenogenital syndrome due to 11--hydroxylase deficiency was treated with appropriate steroid replacement, which fully compensated for the deficiency. An intercurrent febrile illness with anorexia and vomiting necessitated an abrupt discontinuation of steroids. He presented with classic signs of pseudotumor cerebri 3 days following steroid withdrawal. Our studies imply that a combination of steroid withdrawal and dehydration with increased sodium urinary excretion, caused rapid fluid shifts within the brain, resulting in intracranial hypertension. At present, when a considerable number of children are on long-term steroids for various reasons, pseudotumor cerebri should be considered in the list of complications during abrupt steroid withdrawal.     

Permanent visual impairment in childhood pseudotumor cerebri

Contrary to statements in the literature, youth does not seem to confer protection from visual loss in pseudotumor cerebri. We examined five children and adolescents who suffered permanent loss of visual acuity or field in association with this disorder. All of them had impairment at the time of diagnosis, and two of the five developed further visual failure while under observation. Children and adolescents with pseudotumor should be kept under the same close ophthalmic surveillance as has been advocated for adults with this disease.     

Sutural diastasis caused by pseudotumor cerebri

The diagnosis of pseudotumor cerebri is based on the triad of: (1) papilloedema, (2) elevated intracranial pressure with a normal cerebrospinal constituency and (3) normal central nervous system imaging studies. However, the diagnosis of pseudotumor cerebri is not always straightforward. We report a 19-year-old morbidly obese girl who developed pseudotumor cerebri associated with sutural diastasis of the cranium based on the direct radiographies. Following a ventriculo-peritoneal shunt operation, we demonstrated the closing and perisuturally sclerosis on all major cranial sutures. A careful examination of direct radiographies might be critical for diagnosis of pseudotumor cerebri. Prompt recognition, evaluation, and treatment are needed to prevent permanent visual loss.     

Pseudotumor cerebri in men

We describe 29 men with pseudotumor cerebri (PTC) and compare them with aged-matched women with PTC and male control subjects. Pseudotumor cerebri occurs less frequently in men but in a similar age distribution. Although signs and symptoms of PTC are similar in men and women, men may require surgical treatment for impending visual loss. Black men appear to be at greater risk to loss of vision. Even though men with PTC are less likely to be obese than women, they tend to be more obese than control subjects and should be counseled on weight-reduction diets.     

Acute Q fever in children presenting with encephalitis

We describe the cases of three children with encephalitis associated with Coxiella burnetii infection. Neurologic features ranged from status epilepticus and coma to headache and pseudotumor cerebri syndrome. Patients had good response to antibiotic treatment with doxycycline and recovered fully. Q fever should be included in the differential diagnosis of children with encephalitis, and routine serological testing should be considered, especially in endemic areas.     

Magnetic resonance venography in idiopathic pseudotumor cerebri.

OBJECTIVE: To inform clinicians about the use of magnetic resonance (MR) venography in idiopathic pseudotumor cerebri. MATERIALS AND METHODS: A prospective study to evaluate for the presence or absence of dural sinus thrombosis using MR imaging and MR venography of the brain in 22 consecutive young, female, overweight patients with typical pseudotumor cerebri. RESULTS: None of the 22 MR imaging and MR venography studies showed venous sinus thrombosis. CONCLUSION: Magnetic resonance venography might not add significantly to the evaluation of typical idiopathic pseudotumor cerebri but may be indicated in atypical cases (e.g., male, thin, or elderly patients).     

The changing picture of pseudotumor cerebri in children

Among 38 children with pseudotumor cerebri only 3 cases were due to ear disease or its complications. The commonest cause was a refeeding syndrome either due to nutritional deprivation or as an early finding in the treatment of cystic fibrosis. It is uncommon to find the cause of pseudotumor in older children but in those under 6 years the cause was found in 85%. Because of the adverse effects of steroids we use this treatment in the more resistant cases.     

Different etiologies of acquired torticollis in childhood

Introduction

Torticollis can be congenital or may be acquired in childhood. Acquired torticollis occurs because of another problem and usually presents in previously normal children. The causes of acquired torticollis include ligamentous, muscular, osseous, ocular, psychiatric, and neurologic disorders.

Objective

We performed this study to evaluate the underlying causes of torticollis in childhood.

Material and methods

Ten children presented with complaints of torticollis between April 2007 and April 2012 were enrolled in this study. The additional findings of physical examination included neck pain, twisted neck, walking disorder, imbalance, and vomiting The identified etiologies of the enrolled children was acute disseminated encephalomyelitis in a 2.5-year-old boy, posterior fossa tumor in a 10-month-old boy, spontaneous spinal epidural hematoma in a 5-year-old hemophiliac boy, cervical osteoblastoma in a 3-year-old boy, arachnoid cyst located at posterior fossa in a 16-month-old boy, aneurysm of the anterior communicating artery in a 6-year-old girl, pontine glioma in a 10-year-old girl, and a psychogenic torticollis in a 7-year-old boy were presented.

Conclusion

There is a wide differential diagnosis for a patient with torticollis, not just neurological in etiology which should be considered in any patient with acquired torticollis. Moreover, early diagnosis of etiological disease will reduce mortality and morbidity. Therefore, clinicians managing children with torticollis must be vigilant about underlying neurological complications.