Intracranial hypertension presenting with severe visual failure, without concurrent headache, in a child with nephrotic syndrome

Authors: Barnett M, Sinha MD, Morrison D, Lim M.

BACKGROUND: Idiopathic intracranial hypertension is a condition typically characterised by headache, normal level of consciousness, papilloedema and raised cerebrospinal fluid pressure. Children often present with visual loss and atypical features of raised pressure, posing a diagnostic and management challenge. A range of renal disorders can predispose to developing this raised intracranial pressure syndrome. We present a case of severe visual failure in a child with nephrotic syndrome, with no headache when elevated pressure was proven. In nephrotic syndrome, visual failure related to elevated intracranial pressures without concurrent headache symptoms has not been reported previously.

Idiopathic intracranial hypertension from the perspective of headache center

Authors: Ljubisavljevic S, Trajkovic JZ, Sternic NC, Spasic M, Kostic V.

Idiopathic intracranial hypertension (IIH) is a pathological state defined as an increase of intracranial pressure in the absence of a causative pathological process. The aim of this study was to evaluate the clinical features of the patients with IIH diagnosed in our Headache Center according to the current knowledge of this disorder. In the retrospective and cross-sectional analysis of 3395 patients we present 12 newly diagnosed IIH patients, ten women and two men, aged from 19 to 51, with obtained values of cerebrospinal fluid pressure between 250 and 680 mm of water. The symptoms of IIH clinical presentation have been headache, reported by 92 % of patients; papilledema, noted in 67 %; and cranial nerve impairment (25 %). The results obtained from presented patients confirmed the presence of headache features that are included in criteria for headache attributed with IIH in majority of them: progressive, daily, diffuse, non-pulsatile headache with aggravation by coughing or straining. Decrease of pain intensity after lumbar puncture was noted in all patients. We notice the relatively small proportion of patients with headache attributed to IIH among the patients treated in our Headache Center. The prevalence of IIH is not low and headache is the most frequent presenting symptom; therefore, we could only conclude that some chronic headache patients refractory for treatment are patients with IIH.

Spontaneous Low Pressure, Low CSF Volume Headaches: Spontaneous CSF Leaks

Authors: Mokri B.

Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.

Primary stabbing headache: a new dural sinus stenosis-associated primary headache?

Authors: Montella S, Ranieri A, Marchese M, De Simone R.

Primary stabbing headache (PSH) is a primary syndrome of unknown aetiology, characterised by brief, jabbing stabs predominantly felt in the orbital, temporal and parietal areas, whose frequency may vary from one to many per day, usually responding to indomethacin. PSH frequency in the general population is not well defined, but recent evidence suggests it could be more frequent than previously thought. In clinical series, PSH incidence was 33/100,000 per year, while in a population study 35.2 % prevalence was found. PSH was previously described as isolated or associated to other headache syndromes, most frequently with migraine. There is evidence that an idiopathic intracranial hypertension without papilledema, a condition usually associated to significant stenosis of dural sinuses (93 % sensitivity and specificity), is much more prevalent than believed and may run asymptomatically in up to 11 % of otherwise healthy individuals. In migrainous prone people, a sinus stenosis-associated intracranial hypertension without papilledema (ss-IHWOP) comorbidity may represent a powerful risk factor for progression of pain. Besides migraine, significant sinus stenosis has been found overrepresented also in chronic tension type headache as well as in exertional, cough, sexual activity-associated headaches (all indomethacin responsive primary headaches) and in altitude headache (an acetazolamide responsive condition). To explore the possible association between venous outflow disturbances and PSH, we retrospectively investigated the co-occurrence of sinus venous stenosis in patients referring to our headache centre since 2004 diagnosed with PSH who completed the diagnostic protocol. Out of 50 consecutive patients reporting PSH as the main or as accessory complaint, 8 (6 females, 2 males) performed MR venography (MRV). All MRV revealed significant unilateral or bilateral sinus stenosis. Mean age at PSH onset was 35.3 ± 18.9 years (range 11-67 years). Duration of attacks ranged 1-3 s. Median daily frequency of attacks was 4 (range 2-20); median number of days per month with PSH presentation was 14 (range 4-30). Six patients described attacks in temporal or parietal areas, one at the top of the head, and one in the occipital area. Only one patient had isolated PSH; all the others were diagnosed also with migraine without aura. Seven out of eight patients responded to indomethacin 75 mg/die, and one to topiramate 100 mg/die. Interestingly, both drugs share with acetazolamide a CSF pressure lowering effect. Our findings indicate that PSH is associated with central sinus stenosis and suggest that an undiagnosed ss-IHWOP might be involved in PSH pathogenesis.

Headache prevalence and clinical features in patients with idiopathic intracranial hypertension (IIH)

Authors: D Amico D, Curone M, Ciasca P, Cammarata G, Melzi L, Bussone G, Bianchi Marzoli S.

Headache is a key symptom of idiopathic intracranial hypertension (IIH). Operational diagnostic criteria for "Headache attributed to IIH" are included in the international classification of headache disorders, the ICHD-2. The association of IIH with obesity was established by several reports. We investigate the prevalence of headache and its main clinical features in a clinical sample of IIH patients. The possible correlations between the presence of headache and body mass index (BMI) and intracranial pressure (ICP) levels were studied in a consecutive clinical series of patients, in whom diagnosis of IIH was confirmed by exclusion of secondary forms and by the evidence of increased ICP. Differences for age, BMI, and ICP between patients with and without headache and between males and females were assessed with Mann-Whitney U test. Spearman's correlation analysis was used to assess relationships between age, BMI, and ICP. P value < 0.05 was used to set statistical significance. 40 patients entered the study (9 males, 31 females; mean age 39, 8 years, SD 13.2). Headache was reported by 75 % patients. Those characteristics which are included in the present international diagnostic criteria for "Headache attributed to IIH" were reported by a remarkable proportion of the studied patients, but not by all. On the other hand, some headache features usually attributed to migraine forms, and which are not among the required criteria were present in some patients: pulsating quality and unilateral distribution of pain in around 20 %, and migrainous associated symptoms in more than 40 % of the sample. According to statistical analyses, no differences were found for age, BMI, and ICP between patients with and without headache. Our results confirmed the strong association between headache and IIH. Although no significant correlations between some of the key features of IIH were found in this study, we suggest that further studies on larger series-possibly with a longitudinal evaluation-are needed, to help clinicians in categorizing different subgroups among IIH patients as well as in identifying the main factors influencing the prognosis of this disorder.

Cerebral sinus thrombosis - an uncommon but important differential diagnosis to headache, stroke and seizures. Cases and overview

Authors: Sveinsson OA, Kjartansson O, Valdimarsson EM.

Thrombosis of the cerebral veins and sinuses is an unusual but important cause of increased intracranial pressure and stroke, especially in the young and middle aged. Pregnant women, especially during the puerperium, and individuals with thrombophilia are a special risk group. What makes the diagnosis difficult is the vast range of symptoms including: headache, nausea, vomiting, blurry vision, reduction of consciousness, aphasia and motor and sensory disturbances. We present four cases which reflect the diverse clinical presentation of the disease. Key words: cerebral sinus thrombosis, raised intracranial pressure, stroke, anti-coagulation. 


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