Management of Spontaneous Intracranial Hypotension During Pregnancy: A Case Series.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a rare condition resulting from cerebrospinal fluid (CSF) volume depletion, nearly always from spontaneous CSF leaks. CSF pressure in SIH is usually normal; low CSF pressure is found in a substantial minority of patients. SIH is uncommonly described in pregnancy. CASE SERIES: Five women with SIH during pregnancy have been conservatively treated adopting bed rest and overhydration. After prolonged conservative treatment, only 1 patient showed complete symptoms resolution. A rare SIH complication as cerebral venous thrombosis has been reported in 1 case. All 4 remaining patients had lumbar epidural blood patch (EBP) with symptoms disappearance. CONCLUSIONS: EBP might be proposed to SIH patients also during pregnancy and after a brief period (~10 days) of ineffective conservative treatment, because it could allow faster symptoms improvement and complete recovery. Furthermore, EBP would avoid prolonged bed rest with the risk of SIH severe complications.

Stroke-like attack: first episode of sporadic hemiplegic migraine.

Hemiplegic migraine (HM) is a rare migraine with aura; it can be familiar or sporadic. A 46-years-old man presented left migraine followed by right hemiparesis with bilateral plantar flexion of cutaneous plantar reflex (CPR). Brain CT and CT-angiography were normal. The next day patient got worse. The EEG showed left fronto-temporal cuspidate delta waves and brain MRI showed a minimal hyperintensity at T2-sequences in the left frontal cortex with a minor representation of the cortical veins at susceptibility weighted imaging sequences. After 3 days, he had a progressive neurological improvement. After 2 weeks, EEG and brain MRI were normal. He was discharged with diagnosis of probably first attack of sporadic HM and after 8 months he was asymptomatic. The normal CPR on the hemiplegic side might be a clinical marker of functional hemiplegia. For the international classification of headache disorder (ICHD-3) two attacks are necessary for HM diagnosis. We propose for the first attack of HM to make diagnosis of "probable" HM as expected to the same ICHD-3 for migraine. Further studies are necessary to support our hypotheses.

Modified Valsalva test differentiates primary from secondary cough headache.

I suppose that the patient number 14, reported in the article of RJ Lane et al. on "Modified Valsalva test differentiates primary from secondary cough headache "in a recent issue of your esteemed journal, was probably suffering from spontaneous intracranial hypotension syndrome (SIH) caused by cervical manipulation.

Acute confusional migraine in CADASIL: A case report and literature review.

BACKGROUND: Acute confusional state associated with migraine in adults is an infrequent entity. Around 30-60% of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) patients get affected by migraine attacks-the majority with aura-often as the first symptom of the disease. Acute confusional state during migraine has been rarely described in CADASIL patients and a complete neuropsychological assessment during the acute phase has never been conducted so far. CASE SUMMARY: We here describe the clinical and neuropsychological features of two distinct episodes of ACM in a 54-year-old female with CADASIL. EEG recording during acute confusional migraine and after attack resolution and neuroimaging has been reported. DISCUSSION AND LITERATURE REVIEW: This paper also reports a literature review on the topic of ACM in CADASIL highlighting a lack of adequate knowledge about this entity among clinicians and prompting further larger studies to explore its incidence and characteristics.

Cerebral venous thrombosis in spontaneous intracranial hypotension: A report of 8 cases and review of the literature.

BACKGROUND: The occurrence of cerebral venous thrombosis (CVT) in patients with spontaneous intracranial hypotension (SIH) raises difficult practical questions regarding the management of the two conditions. The first-line therapy for CVT is anticoagulation (AC); however, its potential benefit in SIH/CVT patients, especially if complicated by subdural haematoma, must be carefully evaluated taking account of the intracranial haemorrhage risk. Venous system recanalization and good prognosis in SIH/CVT patients treated with epidural blood patch (EBP), the main treatment option for SIH, have been already described. METHODS: We reviewed our cases of SIH complicated by CVT among a cohort of 445 SIH patients observed and treated during the last years. All published case reports and case series reporting patients with SIH and CVT were also ascertained and reviewed. RESULTS: Eight (2%) out of 445 patients suffering with SIH, were also diagnosed with CVT. All patients observed had orthostatic headache, three of them experienced a change in their headache pattern over the SIH course. Six out of eight patients received both AC and EBP treatments. Two patients were treated using only AC or EBP. A bilateral subdural haematoma enlargement after 1 month of AC was observed in one case. Complete CVT recanalization after treatment was obtained in three patients, including two with multiple CVT at baseline; partial CVT recanalization was achieved in two patients. Three patients experienced no CVT recanalization. After 6-48 months' follow-up all patients were still asymptomatic. CONCLUSIONS: The use of AC therapy should be weighed against the intracranial haemorrage risk and should be monitored carefully if initiated. Effective and prompt EBP, even without AC therapy, might lead to a good prognosis in selected cases.

Focus on therapy of primary stabbing headache.

Primary stabbing headache (PSH) is a short-lasting but troublesome headache disorder, which has been known for several decades. The head pain occurs as a single stab or as a series of stabs generally involving the area supplied by the first division of trigeminal nerve. Stabs last for approximately a few seconds, occurring and recurring from once to multiple times per day in an irregular pattern. For the diagnosis of PSH, it is mandatory that any other underlying disorder is ruled out. Indomethacin represents the principal option in the treatment of PSH, despite therapeutic failure in up to 35% of the cases. Recent reports showed that cyclooxygenase-2 (COX-2) inhibitors, gabapentin, nifedipine, paracetamol and melatonin may also be effective. In this report, we focus on the therapy of PSH summarizing the information collected from a systematic analysis of the international literature over the period 1980-2009.

Focus on therapy of hypnic headache.

Hypnic headache (HH) is a primary headache disorder, which occurs exclusively during sleep and usually begins after 50 years of age. There are no controlled trials for the treatment of HH. We reviewed all the available papers, including 119 cases published in literature up to date, reporting the efficacy of the medications used to treat HH. Acute treatment is not recommended, since no drug proved to be clearly effective and also because the intensity and the duration of the attacks do not require the intake of a medication in most cases. As for prevention, a wide variety of medications were reported to be of benefit in HH. The drugs that were found to be effective in at least five cases are: lithium, indomethacin, caffeine and flunarizine. Lithium was the most extensively studied compound and demonstrated to be an efficacious treatment in 32 cases. Unfortunately, despite its efficacy, significant adverse effects and poor tolerability are not rare, mainly in elderly patients. Many patients reported a good response to indomethacin, but some could not tolerate it. Caffeine and melatonin treatments did not yield robust evidence to recommend their use as single preventive agents. Nevertheless, their association with lithium or indomethacin seems to produce an additional therapeutic efficacy. A course of lithium should be tried first, followed 3-4 months later by tapering. If headache recurs during tapering, a longer duration of therapy may be needed. If lithium treatment does not provide a significant response, indomethacin can be commenced as second-line approach. If these treatments prove to be ineffective or poorly tolerated, other agents, such as caffeine and melatonin, can be administered.

Focus on therapy: hemicrania continua and new daily persistent headache.

Hemicrania continua (HC) and new daily-persistent headache (NDPH) represent the only two forms of chronic daily headache in Chap. IV "Other Primary Headaches" of the second edition of the International Classification of Headache Disorders. HC and NDPH are rare and poorly defined from a pathophysiological point of view; as a consequence, their management is largely empirical. Indeed, there is a lack of prospective, controlled trials in this field, and treatment effectiveness is basically inferred from the results of sparse open-label trials, retrospective case series, clinical experience and expert opinions. In this narrative review we have summarised the information collected from an extensive analysis of the literature on the treatment of HC and NDPH in order to provide the best available and up-to-date evidence for the management of these two rare forms of primary headache. Indomethacin is the mainstay of HC management. The reported effective dose of indomethacin ranges from 50 to 300 mg/day. Gabapentin 600-3,600 mg tid, topiramate 100 mg bid, and celecoxib 200-400 mg represent the most interesting alternative choices in the patients who do not tolerate indomethacin or who have contraindications to its use. NDPH is very difficult to treat and it responds poorly only to first-line options used for migraine or tension-type headache.

Focus on therapy of the Chapter IV headaches provoked by exertional factors: primary cough headache, primary exertional headache and primary headache associated with sexual activity.

Primary cough headache, primary exertional headache and primary headache associated with sexual activity are distinct entities, even though they share several features: acute onset, the absence of structural brain disease and exertional factors as precipitating events. In this short review, we illustrate the possible treatment strategies on the basis of information collected from a systematic analysis of the international literature.

Spontaneous intracranial hypotension: review and expert opinion.

Spontaneous intracranial hypotension (SIH) results from spinal cerebrospinal fluid (CSF) leaking. An underlying connective tissue disorder that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. During the last decades, a much larger number of spontaneous cases are identified and a far broader clinical SIH spectrum is recognized. Orthostatic headache is the main presentation symptom of SIH; some patients also have other manifestations, mainly cochlear-vestibular signs and symptoms. Differential diagnosis with other syndromes presenting with orthostatic headache is crucial. Brain CT, brain MR, spine MRI, and MRI myelography are the imaging modalities of first choice for SIH diagnosis. Invasive imaging techniques, such as myelography, CT myelography, and radioisotopic cisternography, are progressively being abandoned. No randomized clinical trials have assessed the treatment of SIH. In a minority of cases, SIH resolved spontaneously or with only conservative treatment. If orthostatic headache persists after conservative treatment, a lumbar epidural blood patch (EBP) without previous leak identification (so-called "blind" EBP) is a widely used initial intervention and may be repeated several times. If EBPs fail, after the CSF leak sites identification using invasive imaging techniques, other therapeutic approaches include: a targeted epidural patch, surgical reduction of dural sac volume, or direct surgical closure. The prognosis is generally good after intervention, but serious complications may occur. More research is needed to better understand SIH pathophysiology to refine imaging modalities and treatment approaches and to evaluate clinical outcomes.