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.

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.

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.

Treatment and outcome of subdural hematoma in patients with spontaneous intracranial hypotension: a report of 35 cases.

Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headache, low CSF pressure and diffuse pachymeningeal enhancement on brain MRI. SIH results from spontaneous CSF leakage leading to brain sag. Sometimes, tearing of bridging veins may produce subdural hematomas (SDHs). Patients with SDH were identified retrospectively from 212 consecutive SIH patients. Data were collected on demographics, clinical courses, neuroimaging findings, treatment and outcome of SDH. Thirty-five patients (16%), (6 women, 29 men; aged 33-68; mean, 50 years) with SDH were recruited. They were divided into two groups: initially withSIH diagnosed (n = 29) and undiagnosed (n = 6). After conservative treatment, the first group underwent a lumbar epidural blood patch (EBP) (n = 27) and emergent evacuation of symptomatic SDH (n = 2). After EBP, ten patients had enlarged SDH. Nine of them underwent evacuation of symptomatic SDH with mass effect (ME). In the second group, three patients with clinical worsening from SIH underwent, erroneously, evacuation of mild SDH. They worsened after the evacuation; after SIH diagnosis was made, they underwent one EBP (n = 2) and three EBPs (the patient with coma). The other three cases with symptomatic SDH with ME underwent evacuation with recurrence of SDH (n = 2). All 35 patients enjoyed a good outcome. If conservative treatment is insufficient, EBP should be performed prior to hematoma irrigation. When an emergent evacuation is necessary before EBP, it is preferable to perform, after surgery, one early EBP before the patient gets up to prevent frequent recurrences of SDH by underlying CSF leakage.

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.

Early pain relief from orthostatic headache and hearing changes in spontaneous intracranial hypotension after epidural blood patch.

Spontaneous intracranial hypotension (SIH) is a neurological condition characterized by orthostatic headache (OH), low cerebrospinal fluid (CSF) pressure and diffuse pachymeningeal enhancement on brain magnetic resonance imaging (MRI). Hearing changes (HC) are also a common clinical finding. At present, epidural blood patch (EBP) is the most recommended treatment. Our study aimed at describing clinical variability of SIH patients. We also aimed at measuring the EBP efficacy on OH and HC in patients affected by SIH, by asking them to rate their levels of discomfort on a VAS. 28 consecutive patients were recruited. All of them complained about OH, 16 of them also reported HC. They were all treated with EBP. Two clinical psychologists interviewed them before and after the procedure, asking to rate the intensity of their OH and HC on a VAS at different time points: the day before the procedure, between 24 and 48 h after it and 2 months after treatment. Before EBP, patients rated their OH as 5 (IQR 2-7) and their HC as 4 (IQR 2-5.75). 24/48 h after EBP, a significant improvement in OH (median 0, IQR 0-0; p < 0.001) and HC (median 1, IQR 0-2; p < 0.05) was found. At follow up assessments, all patients reported a complete relief from their OH and four out of 16 patients only still reported mild HC. Our data show for the first time the early and durable efficacy of EBP on OH and HC in patients affected by SIH.