Atypical Spontaneous Intracranial Hypotension With a Head‐Shaking Headache

Spontaneous intracranial hypotension (SIH) is typically characterized by orthostatic headache; however, various atypical manifestations of SIH have been reported recently. We report here the case of a 46‐year‐old man with headache secondary to SIH, which was nonorthostatic, triggered only when the patient shook his head. We suggest that SIH should be suspected in patients with headache induced by head‐shaking, even without orthostatic features, especially when the headache is accompanied by other symptoms commonly associated with SIH.     

Spontaneous intracranial hypotension--a case report

Positional or orthostatic headache symptoms secondary to intracranial hypotension are usually seen as complications in patients after medical procedures, such as lumbar puncture. We describe a case of spontaneous intracranial hypotension (SIH) in a 43-year-old man without a previous history of headaches. He presented to the Emergency Department (ED) with a new-onset orthostatic headache without any history of recent medical procedure, such as lumbar puncture (LP). The ED evaluation included a normal neurologic examination and normal computed tomography (CT) scan of the brain. An LP showed low opening pressure, elevated protein, 46 RBCs and one lymphocyte. Subsequent evaluation with radionuclide cisternography confirmed a cerebrospinal fluid (CSF) leak in the area of the upper thoracic spine, and treatment with an epidural blood patch dramatically improved his symptoms. SIH is due to CSF leakage, usually in the area of the cervical or upper thoracic spine, often without a clear etiology. Conservative medical management including bed rest, oral hydration and caffeine intake is the usual first line treatment. An epidural blood patch is often a dramatically effective treatment that can be done once the presence and location of the leak is identified. Although mostly benign, this condition occasionally can be associated with the formation of clinically significant subdural fluid collections or hematomas. SIH is an increasingly recognized cause of headache.     

Two cases of benign abducens nerve palsy in children and their long-term follow-up

Our aim was to explore the clinical characteristics and diagnostic methods of benign abducens nerve palsy in children. The clinical and laboratory characteristics, treatment approach, and prognosis of two children with benign abducens nerve palsy were retrospectively evaluated. The main clinical symptoms of the two children were limited binocular movement accompanied by double vision, and the positive physical signs were limited binocular abduction accompanied by diplopia. No abnormalities were found in laboratory examinations or in imaging of the head, chest, and abdomen. Both children were treated with B vitamins, methylprednisolone, and gamma globulin, and the clinical symptoms disappeared within 3 months of starting treatment. The cause of benign abducens nerve palsy in children is unknown, but may be related to immune factors. In the two cases presented here, the patients recovered after treatment with immunomodulators.     

Dramatic headache relief after sumatriptan in a patient with a pituitary macroadenoma

We are reporting an interesting case of pituitary macroadenoma. The patient presented with sudden, bifrontal, pulsating headache; photophobia; and an abducens nerve palsy, due to extension of the tumor into the cavernous sinus region. The headache resolved completely after a subcutaneous injection of sumatriptan.     

Subdural haematoma in patients with spontaneous intracranial hypotension

The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.     

Ophthalmoplegic migraine with reversible enhancement of intraparenchymal abducens nerve on MRI

We describe a patient with ophthalmoplegic migraine and right abducens nerve palsy, in whom serial magnetic resonance imaging showed a transient, gadolinium-enhancing lesion in the right lower pons, during both headache and the headache-free period. The enhancing linear lesion was felt to represent intraparenchymal fibers of the affected abducens nerve. The possible pathophysiology of this unique finding is discussed.     

Application of IHS criteria to headache attributed to spontaneous intracranial hypotension in a large population

We applied the recent International Headache Society (IHS) criteria for headache related to spontaneous intracranial hypotension (SIH) to 90 consecutive patients with a final diagnosis of SIH confirmed by cerebral magnetic resonance imaging with contrast. Orthostatic headache (developing within 2 h of standing or sitting up) was present in 67 patients (75%) but appeared within 15 min after standing or sitting—as required by point A of the criteria—in only 53 (59%). Forty-four (49%) patients did not satisfy point A, including 22 (24%) with non-orthostatic headache and 14 (16%) with headache developing ≥ 15 min after standing or sitting up; 80 (89%) did not satisfy point D. Only three (3%) patients had headache fully satisfying the IHS criteria. These findings indicate that the current IHS criteria do not capture most patients with SIH-associated headache. Excluding the requirement for response to epidural blood patch (criterion D) and considering headaches appearing within 2 h of sitting or standing up would capture more patients.     

Recurrence of spontaneous intracranial hypotension with subdural hematomas

A 59-year-old man developed postural headache associated with a low CSF pressure. A CT scan revealed no abnormal findings and the orthostatic symptoms resolved without treatment 6 weeks after onset. He was diagnosed as having spontaneous intracranial hypotension (SIH) and remained symptom-free until he experienced recurrence of postural headache 9 months later. A lumbar puncture demonstrated low CSF pressure, and a CT scan revealed slit-like ventricles with narrowing of the sulci, Sylvian fissures, and infratentorial cisterns, in addition to bilateral subdural masses. After draining the hematomas, his symptoms resolved completely, and a follow-up CT scan was normal. We hypothesize that recurrent SIH in this case was due to small recurrent tears of a root sleeve. This case emphasizes the importance of follow-up of SIH for at least 9 months after resolution of symptoms.     

A case of spontaneous intracranial hypotension with typical magnetic resonance images

Spontaneous intracranial hypotension (SIH) is a rare syndrome defined by postural headache, associated with a low cerebrospinal fluid pressure, without history of previous dural trauma or invasive treatment on rachis. We reported a case of a patient with postural headache caused by SIH identified by magnetic resonance images and treated with saline solution infusion with complete remission of symptoms.     

Cranial Magnetic Resonance Imaging in Spontaneous Intracranial Hypotension after Epidural Blood Patch

▪ Abstract:   Spontaneous intracranial hypotension (SIH) is a syndrome characterized by orthostatic headache, nausea, vomiting, photophobia, and diplopia. Subdural effusion, diffuse dural enhancement, dilatation of epidural veins, and increased height of hypophysis are cranial magnetic resonance (MR) imaging findings in SIH. Epidural blood patch is reportedly one of the effective treatment options. We present the follow-up MR imaging findings in a case of SIH after a successful epidural blood patch treatment. We propose that cranial MR imaging as an objective test to evaluate the success of epidural blood patch treatment. ▪     

Spontaneous intracranial hypotension presenting as mental deterioration

A 55-year-old woman had new onset of postural headache followed by change of mental status 3 weeks later. Magnetic resonance imaging (MRI) of the brain and whole spine showed typical spontaneous intracranial hypotension (SIH) findings, bilateral subdural hematoma, and cerebrospinal fluid leakage over the T7-T9. Her headache and mentality improved after epidural blood patches. Early recognition and correct diagnosis are crucial for successful treatment in patients with SIH presenting with mental confusion.     

Treatment Outcomes in Spontaneous Intracranial Hypotension: Do Epidural Blood Patches Stop the Leaks?

Abstract:   Spontaneous intracranial hypotension (SIH) is a postural headache syndrome unrelated to dural puncture. Because of the increasing failure of epidural blood patch (EBP) to relieve headache in SIH, we retrospectively investigated the epidemiological features and treatment outcomes in 55 cases of SIH. The study population was stratified by age and sex; continuous variables were compared for differences by t -tests; categorical variables were compared by Chi-squared analysis or Fisher exact tests. Significant differences were identified by P values of 0.05 or less. The mean age of the study population was 44 ± 12 years with a female to male ratio of 1.3:1.0. Men presented with subdural hematomas ( P  = 0.001) more often than women. Meningeal enhancement on contrast magnetic resonance imaging (MRI) was the most consistent radiographic finding. Radionuclide cisternography (RC) demonstrated thoracolumbar dural leaks in 16 of 22 patients. EBP failures were more common in patients aged 40 and younger than in older patients ( P  = 0.003). Postural headache from SIH was not uniformly responsive to EBP, and had significant comorbidities, especially in men. The management of postural headache in SIH by other techniques to restore brain position and cerebrospinal fluid dynamics should be investigated.     

Atypical spontaneous intracranial hypotension (SIH) with nonorthostatic headache

BACKGROUND: Some patients with spontaneous intracranial hypotension (SIH) often do not demonstrate typical orthostatic headache, which is contrary to the typical SIH syndrome. They usually have an obscure and intermittent headache, regardless of their positional change. OBJECT: The objective of this study is to investigate the clinical course of atypical SIH that manifests with diffuse pachymeningeal enhancement, but no orthostatic headaches. METHODS: Between January 1997 and December 2005, we observed a total of 6 patients who revealed atypical presentations including nonpostural headaches and normal cerebrospinal fluid (CSF) pressure, despite the diffuse pachymeningeal enhancement seen on their MR images. For a comparison of the clinical features and the disease course, 13 other SIH patients with typical clinical manifestations were selected as a control group. RESULTS: Cerebrospinal fluid leakage sites were confirmed in only one patient through a variety of diagnostic tools; in contrast, definite focal CSF leakage sites were found in 12 of 13 patients with typical SIH. The 6 atypical SIH patients were treated with conservative treatment, including strict bed rest and intravenous hydration for 2 to 3 weeks. After a mean follow-up of 12 months, their headaches were gradually relieved after 2 to 3 weeks of conservative treatment only. CONCLUSIONS: All SIHs do not necessarily show the typical clinical manifestations. The atypical finding of SIH such as nonorthostatic headache or normal CSF pressure may be the result of a normal physiologic response to the typical SIH as a compensatory reaction. Therefore, when faced with patients showing findings compatible with SIH on the brain MR images, regardless of nonpostural headache, atypical SIH should be suspected.     

Chronic paroxysmal hemicrania: the first possible bilateral case

There are three headaches syndromes that are typically characterized by strictly unilateral and always same-sided attacks: cluster headache, "cervicogenic" headache, and chronic paroxysmal hemicrania (CPH). In rare cases, cluster headache also occurs bilaterally; "cervicogenic" headaches probably as well. We present a patient with a probable bilateral CPH. To our knowledge no such case has previously been described.     

A transient third cranial nerve palsy as presenting sign of spontaneous intracranial hypotension

Spontaneous intracranial hypotension is an uncommon cause of sudden and persistent headache: associated symptoms are common, among which there are cranial nerve palsies, especially of the abducens nerve. We report a case of a 21-year-old man with a transient and isolated third nerve palsy due to spontaneous intracranial hypotension. To our knowledge, there are only few reports in the literature of such association.     

Trigemino-autonomic headache related to Gasperini syndrome

We report the association of ipsilateral trigemino-autonomic headache to a case of right-sided nuclear facial and abducens palsy (Gasperini syndrome), ipsilateral hypacusis and right hemiataxia, caused by the occlusion of the right anterior inferior cerebellar artery. Short-lasting attacks of mild to moderate ipsilateral fronto-periorbital head pain, accompanied by lacrimation and mild conjunctival injection during more severe attacks, were present from the onset of symptoms, with a gradual worsening over the next few months and remitting during naproxen therapy. Magnetic resonance imaging showed an infarct in the right cerebellar peduncle, extending toward the pontine tegmentum, also involving the ipsilateral spinal trigeminal nucleus and tract and the trigeminal entry zone. Gasperini syndrome may be accompanied by ipsilateral trigemino-autonomic head pain.     

New daily persistent headache

New daily persistent headache was first described by Vanast in 1986 as a benign form of chronic daily headache that improved without therapy. In the headache specialist’s office, new daily persistent headache is anything but benign and is thought to be one of the most treatment refractory of all headache conditions. Little is known about this syndrome. It is unique in that the headache begins daily from onset, typically in a patient without a history of headache, and can continue for years without any sign of alleviation despite aggressive treatment. This article discusses the epidemiology, diagnostic criteria, clinical characteristics, and treatment strategies for new daily persistent headache.     

Management of an intracranial hypotension patient with diplopia as the primary symptom: A case report

BACKGROUNDIntracranial hypotension (IH) is a disorder involving cerebrospinal fluid (CSF) hypovolemia due to spontaneous or traumatic spinal CSF leakage and is easily being misdiagnosed or missed, especially in these patients without the prototypical manifestation of an orthostatic headache. At present, the management of IH with both cranial nerve VI palsy and bilateral subdural hematomas (SDHs) is still unclear.CASE SUMMARYA 67-year-old male Chinese patient complained of diplopia on the left side for one and a half mo. Computed tomography revealed bilateral SDHs and a midline shift. However, neurotrophic drugs were not effective, and 3 d after admission, he developed a non-orthostatic headache and neck stiffness. Enhanced magnetic resonance imaging revealed dural enhancement as an additional feature, and IH was suspected. Magnetic resonance myelography was then adopted and showed CSF leakage at multiple sites in the spine, confirming the diagnosis of having IH. The patient fully recovered following multiple targeted epidural blood patch (EBP) procedures.CONCLUSIONIH is a rare disease, and to the best of our knowledge, IH with diplopia as its initial and primary symptom has never been reported. In this study, we also elucidated that it could be safe and effective to treat IH patients with associated cranial nerve VI palsy and bilateral SDHs using repeated EBP therapy.     

Spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is an increasingly recognized syndrome. Postural headache with typical findings on magnetic resonance imaging (MRI) are the key to diagnosis. Delay in diagnosing this condition may subject patients to unnecessary procedures and prolong morbidity. We describe a patient with SIH and outline the important clinical and radiographic features of this syndrome. Headache due to SIH is similar to headache occurring after lumbar puncture. Patients with postural headaches should have brain MRI before lumbar puncture. Meningeal abnormalities with typical clinical features are helpful in establishing the diagnosis. When correctly diagnosed, SIH management, in most cases, is easy and highly effective.