Intracranial hypotension syndrome following chiropractic manipulation of the cervical spine

Cervical spine manipulation has been associated with several disorders such as cervical arteries dissection, but rarely has a relationship with intracranial hypotension been reported. We describe a patient showing intracranial hypotension syndrome following chiropractic cervical spine treatment. Magnetic resonance showed the presence of dural leakage at cervical level, suggesting the pathogenesis of the syndrome. We state that cervical spine manipulation should be considered a treatment with risk of neurological complications, including the occurrence of intracranial hypotension.     

Spontaneous intracranial hypotension syndrome: report of twelve cases

PURPOSE: To investigate clinical, MRI, and radioisotope findings and therapeutic outcome of the syndrome of spontaneous intracranial hypotension (SIH). BACKGROUND: Spontaneous intracranial hypotension is characterized by orthostatic headache, low CSF pressure, and MRI findings of diffuse pachymeningeal gadolinium enhancement without previous history of head trauma or lumbar puncture. Spontaneous CSF leakage from a spinal dural tear has been suggested as the underlying pathogenic mechanism of SIH. Most patients recover without sequelae, but subdural collections have been described in a few. METHODS: Twelve consecutive patients (10 females, 2 males, mean age 39 years) with headache related to the syndrome of spontaneous intracranial hypotension were investigated. RESULTS: Eleven patients presented orthostatic headache, one patient had continuous nonpostural headache. Additional clinical symptoms included nausea, vomiting, tinnitus, diplopia, and back pain. All the patients had low CSF opening pressure, seven had increased CSF albumin, and four had pleocytosis. Brain MRI showed diffuse pachymeningeal gadolinium enhancement. Other features included subdural fluid collections (hematoma/hygroma) in four patients, downward displacement of the brain in four patients, and enlargement of the pituitary gland in one patient. Radioisotope cisternography results indicated, in two patients, a CSF leakage site in the cervico-thoracic region, and in one patient showed limited ascent of the tracer to the cerebral convexity and early appearance of radioisotope in the bladder. All the patients had complete resolution of headache with conservative treatment. CONCLUSIONS: Patients with SIH have distinct MRI and sometimes radioisotope cisternographic abnormalities and generally respond favorably to conservative management.     

Bilateral subdural hematomas following routine lumbar diskectomy

Intracranial hypotension is a rare, and possibly underrecognized, cause of headache in middle age. Occurring spontaneously in the vast majority of cases, it has been occasionally reported after certain neurosurgical procedures involving craniectomy. We report a unique situation in which a patient developed severe postural headache typical of intracranial hypotension, which was complicated by bilateral subdural hematomas, immediately following a routine lumbar diskectomy; the headache resolved spontaneously. We suggest that an intraoperative microscopic dural breach was the site of sustained, but self-limited, cerebrospinal fluid leakage that eventually led to intracranial hypotension.     

Overlapping syndrome]

Overlapping syndrome (OS) is usually used as the term of the combinations of three connective tissue diseases, i.e., systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS) and polymyositis (PM) or dermatomyositis (DM). OS is sometimes confused with mixed connective tissue disease (MCTD) since the definitions of the both diseases have not been established yet. Rheumatoid arthritis (RA) is a distinct disease and only exceptionally associated with the other CTD. These rare cases include destructive arthritis of SLE and PSS, multiple peripheral type of psoriatic arthritis, and arthritis associated with X-linked hypogammaglobulinemia and selective IgA deficiency. The conditions complicated with RA are not uncommon. They are osteoporosis, Sjogren's syndrome, amyloidosis and so on. There are some rare conditions or diseases which will be able to develop to RA. These peculiar cases include juvenile rheumatoid arthritis, adult onset Still's disease, polymyalgia rheumatica and palindromic rheumatism.     

Intrathecal gadolinium-enhanced MR-cisternography in spontaneous intracranial hypotension associated with Behcet's syndrome

We report a 32-year-old woman diagnosed with spontaneous intracranial hypotension (SIH) and Behcet's Syndrome. Suspicion of SIH was based on cervical MRI findings, and magnetic resonance (MR)-cisternography revealed dural leaks bilaterally at lumbar region. MR-cisternography is a recently performed technique and gaining importance in demonstration of CSF fistula.     

Recent increase in non-tuberculous mycobacterial infection in patients with connective tissue diseases in Japan

BackgroundNon-tuberculous mycobacterial (NTM) infection is currently a growing health concern due to the increasing incidence and the need for prolonged therapy. In patients with connective tissue diseases, use of immunosuppressants may lead to an increased risk of NTM infection. However, few studies have examined the recent incidence of NTM infection among connective tissue diseases patients. This study investigated recent trends in NTM infection among connective tissue diseases patients.MethodsWe included adult patients from whose cultures NTM were isolated between January 2009 and October 2017 in our hospital. By reviewing their medical records, connective tissue diseases patients were identified. Types of connective tissue disease, NTM species, and treatment of NTM infection were extracted.ResultsNTM was isolated from 657 patients during the period. Among these, 24 patients had connective tissue diseases. The number and rate of NTM isolates from connective tissue diseases patients increased during the period, with 4 patients 2009 to 2012 (1.9%), and 20 patients from 2013 to 2017 (3.3%; P = 0.04). The proportion of Mycobacterium avium complex (MAC) to total NTM tended to be lower among connective tissue diseases patients (58.3%) than among non-connective tissue disease-patients (72.8%), but the difference was not significant (P = 0.20). Mycobacterium xenopi was significantly more frequent in connective tissue disease patients than in non-connective tissue diseases patients (P < 0.01).ConclusionThe recent increase in the incidence of NTM infections in connective tissue diseases patients was larger than that in the total population. NTM species other than MAC were isolated from connective tissue diseases patients.     

Pathological contrast enhancement of the oculomotor and trigeminal nerves caused by intracranial hypotension syndrome

The typical symptom of intracranial hypotension syndrome is orthostatic headache. The headache may also be accompanied by neck pain and stiffness, low backache, radicular symptoms, quadriplegia, interscapular pain, nausea/vomiting, and cranial nerve involvement symptoms (hearing and visual problems, face pain and numbness, hypogeusia). Radiologically, on cranial magnetic resonance imaging, intracranial hypotension syndrome is characterized by dural thickening and contrast enhancement, subdural effusion, engorgement of the venous structures, sagging or downward displacement of the brain, and pituitary hyperemia. Although clinical findings related to cranial nerves 3 and 5 have been described in intracranial hypotension, pathological contrast enhancement of these nerves has not. We present a 32-year-old patient whose cranial magnetic resonance imaging shows bilateral pathological contrast enhancement of cranial nerves 3 and 5 and describe a new imaging finding in intracranial hypotension syndrome.     

Patulous Eustachian tube in spontaneous intracranial hypotension syndrome

A 48-year-old slender woman presented with a rare case of spontaneous intracranial hypotension syndrome manifesting as patulous Eustachian tube. The patient complained of sudden onset of ear fullness and nasal voice as well as typical orthostatic headache. Patulous Eustachian tube was identified by observation of synchronous movement of the tympanic membrane with respiration and swallowing. The diagnosis of spontaneous intracranial hypotension was confirmed by negative cerebrospinal fluid pressure, and typical magnetic resonance imaging and radioisotope cisternography findings. All symptoms completely resolved within a few days after epidural blood patch treatment. Changes in the venous blood distribution led by collapse of the dural sac of the cervical spine in the standing position presumably caused decreased size of the pterygoid venous plexus around the Eustachian tube.     

Coexistence of multiple and widespread cardiovascular complications in a patient with Marfan syndrome

Inherited connective tissue diseases such as Marfan syndrome are frequently associated with cardiovascular manifestations. Aortic involvement with dilation and dissection is the most common finding and the major cause of death in Marfan syndrome patients. We report the echocardiographic study of a 53‐year‐old male patient with uncommon coexistence of cardiovascular abnormalities typical of connective tissue disease at first clinical presentation in acute clinical setting: dissection of the descending aorta associated with severe mitral regurgitation due to leaflet flail and massive aortic insufficiency due to ascending aortic enlargement, leading to left ventricular dilation and dysfunction. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound, 2013     

Water-tight closure of spinal dura with a new clot suture technique (author's transl)]

A new clot-suturing Technique (using high-concentration fibrinogen solutions) for water-tight closure of the dura is reported. Six dogs underwent laminectomy of the thoracic spine with medial longitudinal incision in the chordal dura. After the dural split had been sealed with natural tissue adhesive and closure of the wound in layers, the animals were sacrificed at intervals of 1 to 21 days postoperatively and the chordal segments involved were removed and histologically examined. Early fibrinolysis of the clot was prevented by adding a natural proteinase inhibitor and factor XIII concentrate to the clotting substances. Histological analysis showed that healing was almost complete after 2 weeks, with delicate connective tissue overgrowing the dural split. After complete reabsorption of fibrin, the originally abundant absorbent granulation tissue had largely disappeared. Among the satisfactory results of fibrin suturing are optimum healing tendency in the fibrin-sutured region, absence of tissue irritation and neurotoxicity, which are known attributes of the synthetic alkl-cyano-acrylate tissue adhesives.     

结缔组织病累及呼吸系统的影像特点

结缔组织病累及呼吸系统可表现为间质性肺疾病、弥漫性肺泡损伤、肺泡出血、肺血管病变、胸膜病变及气道病变等。其临床表现缺乏特异性, 肺功能和胸部CT是最常用的检查手段。不同结缔组织病累及呼吸系统的影像特征具有相似性, 但又各具特点。本文就结缔组织病呼吸系统受累相关影像表现进行概述, 以提升读者的认知并指导临床实践。     

Effect of phytohemagglutinin on concentrations of glucocorticoid receptors in lymphocytes from patients with connective tissue diseases

We examined the effect of phytohemagglutinin (PHA) on glucocorticoid receptors in lymphocytes, using peripheral mononuclear leukocytes from patients with connective tissue diseases. Glucocorticoid receptor concentrations and dissociation constants (Kd) for [3H]dexamethasone binding to lymphocytes from patients with rheumatoid arthritis, Sj?gren's syndrome, and other connective tissue diseases did not differ significantly from values for normal volunteers. Kd values correlated positively with glucocorticoid receptor concentrations after PHA stimulation both in normal volunteers and in patients with connective tissue diseases. The correlations (r values) for the control group and patients were not significantly different.     

Barium‐induced pneumatosis intestinalis in a patient with anti‐synthetase syndrome

Pneumatosis intestinalis is defined as the presence of gas in the bowel wall. The combination of the two risks, pre‐existing connective tissue diseases and barium contrast examination, may trigger pneumatosis intestinalis, albeit at a low incidence. Clinicians should be aware of the condition for proper differential diagnosis.     

Intracranial hypotension syndrome in a patient due to suboccipital craniectomy secondary to Chiari type malformation

Intracranial hypotension syndrome (IHS) is a rare disorder characterized by a decrease in cerebrospinal fluid pressure to less than 60 mm H₂O. The syndrome is associated with occipital headache radiating to the frontal and temporal zones. The current clinical case describes the manifestation of IHS in a twenty-five year old female with a history of suboccipital craniectomy due to Chiari I malformation nine years earlier. The patient was admitted to the hospital complaining about postural, mainly occipital, headache during the last three months, aggravated by being in an upright position. The magnetic resonance imaging (MRI) revealed engorgement of the dural venous sinuses, significant enlargement of the pituitary gland and download displacement or sagging of the brain with effacement of the perichiasmatic cisterns and the prepontine cistern, while the spinal T2W MRI revealed a 7 mm × 2.5 mm dural defect with an extradural cerebrospinal fluid collection at the dorsal soft tissues of the cervical spine. The previous imaging did not reveal subdural effusions.     

Fascia lata graft as a dural substitute in neurosurgery

We present 37 cases in which fascia lata was used for dural patching when there was inadequate regional tissue, such as pericranium or temporalis fascia to repair the dural defect. Operative indications included tumor in 17 patients (46%), trauma in nine (25%), cerebrospinal fluid fistula in seven (19%), infection in two (5%), dural patching in two (5%). Follow-up ranged from one month to five years, with an average follow-up of two years. There were no cranial or spinal complications related to the fascia lata grafting (including cerebrospinal fluid leakage, meningitis, and wound infection). Fascia lata is relatively simple to obtain, but harvesting necessitates a second incision; this second incision resulted in no complications in our patients.     

Cutaneous immunofluorescence

Immunofluorescence, especially direct immunofluorescence of skin, has been widely used for diagnosis of connective tissue diseases. It is principally of value for LE and may be used also as a prognostic test for SLE. It is of relatively little value for diagnosis of other connective tissue diseases and appears not to predict the outcome of undifferentiated connective tissue disease. Cutaneous immunofluorescence is widely available and has an established place in the field of dermatopathology. As with other microscopic interpretations, substantial experience, along with an understanding of basic immunopathologic principles, is necessary for reliable results. Antibodies to certain ultrastructural components of the basement membrane zone are now available. These are being evaluated for further diagnostic applications to LE and other diseases that involve the epidermal basement membrane zone.     

胸腰椎手术后脑脊液漏的防治

目的 探讨胸、腰椎手术合并硬脊膜破裂的处理及并发脑脊液漏的治疗措施.方法 回顾性分析64例硬脊膜破裂的处理及其9例脑脊液漏的治疗情况.结果 行硬脊膜修补52例,并发脑脊液漏5例;未行硬脊膜修补12例,并发脑脊液漏1例;术中未见硬脊膜损伤而术后发生脑脊液漏3例;9例脑脊液漏患者无一例行切口探查硬脊膜修补术,均经合理卧位、漏口缝合、取头低脚高位、局部加压等治疗痊愈.结论 硬脊膜破裂应积极修补;合理的卧位、头低脚高位对脑脊液漏的防治有十分重要的意义.     

Relationship between snRNA species contained in nuclear antigens recognized by autoantibodies and the clinical profile in systemic rheumatic diseases

Antibodies to extractable nuclear antigens (ENA) are generally used in the diagnosis of connective tissue diseases. Using a rapid, very sensitive method we have shown that extractable nuclear antigens, which are now well-characterized at the molecular level, differ by their RNA content. The method was applied to the sera of 17 patients suffering from different connective tissue diseases. The results show that mixed connective tissue disease (MCTD) and other mild connective tissue diseases are characterized by the presence in the antigen of U1 small nuclear RNA (U1 snRNA) only. On the other hand, antibodies from 6 out of 8 patients tested with Systemic Lupus Erythematosus (SLE) recognize antigens exhibiting a more complex RNA pattern. Three of them precipitated all five snRNAs U2, U1, U4, U5, U6 whereas some snRNAs were lacking or quantitatively less important in precipitates obtained with the three others.     

Isolated Cortical Venous Thrombosis Associated With Intracranial Hypotension Syndrome

The association of intracranial hypotension syndrome with cerebral venous thrombosis is rare. We report our experience with isolated cortical venous thrombosis, which developed after unsuccessful epidural anesthesia. Magnetic resonance imaging showed characteristic imaging findings of intracranial hypotension syndrome, such as dural thickening and brain sagging. We also detected right parietal venous hemorrhagic infarction secondary to right-sided cortical venous thrombosis. After the treatment of intracranial hypotension via epidural blood patch, heparin was used to treat cortical venous thrombosis.     

结缔组织病与肺动脉高压

肺动脉高压是多种结缔组织病在呼吸系统的表现之一，它的发病机理尚不完全明确，由于其症状出现晚且不典型，多数患者在确诊时已发展至肺源性心脏病，治疗难度增加，预后甚差，死亡率高。因此对结缔组织病相关的肺动脉高压早期诊断，及时合理的治疗十分重要。本文就结缔组织病相关的肺动脉高压的流行病学、发病机理、治疗等方面作以综述。