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Objective:To investigate the clinical characteristics of cerebral hemorrhage caused by tentorial dural arteriovenous fistula (TDAVF).Methods:An unusual TDAVF case admitted to the Department of Neurology, Qilu Hospital, Shandong University in March 2020, complicated with hypertension with successive bilateral basal ganglia hemorrhage in short term was reported. The characteristics of cerebral hemorrhage caused by TDAVF reported in the literature were summarized and analyzed.Results:Digital subtraction angiography (DSA) revealed that there was arteriovenous fistula in the tentorial foramen area of this patient (male, 33 years old), and the TDAVF was fed by the right meningohypophyseal trunk, bilateral middle meningeal artery and posterior cerebral artery. A shunted pouch was present in the tentorial foramen area, and retrograde reflux drainage was seen in the deep venous system, from the meningeal vein to superior sagittal sinus or sigmoid sinus. Transarterial embolization was performed and subsequently DSA showed obliteration of the fistula. This patient experienced no clinical decline or rehemorrhage during the 12 months follow-up period. Forty-one cases of TDAVF with hemorrhage of cerebral parenchyma which were reported before March 30, 2021 with detailed clinical and imaging data were summarized. The average age of onset of this group of patients was 57.2 years, and the ratio of male to female was about 3∶1. The hemorrhage was located in superior of the tentorium in 17 cases (41%), while in inferior of the tentorium in 24 cases (59%). Supratentorial intracerebral hemorrhage mainly occurred in occipital lobe and thalamus. DSA showed that the arteriovenous fistula was classified as Borden type Ⅲ or Cognard type Ⅳ in 36 cases (88%). Twenty-nine patients (71%) underwent a single surgical procedure, while 12 cases (29%) underwent combined surgical or other treatments. Overall, 37 patients (90%) achieved angiographically documented obliteration of the fistula and 39 patients (95%) experienced good or excellent outcomes.Conclusions:TDAVF often presents as cerebral parenchymal hemorrhage which is common in supratentorial region, but rare in basal ganglia region. The cause of cerebral hemorrhage in patients with hypertension may not be attributed to hypertension. Early diagnosis and intervention are of great significance to improve the prognosis of patients.
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Objective:To summarize the clinical, pathological and molecular biological characteristics of one patient of paranodal disease with anti-contactin-associated protein 1 (Caspr 1) antibodies.Methods:The patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) admitted to Qilu Hospital of Shandong University from August 2018 to December 2020 were retrospectively studied. The clinical data of one acute onset CIDP patient with anti-Caspr 1 antibodies were collected and retrospectively analyzed with literature review. Anti-nodal/paranodal IgG and their subclasses in serum and cerebrospinal fluid (CSF) were investigated by immuno?uorescence assays. Pathological characteristics were explored by sural nerve biopsy further.Results:The patient presented with tremor, ataxia and neuropathological pain besides symmetrical limb muscle weakness and hypaesthesia. The CSF protein was elevated significantly. The brachial plexus and lumbosacral plexus magnetic resonance imaging showed enlarged nerve roots. The patient was responsive well to intravenous immunoglobulin and steroids in acute phase, while the symptoms improved significantly with rituximab in chronic phase. Autoantibodies against Caspr 1 were detectable in serum and CSF, with IgG4 predominant. Sural nerve biopsy revealed segmental demyelination and myelin digestion chamber. Dispersed lamellae of myelin sheath and axonal degeneration were confirmed by electron microscopy.Conclusions:Tremor, ataxia, neuropathic pain, significantly elevated CSF protein and enlarged nerve roots are suggestive of paranodal diseases with anti-Caspr 1 antibodies. For patients with suspected Guillain-Barre syndrome/CIDP and above phenotypes, nodal/paranodal antibodies and antibody subtypes should be detected to optimize the treatment.
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Objective To describe the features of clinical,imaging and cerebral spinal fluid (CSF) of listerial rhombencephalitis to improve the understanding of this disease in clinical practice.Methods All the clinical data of three cases of listerial rhombencephalitis from April to August 2017 in Qilu Hospital were collected and analyzed retrospectively.Results All the three cases were healthy adult women before,with a rapidly progressive course,beginning with fever (38.2-40 ℃),headache accompanied by nausea and vomiting,followed by cranial paralysis,dysphagia and paralysis of the limbs on the 2nd to 5th day of onset,and developed to acute respiratory failure and unconsciousness on the 5th to 8th day of onset.All the three patients were diagnosed with CSF culture positive for Listera monocytogenes on the 3rd to 5th day after admission.The initial CSF lactic acid increased significantly,representing 12.3,12.0 and 10.0 mmol/L respectively;CSF white blood cells were 416× 106/L,760× 106/L and 793× 106/L,respectively,and the protein levels were 0.76 g/L,0.57 g/L and 1.47 g/L,respectively.Brain images showed brain stem was involved in all the three patients,therein cases 1 and 3 with cerebellar hemisphere involved,case 2 with upper cervical spinal cord involved,and case 1 with supratentorial hydrocephalus involved.After treatment with sensitive antibiotics,case 1 recovered,case 2 died,and case 3 lived with dysphagia.Conclusions Listerial rhombencephalitis should be suspected when a patient started with fever and headache,rapidly progressed to cranial nerve paralysis,brainstem symptoms,and acute respiratory failure,especially when the brain imaging suggested brainstem involved with increased white blood cells and apparently elevated lactic acid level of CSF.The earlier the sensitive antibiotics initiated,the better the patients' prognosis.
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Objective To investigate the clinical and pathological features of Guillain-Barré syndrome with treatment-related fluctuations (GBS-TRF).Methods Clinical data were obtained from medical records of patients with GBS-TRF during the period 1999 to 2014 in our Hospital.Sural nerve specimens were collected and summarized retrospectively (two cases).Results Eight of 868 cases with GBS had at least one TRF including three chronic hepatitis B patients.The onset of disease was ranged in age from six to 63 years, averaging 34 years.It is more common in men than in women in a ratio of seven to one.Triggering infections occurred in three patients.The initial symptom included weakness of the lower limbs (five cases) and upper extremities (three cases).Sensory symptom was presented in six patients.Five patients had associated respiratory paralysis.None of them had cranial nerve palsy or autonomic dysfunction.The examination of cerebrospinal fluid showed protein and cell separation.Five patients had two attacks, one had three attacks and two had six attacks.The interval between attacks ranged between 14 days and 46 days (mean 23 days).The striking pathologic finding was the presence of sectional selective nerve fiber degeneration (SNFD) with evidence of demyelination.Conclusions Patients with GBS-TRF show similar onset age, preceding infection, cerebrospinal fluid findings, and electrophysiologic characteristics comparing to patients with GBS,while there are more male patients than female patients.SNFD found in sural nerve biopsy reveals ischemic neuropathy, which predicts that injury of arterioles might play an important role in the pathogenesis of GBS-TRF.
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Objective To investigate the clinical and pathological features of Guillain-Barré syndrome with treatment-related fluctuations ( GBS-TRF ).Methods Clinical data were obtained from medical records of patients with GBS-TRF during the period 1999 to 2014 in our Hospital.Sural nerve specimens were collected and summarized retrospectively ( two cases ).Results Eight of 868 cases with GBS had at least one TRF including three chronic hepatitis B patients.The onset of disease was ranged in age from six to 63 years, averaging 34 years.It is more common in men than in women in a ratio of seven:one.Triggering infections occurred in three patients.The initial symptom included weakness of the lower limbs ( five cases ) and upper extremities ( three cases ).Sensory symptom was presented in six patients.Five patients had associated respiratory paralysis.None of them had cranial nerve palsy or autonomic dysfunction.Five patients had two attacks , one had three attacks and two had six attacks.The interval between attacks ranged between 14 days and 46 days ( mean 23 days ).The striking pathologic finding was the presence of sectional selective nerve fiber degeneration ( SNFD ) with evidence of demyelination.Conclusions Patients with GBS-TRF shows similar onset age , preceding infection , cerebrospinal fluid findings, and electrophysiologic characteristics comparing to patients with GBS ,while there are more male patients than female patients.SNFD found in sural nerve biopsy reveals ischemic neuropathy , which predicts that injury of arterioles might play an important role in the pathogenesis of GBS -TRF.
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Objective To investigate the clinical and pathological features of riboflavin-reactive lipid storage myopathy.Methods Clinical material of 4 patients with riboflavin-reactive lipid storage myopathy were analyzed retrospectively.Results All the patients were subacute onset and presented proximal and axial muscle weakness accompanied by intolerance to excise.Amyotrophy and weakness involved in neck and paraspinal muscle were found in 3 cases,and chewing muscle weakness in 2 cases.Electromyogram showed myogenic changes in 2 cases and reduced conductive velocity of tibial nerves in 1 case.Muscle Biopsy study showed markedly increased lipid droplets in muscle fibers.The ragged red fibers,succinate dehydrogenase strongly reactive vessels and COX deficiency fibers which supported mitochondrial myopathy were not detected.Electromicroscope observation revealed that the structure and quantity of mitochondria were normal.All the patients had a dramatic response to riboflavin treatment.Two cases were cured and the other two were improved significantly.Two cases relapsed 1 year and 5 years later,respectively,and riboflavin was still effective for them.Conclusions Riboflavin-reactive lipid storage myopathy is a myopathy characterized by preferential involvement of neck,paraspinal and chewing muscles.The distinct pathological features are lipid accumulation in muscle fibers without any abnormalities of mitochondrial structure and quantity.Riboflavin alone is effective for this myopathy and this is distinguished from other myopathies.
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Objective To investigate the clinical,genetic and pathological features of familial chronic progressive external ophthalmoplegia (CPEO) type of mitochondrial myopathy.Methods Clinical manifestations, family histories and pathological findings of 21 patients with CPEO type of mitochondrial myopathy from 3 families constellations were analyzed retrospectively.Results All the patients had ptosis and movement disorder of eyeball, with or without myasthenia. An autosomal dominant pattern of transmission was deduced from one family and a maternal transmission appeared most likely in the other two families. The striking and common pathologic findings were presence of ragged red fibers and cytochrome C oxidase (COX) deficiency fibers under microscope. Ultrastructural alterations included subsarcolemmal accumulation of mitochondria, increase of mitochondria with abnormal shape, disarrangement of cristae and paracrystaline inclusion bodies.Conclusions The clinical and pathological features between generations and families seem to be similar. It is suggested that different genetic mode of CPEO may lead to similar clinical and pathological features.