Subarachnoid and intra-cerebral hemorrhage in young adults: Rare and underdiagnosed

Objectives

Convexity subarachnoid and intra-cerebral hemorrhages, in patients aged < 50 years, are always a diagnostic challenge. This condition is characterized by acute headaches with or without neurological symptoms and/or seizures, and by the radiological demonstration of subarachnoid and/or intra-cerebral hemorrhages and, more rarely, by the association of ischemic events.

Patients and methods

In a prospective series of 30 consecutive patients (median age 31 years; 22 women) with a subarachnoid and intra-cerebral hemorrhages, 19 were diagnosed with reversible cerebral vasoconstriction syndrome (RCVS), 7 with cerebral venous sinus thrombosis (CVST), and 4 with a bleeding mycotic aneurysm (MA).

Results

RCVS appeared spontaneously in 16 patients and was related to the postpartum period in three cases. Subarachnoid hemorrhage (SAH) was demonstrated in 24 patients as follows: 18 cases were in cortical areas, 4 were in the polygon of Willis, one was inter-hemispheric, and one was inter-hemispheric/intra-cerebral. A convexity pure intra-cerebral hemorrhage (ICH) was recorded in 6 cases. Among the 7 patients suffering from CVST, the superior sagittal sinus was involved in 4 cases, the transverse sinuses (TS) in 2, and the TS plus sigmoid sinus (SS) in one.

Conclusion

The three most common causes in this series were RCVS, followed by CVST and bleeding from MA. Because of atypical clinical or radiological presentations, this large spectrum of etiologies can cause diagnostic difficulties. Therefore, careful analysis is needed to ensure correct and prompt diagnosis and to avoid any dangerous delays in management.     

Reversible cerebral vasoconstriction following carotid endarterectomy

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe "thunderclap" headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction. We report a patient with RCVS following carotid endarterectomy and review the literature on RCVS following carotid revascularisation.     

Treatment of traumatic acute subdural hematoma in adult hydrocephalus patients with cerebrospinal fluid shunt

Objective

The presence of a cerebrospinal fluid (CSF) shunt is a predisposing factor for the development of subdural hematoma (SDH) in patients with hydrocephalus. However, few reports have addressed how patients with a CSF shunt should be treated in the event of traumatic acute SDH. The purpose of this study was to show how post-traumatic management of CSF shunt affects acute SDH in adult patients with hydrocephalus.

Methods

Twelve patients were studied retrospectively. Pressure settings of shunt valve prior to head injury (HI), severity of HI, treatment on admission, changes in SDH thickness and subsequent hydrocephalus were mainly analyzed.

Results

Ten patients experienced mild HI, with nine showing neurological deterioration until admission. Five patients needed surgical hematoma removal soon after admission. SDH recurred in four cases where shunt pressure levels were kept relatively low. Shunt ligation or raising the pressure level in the programmable valve proved effective for controlling postoperative SDH in such cases. Six of the remaining seven patients underwent only shunt ligation or readjustment of pressure level in the programmable valve on admission. SDH thickness was reduced as ventricles dilated without major neurological complications. Four patients showed delayed development of SDH even though shunts were kept ligated.

Conclusions

Hematoma removal alone may result in hematoma recurrence and require a second treatment comprising shunt management to effectively control hematoma. Using shunt management as the only initial treatment can reduce hematoma volume, but some patients may suffer delayed SDH development and require surgery.     

Intracerebral hematoma complicating herpes simplex encephalitis

Objectives

To describe two patients who developed an intracranial hematoma as a complication of temporal lobe encephalitis due to herpes simplex type 1 virus, and to review the literature.

Patients and methods

The first patient, a 45-year-old woman developed a brain hematoma in the location of the encephalitic lesion on day 9 after the onset of herpes simplex encephalitis (HSE) that required surgical evacuation. The second patient, a 53-year-old woman was being treated for HSE; on day 8 after admission a temporal lobe hematoma with midline shift was disclosed due to persistent headache. Both patients survived but were left with sequelae. We conducted a PubMed/MEDLINE search from 1986 to April 2013 on this topic.

Results

We have found 20 additional cases reported in the literature and review their characteristics. Hemorrhage was present on admission in 35% of pooled patients, and consistently involved the area of encephalitis. Clinical presentation of intracranial hemorrhage overlapped the encephalitic symptoms in two-thirds of the patients. Half of patients underwent surgery. Overall, mortality rate was low (5.2%), and half of patients fully recovered.

Conclusions

Intracranial bleeding, although infrequent, can complicate the evolution of herpes simplex encephalitis and should be borne in mind since its presence may require neurosurgery. Although its presentation may overlap the encephalitic features, the lack of improvement or the worsening of initial symptoms, particularly during the second week of admission, should lead to this suspicion and to perform a neuroimaging study.     

Workload of the emergency room neurologists and the neurological inpatient department in a Greek tertiary care hospital

Background

Data concerning the number of patients presenting to the emergency room (ER) and the workload in the departments in the European Union countries are scarce. In contrast to most European practice, Greek public hospitals in cities with more than one public health facilities are on-call not on a daily, but on a rotational basis. Exact data from Greece on the number of patients referred to the ER or the number of patients admitted to regular inpatient departments of the public hospitals under these conditions is lacking.

Patients and Methods

From February 1st, 2006 to January 31st, 2007, we performed an open prospective study on the workload at the ER as well as the inpatient department of the Neurological Clinic of Papageorgiou General Hospital in Thessaloniki, Greece. In addition, we performed an analysis of the number of inpatient admission to the Department of Neurology per trimester from January 1st, 2002 until March 31st, 2008.

Results

During the study period, a total of 5901 patients required neurological examination in the ER (8.75% of all ER patients). In parallel, 2054 patients were admitted to the neurological ward for hospitalization exceeding 24 h with a mean hospitalization of 3.95 days.

Conclusion

These data suggest that the workload of a tertiary care neurological in- and outpatient department is large. More research is needed to determine the impact this has on patient outcomes.     

Progressive neuropsychiatric manifestations of phenylketonuria in adulthood

Introduction

Neuropsychiatric signs and MRI abnormalities can occur in patients with phenylketonuria in adulthood. We describe clinical and radiological features of phenylketonuric patients and we discuss the advantage of continuing diet in adulthood.

Method

We report late onset neuropsychiatric symptoms of four phenylketonuric patients (33–45 years) diagnosed in infancy and report the case of a patient (33 years) diagnosed with phenylketonuria because of late onset neurological signs. We describe clinical and radiological features of these 5 patients, and their evolution under diet and propose a review of the literature.

Results

The main neurological abnormalities in phenylketonuric patients diagnosed in infancy are: brisk reflexes, spastic paraparesis, psychiatric signs that appear 10.5 years after the diet arrest. A leukoencephalopathy was present in 93% of cases and 91.7% improve clinically after poor phenylalanine diet reintroduction. In 4 patients, neurological abnormalities (spastic paraparesis, dementia, Parkinsonism) led to the late diagnosis. Two of them had a leukoencephalopathy on brain MRI. Patients had high levels of phenylalanine (above 1500 μmol/L) when neuropsychiatric signs occurred. Improvement after diet suggests that hyperphenylalaninemia has a direct toxic effect on the brain.

Discussion/Conclusion

The long-term follow-up of phenylketonuric patients is mandatory to depict and treat neurological complications in time. Diet reintroduction is efficacious in most cases.     

Fibrinolytic therapy versus craniotomy for anticoagulant-associated intracerebral hemorrhage

Object

Anticoagulant-associated intracerebral hemorrhages (AAICH) have a high morbidity and mortality, necessitating urgent treatment. We examined outcomes after conventional craniotomy and stereotactic fibrinolytic therapy in a series of patients with anticoagulant-associated hemorrhages.

Methods

Among 129 consecutive surgically treated patients with supratentorial intracerebral hemorrhage, 27 patients with AAICH were identified (mean age 62; range 36–79). Thirteen patients underwent craniotomy for surgical hematoma evacuation, and 14 patients hematoma puncture and catheter placement for clot lysis. The groups had comparable major prognostic factors such as hematoma volume, age, and Glasgow coma scale (GCS) score at admission.

Results

Nine patients died despite treatment (mortality = 33%). Mortality in the craniotomy group was comparable to that of the lysis group (46% versus 21%; p = 0.13). Good outcomes (Glasgow outcome score of 4 or 5) were seen in 3 craniotomy patients (23%) and 2 fibrinolysis patients (14%). Half the patients survived with major neurological deficits (GOS 2 or 3) (n = 13; 48%). One rebleed was observed two days after uneventful craniotomy and hematoma removal, while no patient who underwent fibrinolysis had rebleeding.

Conclusions

Approximately one-fifth of patients with AAICH managed surgically may have good outcomes. Mortality and favourable outcome rates are comparable between craniotomy and fibrinolytic therapy. Fibrinolytic therapy appears to be a reasonable less invasive alternative treatment modality for intracerebral hemorrhage in the anticoagulated patient.     

Posterior reversible encephalopathy syndrome as a complication of acute lupus activity

Objectives

We aimed to describe the clinical and imaging characteristics; associated risk factors and neurological outcome of posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE).

Methods

From October 2001 to January 2007, we identified patients with SLE and the criteria for PRES in our institution, which is a tertiary-care referral center for patients with SLE; the patients were evaluated at baseline and followed to determine the clinical outcome.

Results

We identified 22 episodes of PRES in 21 patients; 20 (95.2%) were women, mean age of onset was 24.9 ± 8.6 years, all patients had high systemic activity (SLEDAI scores from 12 to 39). Acute hypertension was observed in 18 episodes (81.8%), and renal failure in 16 (72.7%); only 3 patients were on cyclophosphamide at the time of the onset of PRES. Persistent neurological deficit was observed in 2 cases; one patient died during the acute episode.

Conclusions

PRES is a central nervous system syndrome that is observed in SLE patients. It was associated mainly to high systemic activity, acute hypertension, and renal failure. Although reversibility is common, residual neurological damage may be observed.     

Manifestaciones neurológicas de los mixomas cardiacos. Experiencia en un centro de referencia

Introduction

Cardiac myxoma is an important but uncommon cause of stroke in younger patients. Few published case series analyse the frequency and clinical presentation of neurological complications in patients with myxoma.

Objective

To list all neurological complications from cardiac myxoma recorded in our hospital in the past 28 years.

Patients and methods

We retrospectively reviewed the neurological manifestations of cardiac myxoma in patients treated in our hospital between December 1983 and March 2012.

Results

Of the 36 patients with cardiac myxoma, 8 (22%) presented neurological manifestations. Half were women and mean age of patients was 52.4 ± 11.6 years. Sudden-onset hemiparesis was the most frequent neurological symptom (63%). Established ischaemic stroke was the most common clinical manifestation (75%), followed by transient ischemic attack. The most commonly affected territory corresponded to the middle cerebral artery. Myxoma was diagnosed by echocardiography in all cases. Mean myxoma size was 4.1 cm and most of the tumours (63%) had a polypoid surface. All tumours were successfully removed by surgery. There were no in-hospital deaths.

Conclusions

Cardiac myxomas frequently present with neurological symptoms, especially ischaemic events (established stroke or transient ischaemic attack), in younger patients with no cardiovascular risk factors. The anterior circulation is more frequently affected, especially the middle cerebral artery. Echocardiography can facilitate prompt diagnosis and early treatment of the lesion.     

Risk factors for inpatient venous thromboembolism despite thromboprophylaxis

Introduction

Venous thromboembolism (VTE) is the most common preventable cause of morbidity and mortality in the hospital. Adequate thromboprophylaxis has reduced the rate of hospital-acquired VTE substantially; however, some inpatients still develop VTE even when they are prescribed thromboprophylaxis. Predictors associated with thromboprophylaxis failure are unclear. In this study, we aimed to identify risk factors for inpatient VTE despite thromboprophylaxis.

Materials and methods

We conducted a case–control study to identify independent predictors for inpatient VTE. Among patients discharged from the BJC HealthCare system between January 2010 and May 2011, we matched 94 cases who developed in-hospital VTE while taking thromboprophylaxis to 272 controls who did not develop VTE. Matching was done by hospital, patient age, month and year of discharge. We used multivariate conditional logistic regression to develop a VTE prediction model.

Results

We identified five independent risk factors for in-hospital VTE despite thromboprophylaxis: hospitalization for cranial surgery, intensive care unit admission, admission leukocyte count > 13,000/mm³, presence of an indwelling central venous catheter, and admission from a long-term care facility.

Conclusions

We identified five risk factors associated with the development of VTE despite thromboprophylaxis in the hospital setting. By recognizing these high-risk patients, clinicians can prescribe aggressive VTE prophylaxis judiciously and remain vigilant for signs or symptoms of VTE.     

Intracranial intraparenchymal and intraventricular schwannomas: Report of 18 cases

Objective

Intracranial schwannomas of the brain, which are unrelated to the cranial nerves, are extremely rare. In this article, we present a series of eighteen cases of intracranial intraparenchymal and intraventricular schwannomas, which is the largest series to date.

Methods

During the 10-year period from January 2000 to October 2010, we encountered 2491 histologically established cases of intracranial schwannomas, of which only 18 were not related to the cranial nerves. Clinical profiles, radiological features, surgical procedures, intraoperative findings and outcomes were extracted from the patient records and neuroimaging data.

Results

No patients were preoperatively diagnosed with schwannoma. The diagnosis of schwannoma was made by pathological examination with H&E staining and immunohistochemical examination. The 18 cases with intracranial ectopic schwannomas account for 0.8% of all the intracranial schwannomas that were observed within the same time period at our hospital. The age distribution of the patients ranged from 7 to 78 years. There was a slight male predominance: 11 male and 7 female patients (M:F = 1.6:1). Headaches were the most common presenting symptom and were found in most cases. Common neuroradiological characteristics included peritumoral edema and intralesional cysts.

Conclusion

Intracranial intraparenchymal and intraventricular schwannomas are rare, benign neoplasms that cannot be preoperatively differentiated from other parenchymal tumors. Surgical excision is curative, and the long-term prognosis is good. Additional studies are needed to confirm the histogenesis of this schwannoma type.     

Management outcome of NPC-related and non-NPC-related brain abscess in Hong Kong

Objectives

(1) To review the patient profile, management outcome and prognostic factors of brain abscess; (2) To compare the neurological outcome of nasopharyngeal carcinoma (NPC)-related brain abscess with non-NPC related brain abscess.

Method

Retrospective review of consecutive patients diagnosed (radiologically and/or microbiologically) with brain abscess in a regional neurosurgical center in Hong Kong over a nine year period.

Results

Fifty-four patients were recruited into this study. There were 37 male and 17 female patients. Eighteen (33%) patients had previous radiotherapy for nasopharyngeal carcinoma. Only 31 (57%) patients had fever on presentation. White cell count and/or C-reactive protein, was raised in 41 (76%) patients on admission. Surgical drainage was carried out in 49 (91%) patients, either by aspiration through a craniotomy, by drainage with corticotomy, or excision of the abscess. Abscess culture was positive in 45 (83%) patients. Common organisms isolated included Streptococcus species (35%) and Peptostreptococcus species (18%). Anaerobes were isolated in 50% of the NPC-related abscesses. The mean follow-up time was 34 months. At the 6 months interval, 24 (44%) patients had good recovery. Favorable outcome was achieved in 30 (55%) patients. NPC-related brain abscess was associated with unfavorable neurological outcome (33%, p = 0.04). There was also a trend towards higher in-patient mortality in patients with NPC-related brain abscess (22%, p = 0.08).

Conclusion

Brain abscess carried a substantial morbidity and mortality despite aggressive surgical and medical treatment. Patients with NPC-related brain abscess had a higher mortality and unfavorable neurological outcome.     

Elderly patients are at increased risk for mortality undergoing surgical repair of dens fractures

Objective

Dens fractures are common cervical injuries in advanced aged patients. The presented study was undertaken to analyze the clinical results and risks of surgically treated patients with dens fractures over 70 years.

Methods

Data of 28 patients (17 female, 11 male) over 70 years treated from September 2004 to October 2009 were recorded. Clinical and radiological parameters were obtained including type of fracture, associated cervical and/or other injuries, comorbidities, symptoms, neurological condition, surgical strategy, postoperative course and complications.

Results

89% were in a good neurological condition before surgery (ASIA E or D). In most cases, surgery was performed at an early stage after trauma (21 patients within 5 days). Ventral screw fixation was the preferred surgical strategy (64%). A slight worsening of neurological functions immediately after operation was only seen in one patient. Five patients died in the early and 2 in the late postoperative course which means a treatment mortality of 25%. Among the surviving patients two had general medical complications.

Conclusion

Type II dens fractures are a common fracture of elderly patients. Our results are good concerning the neurological functions. Surgical and general medical complications were acceptable. However, the study also underlines that mortality rate is high and therefore treatment options should be well-considered in this high risk group.     

Salt-and-pepper eye pain and brainstem stroke

Background and purpose

The salt-and-pepper pain is a characteristic sensory disturbance confined to the eyes and regional facial structures. Although a poor prognosis has been mentioned in previously reported patients, the precise pathomechanism and clinical significance are still unknown.

Patients and methods

We report four patients with ocular salt-and-pepper pain, and review the clinical course, neuroimaging and prognosis in another eight patients reported in the literature.

Results

In our series, they were three men and one woman, and their underlying cause was pontine hemorrhage; hypertensive hemorrhage in three and cavernous hemangioma in one patient, respectively. In these 12 salt-and-pepper patients, the identifiable etiology was exclusively brainstem stroke. Life-threatening or disable neurological deterioration ensued within 24 h after pain onset in all patients. Their ocular pain subsided rapidly after neurological deterioration occurred. A dual excitation of nociceptive quinothalamic pain fiber and disinhibition of trigeminosensory system from pontine reticular formation and cerulotrigeminospinal circuit may be responsible for this pain.

Conclusion

In clinical practice, ocular salt-and-pepper pain in quiet eyes should be alerted for intracranial pathology and neurological deterioration until underlying cause is identified.     

Neurological dysfunction induced by bilirrubin

Introduction

“Kernicterus” is a term currently used to describe bilirrubin induced brain injury in the neuro-pathological studies. This is a confusing term and nowadays we prefer bilirrubin encephalopathy or bilirrubin induced neurological dysfunction. The clinical signs vary and it is clearly decreasing in prevalence in developed countries.

Material and methods

We review a series of 7 patients with bilirrubin encephalopathy and variable neurological manifestations, who were seen in the Neuropaediatric Department in the last 10 years. Only one patient died in the neonatal period with hyperbilirubinaemia, sepsis and multi-organ failure.

Results

Diverse aetiological factors were related to hyperbilirubinaemia. All patients had clinical symptoms due to hyperbilirubinaemia. Neuroimaging during the neonatal period showed involvement of the nucleus pallidus, with hyperintensity in T1 in the brain MR scan as the most consistent finding. All the patients who survived developed neurological signs and we try to correlate them with biochemical, clinical, neuroimaging and neurophysiological parameters.

Conclusions

An increase in the number of patients with bilirrubin encephalopathy has been observed over the last few years, and we attempt to find out the causes. The increased survival of the low birth weight newborns, the increase in the immigration population and the use of diagnostic neuroimaging contribute to this increase. It is a great challenge for the neonatologist and for neuropaediatricians to prevent its occurrence and to minimise the effects of bilirrubin encephalopathy.     

Labor asistencial del equipo de guardia de neurología en un hospital terciario de Madrid: análisis prospectivo durante un año

Introduction

Population ageing, the rising demand for healthcare, and the establishment of acute stroke treatment programs have given rise to increases in the number and complexity of neurological emergency cases. Nevertheless, many centres in Spain still lack on-call emergency neurologists.

Methods

We conducted a retrospective study to describe the role of on-call neurologists at Hospital General Universitario Gregorio Marañón, a tertiary care centre in Madrid, Spain. Sociodemographic characteristics, most common pathologies, diagnostic tests, and destination of the patients attended were recorded daily using a computer database. Results were compared with the general care data from the emergency department.

Results

The team attended 3234 patients (3.48% of the emergency department total). The mean number of patients seen per day was 11.15. The most frequent pathologies were stroke (34%), epilepsy (16%) and headache (8%). The mean stay in the emergency department was 7.17 hours. Hospital admission rate was 40% (7.38% of emergency hospital admissions). The main destinations for admitted patients were the stroke unit (39.5%) and the neurology department (33%). Endovascular or thrombolytic therapies were performed on 76 occasions. Doctors attended 70% of the patients during on-call hours.

Conclusions

Emergency neurological care is varied, complex, and frequently necessary. Neurological cases account for a sizeable percentage of both patient visits to the emergency room and the total number of emergency admissions. The current data confirm that on-call neurologists available on a 24-hour basis are needed in emergency departments.     

Risk factors and short-term outcome in patients with angiographically negative subarachnoid hemorrhage

Objectives

The objective of this study was to identify prognostic factors for clinical outcome in patients with non-traumatic, angiographically negative subarachnoid hemorrhage. Furthermore, the aim was to investigate if patients on anticoagulant therapy may have a more unfavorable outcome than patients not receiving anticoagulant therapy.

Materials and methods

This study contains a retrospective analysis of 95 patients with non-traumatic, non-aneurysmal subarachnoid hemorrhage who were admitted to our institution in the period 2000–2011. The patients were classified according to risk factors, clinical presentation at admission, anticoagulant therapy at the time of hemorrhage and complications. The outcome was assessed with modified Rankin Score at discharge.

Results

Patients who were exposed to risk factors combined with a higher WFNS score at admission had an unfavorable outcome according to the modified Rankin Score at discharge. Unfavorable outcome was evaluated in relation to sex, anticoagulant therapy, hypertension, smoking and alcohol abuse. A strong correlation with anticoagulant therapy, smoking and alcohol abuse was found. None of the patients who developed vasospasms in this study were receiving anticoagulant therapy.

Conclusion

There is a correlation between antithrombotic state at admission and unfavorable outcome for patients with non-traumatic, non-aneurysmal subarachnoid hemorrhage. The results from this study indicate that patients on anticoagulant therapy have a higher risk of developing complications after non-aneurysmal angiographically negative subarachnoid hemorrhage compared to patients who were not receiving anticoagulant therapy.     

Neuroendoscopic options in the treatment of mesencephalic expanding cysts: Report of four cases and review of the literature

Objective

Mesencephalic expanding cysts, also called lacunae, are rare intraparenchymal, multilobulated cavities of variable diameter mostly localized in the thalamo-mesencephalic region. In symptomatic cases, usually presenting with hydrocephalus or midbrain syndrome, surgical treatment is required and, considering their position, a minimally invasive approach should be preferred.

Methods

Four cases of expanding mesencephalic cysts endoscopically treated in three different Italian centers are described. Other possible causes of intracerebral cyst were excluded in all cases by complete neuroimaging and laboratory screening. All patients presented with signs and symptoms of midbrain compression and a slight to moderate ventricular dilation was present in three cases.

Results

All patients underwent endoscopic cyst fenestration into the ventricle, associated with endoscopic third-ventriculostomy (ETV) in two cases and with cyst wall biopsy in one case. One patient suffered from transient worsening of her hemiparesis due to intraoperative bleeding. All patients showed clinical improvement and a reduction in cyst size on follow-up magnetic resonance images (MRI).

Conclusion

Neuroendoscopy appears to be an effective, probably definitive surgical option in the treatment of symptomatic mesencephalic expanding cysts. Associating ETV with cyst fenestration seems to offer more complete treatment. Deep intracystic navigation and cyst wall biopsy should be avoided.     

Traumatic cervical artery dissection in head injury: The value of follow-up brain imaging

Introduction

Traumatic cervical artery dissection (TCAD) is a relative infrequent complication of traumatic brain injury (TBI). Since TCAD is associated with morbidity in a considerable percentage of patients, it is important to obtain clues for recognising TCAD in this category of patients.

Methods

Retrospective case-cohort study in severe TBI patients.

Results

Five patients with traumatic cervical artery dissection after severe TBI, leading to ischemic strokes, are described. Secondary deterioration to coma was present in four out of five patients during admission. The diagnosis of TCAD was delayed in most cases because the secondary deterioration was often attributed to multisystem problems related to trauma patients, i.e. shock or hypoxia or medication effects. Local clinical symptoms and signs suggestive of TCAD are difficult to detect in this patient group. In all patients, the CT-scan on admission demonstrated no abnormalities. A follow-up scan at day 2 revealed that in all patients abnormalities in the vascular territories had evolved.

Conclusion

With this case-cohort study we underline the importance of considering TCAD in severe TBI patients and emphasise the role for standard follow-up brain imaging. Also possible treatment consequences are discussed.