Concurrence of multiple sclerosis and intracranial glioma. Report of a case and review of the literature

We report a 39-year-old female patient known to have multiple sclerosis (MS), who later developed cerebral glioblastoma. The tumor was documented on the brain-magnetic resonance imaging (MRI) during the work-up for an apparent relapsing MS, and was subsequently confirmed pathologically by stereotactic biopsy and the postmortem brain examination. Our case, as well as others, re-emphasizes the need to evaluate the symptoms and brain MRI carefully, even in well-documented MS subjects. The concurrence of MS and intracranial glioma is uncommon. The possible relationship between the 2 diseases was discussed, and related literature reviewed.     

Mollaret's meningitis revisited. Report of a case with a review of the literature

Recurrent aseptic meningitis of Mollaret is a rare condition. We report a Chinese patient with Mollaret's meningitis and describe the characteristic cytological changes in the cerebrospinal fluid. The diagnosis and treatment of this condition is discussed with a review of the English literature.     

Intracerebral chloromas. Report of a case and review of the literature

We report a patient with large intracerebral chloromas while in systemic remission from acute myelogenous leukemia. A favourable outcome resulted from surgical debulking and radiotherapy. Review of other reported cases in the literature showed a uniform female occurrence. We believe that intracerebral chloromas represent reactivation of sanctuary deposits in the central nervous system. The treatment of intracerebral chloroma is discussed.     

Sporadic amyotrophic lateral sclerosis resembling primary lateral sclerosis: Report of an autopsy case and a review of the literature

This report describes the clinicopathological findings of a case of sporadic amyotrophic lateral sclerosis (ALS) resembling primary lateral sclerosis (PLS). A Japanese man developed muscle weakness in the distal part of the right upper extremity at age 59. At age 60 he presented with bradycinesia and rigidity. A neurological examination revealed fasciculation and increased deep tendon reflexes in the extremities. He developed decubitus and vesicorectal disturbance 2 months before his death at age 61. The neuropathological examination revealed not only prom-inent degeneration of the pyramidal tracts, evident in the internal capsule, but also loss of Betz's cells in the motor cortex. There was relative preservation of the neurons in the hypoglossal nuclei and anterior horns of the cervical and lumbar cord. In the anterior horn of the first sacral cord, there were small aggregates of lipofuscin-laden macrophages in locations from which large cells had presumably been lost. Bunina bodies and ubiquitin-immunoreactive neuronal inclusions were present in the anterior horn cells of the spinal cord. On the basis of these clinicopathological findings, we concluded that this case was one of sporadic ALS with predominant involvement of the upper motor neuron system and exhibiting features of PLS.     

The Kleine-Levin syndrome. Report of a case and review of the literature.

Kleine-Levin syndrome is a rare self-limited disorder which usually affects adolescent males and is characterized by episodic hypersomnia, increased appetite, and behavioral/psychiatric disturbances. Individuals are normal between the attacks. The case of an adolescent boy is presented who suffered from recurrent sleepiness, hyperphagia, and behavioral disturbances such as rocking, punching and pacing, and was originally misdiagnosed as suffering from encephalitis. Before the diagnosis of Kleine-Levin was given, the patient underwent unnecessary investigations and treatment which, in turn, complicated his clinical condition both physically as well as psychologically. In the course of five years he had four such episodes which appeared to have progressively milder manifestations. Between episodes he was normal. It is important that the diagnosis is suspected early, especially in adolescent males who present with recurrent episodes of somnolence, increased appetite, and abnormal behavior, since it most often represents a benign and self-limited entity and does not warrant extensive investigations or treatment. It is also important to distinguish this syndrome from more serious organic and psychiatric diseases with more serious prognoses. The differential diagnosis of this syndrome is discussed and a review of the literature is presented including evidence and hypotheses regarding its pathophysiology.     

Tuberculous brain abscess. Report of a case and review of the literature

Tuberculous brain abscess is a rarely reported form of central nervous system tuberculosis. Fifty-seven cases were found in a review of the world's literature; only 16 met rigid diagnostic criteria. Tuberculous brain abscesses are devoid of the granulomatous reaction associated with tuberculosis. Histologically and clinically, these abscesses are similar to pyogenic brain abscesses. An analysis of 16 verified cases from the literature and one reported case showed that tuberculous brain abscesses usually present acutely, often in the third and fourth decades, commonly have a supratentorial location, frequently present with focal neurologic signs, and are associated with historical and laboratory evidence of tuberculosis. Tuberculous brain abscesses may be difficult to differentiate from pyogenic brain abscesses, tuberculomas, and tuberculous meningitis on the basis of clinical, laboratory, and roentgenographic information. Appropriate therapy includes adequate antituberculous chemotherapy and surgical excision.     

经病理确诊的特发性肥厚性硬脑膜炎1例报道并文献复习

目的 探讨特发性肥厚性硬脑膜炎的临床表现、影像学特征、诊断及治疗。方法 报道本院1例经病理确诊的特发性肥厚性硬脑膜炎患者的临床资料并复习相关文献。结果 本例患者表现为慢性反复头痛,头颅磁共振增强扫描示左侧小脑幕异常增生并强化,病理活检提示大量慢性炎症细胞浸润,经过激素冲击治疗头痛缓解,后续小剂量激素联合免疫抑制剂甲氨蝶呤口服,临床预后良好。结论 特发性肥厚性硬脑膜炎病因复杂,多以慢性头痛、多组脑神经麻痹及小脑性共济失调为主要临床表现; 头颅MRI可见特征性硬脑膜肥厚及强化表现; 临床需与多种颅内疾病相鉴别,病理活检可确诊; 激素治疗基础上联合免疫抑制剂可防止病情复发。     

Pasteurella ureae meningitis associated with endocarditis. Report of a case and review of the literature

The first reported case of Pasteurella ureae meningitis associated with endocarditis is described. The patient pursued a fulminant deteriorating course despite appropriate antibiotic therapy begun within 24 h of presentation. Previous reports of Pasteurella ureae meningitis are reviewed. This organism should be considered in the differential diagnosis of gram-negative meningitis, since it is usually responsive to most antibiotics, including penicillin.     

Cerebral manifestations of ergotism. Report of a case and review of the literature.

A patient with diffuse and focal cerebral dysfunction was found to have absent peripheral pulses. Cerbral angiography revealed evidence of an arteritis with bilateral high grade carotid stenosis. When there was no laboratory confirmation of the arteritis, an iatrogenic etiology (ergotism) was suspected. This was later confirmed by the patient. The pertinent literature on ergotism is reviewed, and it is emphasized that ergotism may develop in patients on therapeutic doses of the drug.     

Intracranial neuromuscular choristoma: Report of a case with literature review

Neuromuscular choristoma (NMC), also called neuromuscular hamartoma or nerve rhabdomyoma, is a rare lesion of the spinal and cranial nerves composed of skeletal muscle intimately associated with nerve fibers. Its origin has not been precisely clarified and a malformative event, resulting from aberrant differentiation or a true neoplastic growth, have been proposed by authors. We hereby present a cerebellopontine angle NMC enlarging the eighth cranial nerve in a 3‐year‐old child, that histologically appeared composed of a large amount of striated muscle mixed with nerve fibers. We also provide a review of the intracranial NMC cases reported in the literature and an analysis of proposed hypotheses to explain the presence of muscle cells in nerve trunks.     

Cervical myelopathy complicating cerebral angiography. Report of a case and review of the literature

Transverse cervical myelopathy, at C-6 level, followed injection of Renografin-60 into the right thyrocervical trunk during cerebral angiography. Review of the literature yielded only two cases in which attempted posterior fossa angiography resulted in cervical myelopathy. Two more cases were found. In one, cervical myelopathy occurred during aortography in a patient with coractation of the aorta, and in the other it followed mediastinal angiography. Summation of anoxia, hemorrhage, and cellular toxicity is responsible for spinal cord necrosis following arterial injection of contrast material.     

The syndrome of myoclonic epilepsy with ragged-red fibers. Report of a case and review of the literature

A patient with intention and action myoclonus, epilepsy, ataxia, and mental deterioration in association with ragged-red fibers in striated muscle is described. This patient demonstrated a unique form of erratic myoclonus with continuous EEG spike activity during eye closure. Both the myoclonus and the EEG spiking disappeared on opening the eyes. A defect in the activity of complex I in the respiratory chain was demonstrated.     

Spinal subdural hematoma following cranial surgery: a case report and review of the literature

Spinal subdural hematoma (SDH) following a cranial surgery is extremely rare. We described a 26-year-old patient who developed an SDH at L3-S1 level after the excision of intraventricular meningioma. He was surgically treated with an excellent outcome. It is postulated that the SDH resulted from downward migration of intracranial hematoma.     

Developmental Gerstmann syndrome associated with cerebellar neoplasm. Report of a case and review of the literature]

A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.     

Multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a case and review of the literature

A 31/2-year-old girl presented with frequent falls. She had an unsteady gait, delayed behavioural development absent tendon reflexes and in the legs decreased strength tone and equivocal plantar responses. She then developed ataxia, nystagmus, choreoathetosis, cranial nerve palsies, diminished strength and tone in the arms, sensory deficit in the limbs and autonomic nervous system dysfunction. She became progressively less responsive and succumbed at the age of 63/4 years. Examination of the central, peripheral and autonomic nervous system showed ubiquitous neuronal intranuclear hyaline inclusions and neuronal loss in several sites.     

Spontaneous cranial extradural hematoma: case report and review of literature

Extradural hematoma (EDH) is usually a post‐traumatic sequel but a few cases of spontaneous EDH have been reported. Here we report a woman who presented with spontaneous acute EDH but was later found to have dural metastasis from lung carcinoma. Causal factors have been present in all reported cases, as well as in this case. We propose the term non‐traumatic EDH.     

Hydrocephalic dementia and spinal cord tumor. Report of a case and review of the literature

A 68-year-old woman presented with an 18-month history of low back pain followed by leg weakness, dementia, and incontinence. Myelography revealed an intradural, extramedullary block from L-2 to L-4, and cranial computed tomography demonstrated ventriculomegaly. Excision of a benign schwannoma resulted in rapid relief of back pain and more gradual normalization of mental function and hydrocephalus. Five similar cases of dementia and hydrocephalus complicating spinal cord tumor have been reported. A variety of mechanisms have been proposed to explain the association but the pathophysiology is still unclear. Spinal tumor should be considered in the differential diagnosis of dementia and of communicating hydrocephalus.