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Query: UMLS:C0023380 (lethargy)
 5,697 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Supratentorial subdural effusion is common after infection and trauma, but rarely occurs in the posterior fossa, and is even less commonly unilateral. The authors report a rare case of unilateral traumatic posterior fossa subdural effusion with secondary hydrocephalus. A 6-month-old female infant presented with lethargy, poor appetite, and persistent vomiting after head trauma 2 weeks previously. A non-enhanced brain CT scan revealed a right posterior fossa subdural fluid collection that displaced the fourth ventricle and brainstem and dilated lateral ventricles. While monitoring the intracranial pressure, the baby was treated with temporary external subdural drainage, followed by a permanent subdural peritoneal shunt. The treatment and likely physiopathology of this unique case are discussed.
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PMID:Unilateral traumatic posterior fossa subdural effusion in an infant. 1641 Feb 14

The authors document a rapid development, within 3 weeks, of hydromyelia in a 12 year-old boy. The boy was admitted to a local hospital because of drowsiness and persistent severe neck pain. Neurological examination disclosed a lethargic boy with no neurological deficit other than Parinaud's sign. During his transfer to our department, he presented a cardio-respiratory arrest with coma and bilateral mydriasis. External ventricular drain and craniocervical decompression achieved excellent clinical and neuroradiological outcomes. The development of hydromyelia in this case is caused by obstruction to the natural cerebrospinal fluid pathway at the craniocervical junction and the cardio-respiratory arrest is provoked by a brain stem compression against the clivus and odontoid process. This report illustrates that hydromyelia may complicate acute obstructive hydrocephalus due to acquired Chiari malformation.
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PMID:Acute presentation of hydromyelia in a child. 1694 53

Neurocutaneous melanosis (NM) coexisting with the Dandy-Walker complex (DWC) is a rare condition, with fewer than 15 cases reported in the literature. The authors present a case of an infant with NM and DWC suffering from progressive brainstem compression following ventriculoperitoneal (VP) shunt placement for hydrocephalus. This 1-year-old boy with congenital melanocytic nevi had met normal developmental milestones until the age of 11 months, when he began regressing in ambulation and language function. Intractable vomiting had developed 1 week later. Magnetic resonance (MR) imaging of the brain revealed DWC with hydrocephalus, and spinal MR images demonstrated a proliferative process within the meninges, consistent with NM. The patient underwent right frontal VP shunt placement resulting in immediate symptom relief, but 3 weeks later became irritable, increasingly lethargic, unable to pull to stand, and unable to tolerate solid food without choking. Due to these symptoms and intractable vomiting, the patient presented to the authors' institution. Brain MR imaging revealed a new-onset diffuse cystic process with anterior and posterior brainstem compression, marked kinking of the cervicomedullary junction, melanocyte pigmentation of the left temporal lobe, diffuse leptomeningeal enhancement, and no evidence of hydrocephalus. Consistent with these imaging findings, the degree of brainstem involvement upon gross visualization predictably deterred resection attempts beyond those necessary for biopsy. Pathological examination revealed diffuse melanocytosis, and the family decided not to pursue aggressive measures postoperatively. This report indicates the potential for rapid intracranial manifestation of diffuse melanocytosis in NM patients. Although the prognosis is poor, early neurosurgical involvement in these patients may provide tissue diagnosis and the potential for decompression if the process is caught early in its course.
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PMID:Progressive brainstem compression in an infant with neurocutaneous melanosis and Dandy-Walker complex following ventriculoperitoneal shunt placement for hydrocephalus. Case report. 1815 21

We report a previously healthy two-year-old girl who initially presented with signs of increased intracranial pressure of vomiting, lethargy and unstable gait. She had communicating hydrocephalus and a ventriculoperitoneal shunt was placed. Two years later the girl developed signs of myelopathy and was diagnosed with a spinal cord tumour between Th3 and Th9. We suggest that spinal cord tumour should be considered in patients with increased intracranial pressure or hydrocephalus of unknown origin.
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PMID:[Hydrocephalus as initial presentation of a spinal cord tumour in a child]. 1880 55

Intracerebral hemorrhage is by far the most destructive form of stroke. The clinical presentation is characterized by a rapidly deteriorating neurological exam coupled with signs and symptoms of elevated intracranial pressure. The diagnosis is easily established by the use of computed tomography or magnetic resonance imaging. Ventilatory support, blood pressure control, reversal of any preexisting coagulopathy, intracranial pressure monitoring, osmotherapy, fever control, seizure prophylaxis, treatment of hyerglycemia, and nutritional supplementation are the cornerstones of supportive care in the intensive care unit. Dexamethasone and other glucocorticoids should be avoided. Ventricular drainage should be performed urgently in all stuporous or comatose patients with intraventricular blood and acute hydrocephalus. Emergent surgical evacuation or hemicraniectomy should be considered for patients with large (>3 cm) cerebellar hemorrhages, and in those with large lobar hemorrhages, significant mass effect, and a deteriorating neurological exam. Apart from management in a specialized stroke or neurological intensive care unit, no specific medical therapies have been shown to consistently improve outcome after intracerebral hemorrhage.
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PMID:Clinical review: Critical care management of spontaneous intracerebral hemorrhage. 1910 4

In this report, the authors describe a unique presentation of ventriculomegaly in the setting of diabetic ketoacidosis (DKA). A 15-year-old male, with a history of shunt placement for hydrocephalus and repair of a myelomeningocele, presented to the emergency room with DKA and was found to have ventriculomegaly. At the time of presentation, the patient had a 24- to 48-hour history of lethargy, nausea, vomiting, fever (102.2 degrees F), and polydipsia. A computed tomographic (CT) scan of the head indicated increased ventricular size compared with previous studies, thus prompting a neurosurgical evaluation. Blood glucose level at admission was found to be 1,551 mg/dl, bicarbonate level was 9 mmol/l, and pH was 7.08. The patient was treated for DKA; his fever, lethargy, nausea, and vomiting subsequently resolved. A repeat CT scan obtained 24 h after admission revealed a return of the ventricular system to its baseline size. On the basis of radiographic and laboratory evaluations, the authors hypothesize that the transient ventriculomegaly was directly related to extreme hyperglycemia.
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PMID:Transient ventriculomegaly in an adolescent presenting with shunted hydrocephalus, diabetic ketoacidosis, and hyperglycemia. 1912 93

Nonketotic hyperglycinemia is an autosomal recessive disorder of glycine metabolism. Patients generally present in the neonatal period with lethargy, feeding difficulty, hypotonia, apnea, poorly controlled convulsions, and coma. Myoclonic seizures and burst suppression pattern on electroencephalography are major findings of disease, but development of hydrocephalus is not an expected finding. The present case is that of an infant with acquired hydrocephalus, psychomotor retardation, and myoclonic seizures in whom the final diagnosis was nonketotic hyperglycinemia.
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PMID:Nonketotic hyperglycinemia and acquired hydrocephalus. 1913 33

The authors present the case of an 11-week-old girl in whom hydrocephalus developed secondary to intermittent obstruction of the third ventricle by a choroid plexus cyst. The patient presented to the emergency department at the authors' institution with a 1-day history of projectile vomiting, lethargy, and dysconjugate gaze. Hydrocephalus was confirmed on head CT. During hospitalization, the symptoms resolved with a decrease in ventricular size. One week later, the patient again presented with similar symptoms, and MR images with 3D-constructive interference in steady state sequences revealed that a cyst was blocking the third ventricle. The patient subsequently underwent endoscopic fenestration of the cyst with resolution of hydrocephalus and symptoms. The authors present a unique description of the diagnosis of intermittent obstructive hydrocephalus caused by a third ventricular region choroid plexus cyst in an infant.
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PMID:Treatment of intermittent obstructive hydrocephalus secondary to a choroid plexus cyst. 1995 Oct 46

The author reports the details in 2 cases of infants with familial cerebral cavernomatosis who presented in dire condition from hemorrhagic posterior fossa cavernous malformations. In Case 1, a 4-month-old boy presented with opisthotonos, gaze palsy, and lethargy. Magnetic resonance imaging revealed a multilobulated cavernous malformation in the fourth ventricle with evidence of bleeding and obstructive hydrocephalus. In Case 2, a 7-month-old girl presented with lethargy, followed by rapid neurological decline. Imaging demonstrated a large lesion involving both the brainstem and cerebellum, with obstructive hydrocephalus. Both patients required immediate surgical intervention, and external ventricular drainage and posterior fossa craniotomies were performed. Both patients made excellent recoveries. These cases suggest that infants in families with suspected or confirmed familial cerebral cavernomatosis should be screened at an early age.
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PMID:Giant posterior fossa cavernous malformations in 2 infants with familial cerebral cavernomatosis: the case for early screening. 2080 59

Neonatal onset of carnitine palmitoyltransferase II (CPT II) deficiency is an autosomal recessive, often lethal disorder of the mitochondrial beta-oxidation of long-chain fatty acids. It is a rare multiorgan disease which includes hypoketotic hypoglycemia, severe hepatomuscular symptoms, cardiac abnormalities, seizures and lethargy, as well as dysmorphic features. Until now, only 22 affected families have been described in the literature. An increasing number of mutations are being identified in the CPT2 gene, with a distinct genotype-phenotype correlation in most cases. Herein we report a new case of neonatal CPT II deficiency associated with Dandy-Walker syndrome and sudden death at 13 days of life. CPT II deficiency was suggested by acylcarnitine analysis of dried-blood on filter paper in the expanded newborn screening. Genetic analysis of the CPT2 gene identified the presence of a previously described mutation in homozygosity (c.534_558del25bpinsT). All lethal neonatal CPT II deficiency patients previously described presented severe symptoms during the first week of life, although this was not the case in our patient, who remained stable and without apparent vital risk during the first 11 days of life. The introduction of tandem mass spectrometry to newborn screening has substantially improved our ability to detect metabolic diseases in the newborn period. This case illustrates the value of expanded newborn screening in a neonate with an unusual clinical presentation, combining hydrocephalus and sudden death, that might not commonly lead to the suspicion of an inborn error of metabolism.
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PMID:Neonatal carnitine palmitoyltransferase II deficiency associated with Dandy-Walker syndrome and sudden death. 2164 Dec 54


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