Hydrocephalus - demonstrační kasuistika

V nové verzi portálové platformy bude nabídnuta možnost publikace nového typu příspěvku, který bude označen jako kasuistika. Kasuistiky budou odděleny od běžných multimediálních učebních pomůcek. Tělo každé kasuistiky, tj. každého takového příspěvku bude mít obvyklou kasuistikovou strukturu (Vyšetření, Anamnéza, Diagnóza, Diferenciální diagnóza, Laboratorní výsledky, Histologie, Zobrazovací metody, Terapie, Komentář), přičemž bude možné tuto výchozí strukturu modifikovat k potřebám daného autora či zpracovávané problematiky.

Věk: 60 let
Pohlaví: muž

Anamnéza:

Normal pressure hydrocephalus (NPH) describes a condition that rarely occurs in patients younger than 60 years.2 Enlarged ventricles and normal CSF pressure at lumbar puncture (LP) in the absence of papilledema led to the term NPH. However, intermittent intracranial hypertension has been noted during monitoring of patients in whom NPH is suspected, usually at night. The classic Hakim triad of symptoms includes gait apraxia, incontinence, and dementia. Headache is not a typical symptom in NPH.

Zobrazovací metody:

CT/MRI criteria for acute hydrocephalus include the following:

• Size of both temporal horns is greater than 2 mm, clearly visible. In the absence of hydrocephalus, the temporal horns should be barely visible.
• Ratio of the largest width of the frontal horns to maximal biparietal diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.
• Transependymal exudate is translated on images as periventricular hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluid-attenuated inversion recovery [FLAIR] sequences).
• Ballooning of frontal horns of lateral ventricles and third ventricle (ie, "Mickey mouse" ventricles) may indicate aqueductal obstruction.
• Upward bowing of the corpus callosum on sagittal MRI suggests acute hydrocephalus.

Diagnóza:

Diferenciální diagnóza:

Brainstem syndromes
Macrocephaly
Hydranencephaly
Chronic subdural hemorrhages
Cerebral atrophy
Cerebral tumors
Periaqueductal glioma
Agenesis of corpus callosum
Septo-optic dysplasia
Neuroimaging of vascular malformations and hematomas of the brain

Histologie:

• Thinning and stretching of the cortical mantle may be seen as a result of ventricular dilation.
• In the acute phase, edema of the periventricular white matter is observed. Relatively few neuronal lesions are present. Ventricular ependyma shows cellular flattening and loss of cilia.
• At a later stage, the edema disappears and is replaced by fibrosis, axonal degeneration, demyelination, focal loss of cerebral cortical neurons, cellular flattening, and further loss of cilia.

Terapie:

Acetazolamide (ACZ) and furosemide (FUR) treat posthemorrhagic hydrocephalus in neonates. Both are diuretics that also appear to decrease secretion of CSF at the level of the choroid plexus. ACZ can be used alone or in conjunction with FUR. The combination enhances efficacy of ACZ in decreasing CSF secretion by the choroid plexus. If ACZ is used alone, it appears to lower risk of nephrocalcinosis significantly.

Diskuze/komentář:

Medication as treatment for hydrocephalus is controversial. It should be used only as a temporary measure for posthemorrhagic hydrocephalus in neonates.