Hydrocephalus Resulting from Late-Onset Aqueductal Membranous Occlusion: A Case Report and Review of the Literature

Terada, Yukinori; Yamamoto, Masaya; Motoie, Ryota; Matsui, Yuya; Katsuki, Takahisa; Mori, Nobuhiro; Hashimoto, Kenji

doi:10.1016/j.wneu.2020.02.027

Cited by 8 publications

(16 citation statements)

References 11 publications

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“…Despite obstruction/stenosis of the mesencephalic aqueduct in patients with LAMO hydrocephalus, hydrocephalus development cannot be explained by the classical concept. Namely, the classical concept cannot clarify why patients with aqueduct stenosis/obstruction often need surgery only later in life, as was the case in our Patient 3, although stenosis/obstruction had most likely been present for a long time, even before birth in some cases ( 26 - 28 ). Furthermore, these patients, despite the increase in CSF volume in the lateral ventricles and third ventricle, did not have an increased intraventricular pressure (Patient 3), as would be expected according to the classical concept.…”

Section: Discussionmentioning

confidence: 78%

The role of mesencephalic aqueduct obstruction in hydrocephalus development: a case report

Radoš¹,

Orešković²,

Klarica³

2021

Croat Med J

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We report on three patients with mesencephalic aqueduct obstruction, which completely blocked the cerebrospinal fluid communication between the third and fourth cerebral ventricle, demonstrated by standard and high-resolution magnetic resonance sequences. Only one patient developed radiological and clinical presentation of hydrocephalus, without radiological signs of increased intraventricular pressure. The remaining two patients did not show clinical signs of hydrocephalus and had a normal radiological presentation of the ventricular system. These findings contradict the classical concept of cerebrospinal fluid physiology. This concept assumes a unidirectional circulation of cerebrospinal fluid through the mesencephalic aqueduct from the secretion site, predominantly in the choroid plexuses, to the resorption site, predominantly in the dural venous sinuses. Therefore, the obstruction of the mesencephalic aqueduct would inevitably lead to triventricular hypertensive hydrocephalus in all patients. The current observations, however, accord with the new concept of cerebrospinal fluid physiology, which postulates that cerebrospinal fluid does not circulate unidirectionally because it is both formed and resorbed along the entire capillary network within the central nervous system.

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Section: Discussionmentioning

confidence: 78%

The role of mesencephalic aqueduct obstruction in hydrocephalus development: a case report

Radoš¹,

Orešković²,

Klarica³

2021

Croat Med J

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“…Matsuda et al first described LAMO in 2011. 2 Terada et al defined LAMO as follows 3 : first, the aqueduct of Sylvius showed a membranous structure but no CSF flow; second, no other occlusive lesion and no recent subarachnoid hemorrhage, intraventricular hemorrhage, or meningitis was found that could cause the hydrocephalus; third, the lateral and third ventricles were enlarged, but the fourth ventricle was not; and fourth, the patient was not diagnosed in childhood. No membranous structure was found in the aqueduct of Sylvius in our case, but it was a similar case to LAMO.…”

Section: Discussionmentioning

confidence: 99%

“… 1 Hydrocephalus has been reported in 2 to 23% of NF1 patients, and NF1 patients rarely present with hydrocephalus caused by late-onset aqueductal membranous occlusion (LAMO). 2 3 …”

Section: Introductionmentioning

confidence: 99%

Hydrocephalus in Neurofibromatosis Type 1 Caused by a Cyst Formation Similar to Late-Onset Aqueductal Membranous Occlusion: A Case Report and Review of Literature

et al. 2022

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Cyst formation in the third ventricle and the histopathological findings were rarely reported. We report a similar case of late-onset aqueductal membranous occlusion (LAMO) caused by a thin gliotic cyst and a review of related literature. A 28-year-old woman with enlarged lateral ventricles was referred to our hospital with complaints of headache and dizziness. In our hospital, the obvious cause of the hydrocephalus was unknown on any examination and we decided performing endoscopic third ventriculostomy for hydrocephalus. A thin cyst covering the entrance of the aqueduct was identified and we perforated it. Histopathological finding of the cyst wall was gliosis and our case was similar to LAMO, although not typical. The postoperative symptoms and ventricle size improved for 4 years. When suspecting cases similar to definition of LAMO, neuroendoscopic surgery would be the first-choice treatment and might detect causes undetectable on preoperative imaging such as our thin membrane.

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“…Japanese guidelines ( 2 ) differentiate iNPH from the various types of late-onset congenital hydrocephalus, such as panventriculomegaly with a wide foramen of Magendie and a large cisterna magna (PaVM) ( 7 ), longstanding overt ventriculomegaly in adults (LOVA) ( 8 ), late-onset idiopathic aqueductal stenosis (LIAS) ( 9 - 11 ), and late-onset aqueductal membranous occlusion (LAMO) ( 12 ) demonstrating advanced ventricular enlargement. The international iNPH guidelines do not require DESH findings for diagnosis but instead incorporate these disease groups within iNPH and define a much younger age of 40 years or older for initial onset ( 13 ).…”

Section: Classification Of Normal Pressure Hydrocephalusmentioning

confidence: 99%

Differentiating comorbidities and predicting prognosis in idiopathic normal pressure hydrocephalus using cerebrospinal fluid biomarkers

Nakajima

Kawamura²,

Akiba³

et al. 2021

Croat Med J

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Idiopathic normal pressure hydrocephalus (iNPH) is a condition resulting from impaired cerebrospinal fluid (CSF) absorption and excretion characterized by a triad of symptoms comprising dementia, gait disturbance (impaired trunk balance), and urinary incontinence. CSF biomarkers not only assist in diagnosis but are also important for analyzing the pathology and understanding appropriate treatment indications. As the neuropathological findings characteristic of iNPH have yet to be defined, there remains no method to diagnose iNPH with 100% sensitivity and specificity. Neurotoxic proteins are assumed to be involved in the neurological symptoms of iNPH, particularly the appearance of cognitive impairment. The symptoms of iNPH can be reversed by improving CSF turnover through shunting. However, early diagnosis is essential as once neurodegeneration has progressed, pathological changes become irreversible and symptom improvement is minimal, even after shunting. Combining a variety of diagnostic methods may lead to a more definitive diagnosis and accurate prediction of the prognosis following shunt treatment. Identifying comorbidities in iNPH using CSF biomarkers does not contraindicate shunting-based intervention, but does limit the improvement in symptoms it yields, and provides vital information for predicting post-treatment prognosis.

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Hydrocephalus Resulting from Late-Onset Aqueductal Membranous Occlusion: A Case Report and Review of the Literature

Cited by 8 publications

References 11 publications

The role of mesencephalic aqueduct obstruction in hydrocephalus development: a case report

The role of mesencephalic aqueduct obstruction in hydrocephalus development: a case report

Hydrocephalus in Neurofibromatosis Type 1 Caused by a Cyst Formation Similar to Late-Onset Aqueductal Membranous Occlusion: A Case Report and Review of Literature

Differentiating comorbidities and predicting prognosis in idiopathic normal pressure hydrocephalus using cerebrospinal fluid biomarkers

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