patient does have significant functional abilities, demonstrating
a remarkable discrepancy between her anatomy and clinical
outcome. Our patient could ambulate with cane assistance and
was able to hold short conversations with reasonable short-term
memory despite having very minimal cerebral cortex for motor
function and language processing. These findings are a significant
demonstration of the role of neuroplasticity in preservation
of cognitive and neurological function during infancy. It may
also be suggestive of the redundancy present in the cerebral
cortex, or an increased role of subcortical structures in higher
Another possible explanation for the degree of
functional preservation is that hydrocephalus is a disease of
white matter, but grey matter is relatively spared even in severe
Nevertheless, the patient’s lack of anatomical
improvement following VP shunt insertion sheds light on the
therapeutic challenges in treating LOVA as the brain loses
compliance with such a degree of hydrocephalus. Such a degree
of craniocerebral disproportion increases the risk of subdural
hematoma and low-ICP syndrome following shunt insertion.
This case is reflects the various complexities in cognitive
reserve. Despite the notable adaptive mechanisms of the brain seen
in our patient’s lifelong hydrocephalus, the onset of delirium also
attests to the neurochemical vulnerability of her brain from her
underlying cognitive impairment. As a consequence, identifying
and addressing risk factors for delirium will be paramount in
providing future medical care to improve clinical outcomes.
This case report has never been published and has not been
submitted to any other journal for consideration. Our affiliations
lie with the Schulich School of Medicine & Dentistry and Windsor
Regional Hospital. Furthermore consent was obtained from
the patient in question to share their case in a case report for
academic and educational purposes.
1. American Psychiatric Association. Diagnostic and Statistical Manual of
Mental Disorders. 5th ed. Arlington, VA: American Psychiatric Publishing;
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4. Bellelli G, Morandi A, Davis DHJ, et al. Validation of the 4AT, a new
instrument for rapid delirium screening: a study in 234 hospitalised older
people. Age Ageing 2014;43(4):496–502. doi:10.1093/ageing/afu021.
5. Monette J, Galbaud Du Fort G, Fung SH, et al. Evaluation of the confusion
assessment method (CAM) as a screening tool for delirium in the
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developed for the inpatient setting, including the 4 “A’s” Test
and the confusion assessment method (CAM).
In this case
report, we presented a patient who developed a multi-factorial
cause of delirium.
Changes in cognitive function from baseline should prompt
a full differential diagnosis. Following the DIMS-R framework
may aid in the workup, since delirium is a clinical diagnosis that
is generally not attributed to a single pathology.
should be considered as part of the delirium workup to rule out
structural changes. Treatment of delirium is only effective when
all underlying causes are identified and addressed. Our patient
had multiple possible causes of delirium. Metabolically, she
presented with marked hypernatremia and hyperglycemia, which
are both classic precipitants of delirium. Structurally, the patient’s
profound hydrocephalus may have also contributed, considering
the clinical improvement she experienced following VP shunt
insertion. The key feature of her presentation which prompted
further workup of the patient’s disturbance in cognition is that
she developed unexplained visual hallucinations despite the
correction of her metabolic abnormalities and initial resolution
of her confusion. In the absence of infectious symptoms, this
suggested possible underlying cerebral pathology.
Hallucinations can be the product of one or more of the
following processes: organic brain disease, neurochemical
changes, or psychodynamic forces. While visual hallucinations
are a classical feature of psychosis, they are the most common
psychotic manifestations of delirium, and are more likely to be
associated with multiple medical disorders.
medical causes of visual hallucinations include dementia,
migraines, and seizure activity. Vision impairment can also cause
visual hallucinations, such as in Charles Bonnet syndrome and
The clinical presentation of hydrocephalus and other structural
causes of delirium is highly variable and is related to the degree
and nature of anatomical deformity present. The patient in this
case was found to have severe ventriculomegaly consistent with
Long-standing Overt Ventriculomegaly (LOVA), which was first
described by Oi and his colleagues. Patients with LOVA have
infantile hydrocephalus secondary to congenital aqueductal
stenosis that slowly progresses into adulthood. In congenital
hydrocephalus, the duration of the injury is another factor that
determines the presentation of symptoms. LOVA presents as
a triad of headaches, subnormal IQ, and macrocephaly. Other
features such as gait disturbances, urinary incontinence, and
severe depression may be present as well.
In LOVA, there is marked progressive dilatation of ventricles
and significant loss of cerebral parenchyma. This was seen in this
patient, such that there was “virtually no brain.” Interestingly, this
Canadian Journal of General Internal Medicine
38 Volume 13, Issue 4, 2018
An Unexpected Precipitant of Delirium in a Patient with Developmental Delay