Chronic Cough as the Presenting Symptom of Hydrocephalus

Selmin Karatayli-Ozgursoy, MD; Jacob Dominik, MD; Benjamin Eidelman, MD; Juan C.
Guarderas, MD
Case Report
A 48-year-old female patient presented to the department of otorhinolaryngology with a one-year
history of chronic cough. Her primary physician had prescribed various courses of antibiotics
and cough medicines without benefit. The cough was present throughout the waking hours,
occasionally disrupted her sleep, and was particularly noticeable if she reclined after eating. The
patient was prescribed omeprazole as treatment for possible gastroesophageal reflux disease, but
the cough persisted. Finally, she was referred to the department of allergy-internal medicine for
evaluation and treatment.
The patient's history was negative with respect to allergy, sinus, and pulmonary conditions
except for allergic rhinoconjunctivitis occurring when exposed to cats. She denied dysphonia,
dysphagia, heartburn, reflux, and any pulmonary or cardiovascular symptoms. She was a lifetime
nonsmoker with a history of a 12-pound weight loss, attributed to participation in a
"detoxification program." Family history was noncontributory, as were other aspects of her past
medical and surgical history.
In addition to her persistent cough, the patient related that she had a sense of weakness and
numbness in her arms and legs. Her gait was compromised, and she complained of a sensation of
heaviness in the legs with a tendency to drag her feet. She had also noted slowness in her
response times during conversation.
Otolaryngological examination and heart and lung examinations were normal. Laboratory studies
were unremarkable for complete blood count, chemistry panel, serum iron, vitamin B12, folic
acid level, serum protein electrophoresis, antinuclear antibody and C-reactive protein tests,
paraneoplastic panel, myasthenia gravis serology, and limited specific IgE allergy blood tests.
Computed tomography (CT), chest x-rays, and pulmonary function tests were normal.
Neurological consultation was requested, and the specific neurological history was negative for
syncope, presyncope, alterations of consciousness, confusion, hallucinations, orthostasis,
headaches, visual problems, neck or back pain, and bowel or bladder problems. The neurological
examination revealed intact cognition, no papilledema, decreased distal sensation to pinprick in
the lower extremities and bilateral, mild proximal weakness with minimal exaggeration of stretch
reflexes. An apraxia of gait was noted with poor initiation, reduced foot clearance, and decreased
stride length. The patient's arm swing was reduced, and she had difficulty walking backwards
and sideways. The remainder of the neurological examination was normal.
Magnetic resonance imaging (MRI) of the brain (Figs. 1 and 2) demonstrated hydrocephalus
involving the lateral and third ventricles, a very small aqueduct, and a normal fourth ventricle.
Uniform, linear T2-weighted hyperintensities surrounding the lateral ventricular margins were
present, indicating the presence of transependymal cerebrospinal fluid (CSF) resorption. These
findings were highly suggestive of obstructive hydrocephalus. The patient underwent a lumbar
puncture and the opening CSF pressure was 182 mm H20. Analysis of the CSF revealed no
abnormality. Subsequent videotaping demonstrated some improvement in gait following
withdrawal of 30 mL of fluid. The patient was referred to neurosurgery for further evaluation. A
ventriculoperitoneal (VP) shunt was inserted and a scan on postoperative day 15 confirmed a
right-sided posterior parietal shunt, as well as a newly-decompressed ventricular system. The
patient's neurological symptoms improved considerably in the month following shunt placement,
particularly with respect to memory and gait. The cough also resolved immediately, eliminating
the need for cough medication.
The majority of the follow up was performed by the patient's local providers. Over the 11 months
following shunt implantation, the patient required multiple shunt adjustments. The Codman-
Hakim programmable shunt was adjusted multiple times from between 120 mm H20 to 200 mm
H20. This was done due to development of a right-sided subdural hygroma initially documented
at 7 mm thickness one month after implantation and reaching 13 mm thickness at two months
after implantation. Eventually, at nine months after shunt placement, the patient was stabilized at
a shunt setting of 120 mm H20. During follow up, the patient was documented to have
intermittent recurrence of the cough at two and seven months after shunt implantation.
Discussion
Excluding common causes of chronic cough should always be the first objective, although it may
require the collaboration of experts in many disciplines. In this case, clinical evaluation
uncovered signs indicating a neurological disorder, but the cough was determined to be of
neurogenic origin. This assessment was made only after exhaustive investigations failed to reveal
a systemic cause and, in particular, after a positive response to treatment of the hydrocephalus
was observed. Thus, it is important to review the central mechanisms of cough genesis with
particular reference to hydrocephalus as an etiological condition in neurogenic cough.
Coughing is typically thought of as a protective reflex triggered by several inflammatory or
mechanical changes, and/or inhalation of chemical and mechanical irritants to the airways.
Sensory nerve receptors responding to these stimuli are defined regarding their conductive
properties as rapidly adapting receptors, slowly adapting receptors, or C-fibre receptors. Afferent
nerve activation in the external auditory meatus, pharynx and esophagus can also initiate cough.
Afferent nerve fibers from cough receptors converge via the vagus nerve in the nucleus tractus
solitarius (NTS), and the NTS is connected to the neurons in the central respiratory generator
which coordinates the efferent cough response. In this model, the cough reflex is produced by a
convergent, but plastic, brainstem neural network.
Circumstantial evidence suggests that coughing in humans involves higher brain regulation over
the basic reflex network at the brainstem level. Coughing can be initiated voluntarily. Placebo-
induced suppression of cough, or cough suppression during sleep or under general anesthesia
might be related to the modulation of cortical control. An enhanced cough reflex has been
reported in central nervous system disorders, including cerebellar ataxia and motor neuron
disease. Chronic cough can also be a presenting symptom of a type-I Arnold-Chiari
malformation. Kramer et al described two cases of cough in Arnold-Chiari I; however, in these
cases, the cough precipitated other neurologic symptoms such as numbness, weakness, or
cervical pain, presumably via transiently-increased intracranial pressure. In these cases, it is not
clear whether the cough was a consequence of the Chiari or whether it resolved after surgical
decompression. In this patient, the MRI demonstrated mildly low-lying cerebellar tonsils and a
minimally-crowded posterior fossa. It is conceivable that this may have accounted for the
patient's chronic cough. The relief of hydrocephalus by shunting could have reduced downward
pressure on the posterior fossa, diminishing medullary compression and improving the cough.
However, our patient denied any neck pain or headaches, alone or associated with the cough,
which one might expect if this was indeed the mechanism. Also, lumbar puncture in this instance
might increase tonsillar displacement which should make the cough worse, but it did not. The
patient did have lumbar punctures performed prior to presentation at this institution. The
combination of obstructive hydrocephalus and a low-pressure state due to lumbar puncture could
have accounted for the mildly low-lying cerebellar tonsils. The cough, however, had been
present for almost one year before the lumbar punctures. That may suggest that the lumbar
punctures (LP) were the cause of the mild Chiari-like appearance, but this was not the
mechanism responsible for the chronic cough.
Neurologic disorders have also rarely been noted to present with chronic cough as the initial
symptom. Diffusion-weighted imaging (DWI) changes in the putamen, caudate, and surrounding
cortical areas led them to speculate whether these areas could have some role in the generation of
the chronic cough. In this patient, the hydrocephalus with enlargement of the lateral and third
ventricles affected periventricular subcortical motor fibers, causing a mild upper-motor-neuron
pattern, bilateral lower-extremity weakness, hyperreflexia, and gait apraxia. Local compression
of subcortical fibers and interruption of cortical-basal-ganglia connections by ventricular
expansion may be involved in the generation of our patient's chronic cough. Although
speculative, it is remarkable that previous patients with chronic cough as the presenting symptom
of neurologic disease demonstrate known pathology in the same subcortical regions. Additional
evidence suggesting a role for a cortically-based cough center arises out of reports of impairment
of voluntary cough responses in patients with strokes involving the frontoparietal region in
Parkinson disease, as well as multiple system atrophy.
On initial presentation, it can be difficult to differentiate primary aqueductal stenosis (AS) from
normal pressure hydrocephalus (NPH). Tisell compared the clinical presentations of AS and
NPH and found no difference in the presence of gait disturbances, cognitive function, or urinary
incontinence after adjusting for age. The only significant difference was the presence of
headache in the AS patients preoperatively, and greater improvement with surgery. On our initial
evaluation, it was considered that the patient's presentation was more consistent with NPH.
However, MRI studies revealed narrowing of the cerebral aqueduct and sparing of the fourth
ventricle, which is consistent with primary aqueductal stenosis.
Although LP is frequently used as a diagnostic aid in NPH to help determine a patient's likely
response to shunting, it is usually contraindicated in cases of obstructive hydrocephalus, in which
the trapped ventricular CSF can act like a space-occupying lesion. This patient had already
undergone a diagnostic LP at an outside institution and tolerated it well with some transient
questionable improvement. Given this fact, as well as the similarity of the clinical presentation to
that of NPH, we performed a high-volume LP with video. The patient showed significant
improvement in gait after the high-volume LP.
Traditionally, treatment options for AS provide a route for outflow of entrapped CSF performed
via a VP shunt. More recently, endoscopic third ventriculostomy (ETV) has become a preferred
option in many centers. After the neurosurgery consultation, our patient had a VP shunt. Some
anesthetic drugs are known to decrease cough reflex in patients undergoing surgery. Therefore,
one might consider that the anesthetic drugs administered at the time of shunt placement may
have had some impact on the cough. If so, this effect could have continued only until the end of
the half-life of these drugs. However, elimination, or at least significant improvement of the
cough, was demonstrated for almost one full year after shunt placement.
Ventricular dilatation in our patient might have caused loss or impairment of higher inhibitory
mechanisms of the cough reflex. It is of relevance to examine the possible mechanisms whereby
hydrocephalus may have induced chronic cough. In this patient, both the neurological symptoms
and cough significantly improved after the operation, indicating that compression rather than
irreversible pathological changes were operative in the development of the clinical condition.
Refference:
 http://www.medscape.com/viewarticle/725268_2

Aquino, Lesley Jane P.

BSN4-5A

SUMMARY:

Chronic cough is defined as a daily cough lasting for more than eight weeks. The
journal tackles about an unusual case of chronic cough as the primary manifestation of
obstructive hydrocephalus. Chronic cough in this case was determined to be of
neurogenic origin only after exhaustive investigations failed to reveal a systemic cause,
and, in particular, after a positive response to treatment of the hydrocephalus was
observed. To the best of their knowledge, this is the first report of hydrocephalus
presenting as chronic cough. They believe this case will remind physicians of the
importance of considering neurological disease as a cause of chronic cough after common
causes are excluded.
REACTION:
Patients with a chronic cough are frequently encountered in the daily practice of
internists, general practitioners, and otorhinolaryngologists. Chronic cough is defined as a
daily cough lasting for more than eight weeks. The medical literature lists many causes of
chronic cough. Common causes are those related to nasal and sinus disease, problems in
the throat, various pulmonary conditions, and diseases of the upper gastrointestinal tract,
especially gastroesophageal reflux disease. Only rarely can one find a report of
neurological disease as a cause of chronic cough.

In this journal, an unusual case of chronic cough as the primary manifestation of

obstructive hydrocephalus. This journal is very informative when it comes to the
improvement on the study of the other cause of hydrocephalus.

The relevance of this journal to the nursing education is that this provides
additional information on how chronic cough can be a cause of hydrocephalus and also
for the enhancement of knowledge of the nursing educator about the illness. To the
nursing practice, this will be of help in the planning and improvement of care in the
patient with chronic cough because nurses should not only focus the care in treating the
chronic cough, that the nurses should also focus the care on the underlying cause of it that
the chronic cough is another symptom of having a hydrocephalus. To the nursing
research, this journal will serve as another source on the updates about hydrocephalus and
will be a good title for further study. And to the Philippine setting, the relevance of this
journal is that this will be a good source of new information about hydrocephalus; that
chronic cough can also be now a symptom of having a hydrocephalus, also this will
enhance the knowledge of Filipino nurses and Filipino patients about the illness.

RECOMMENDATION:

My only recommendation is that this new updates about the other symptoms of
hydrocephalus will be further investigated and if by chance, this hypothesis is really true,
then all health care agencies will be informed about chronic cough as the new presenting
symptom of having a hydrocephalus so that all health care providers will plan a care not
only focuses on treating the chronic cough but also further the assessment that maybe the
patient is suffering from hydrocephalus.