Headache Medicine 2020, 11(3):68-71 ISSN 2178-7468, e-ISSN 2763-6178

ASAA

DOI: 10.48208/HeadacheMed.2020.19

Headache Medicine

Case Report

Pseudotumor cerebri secondary to hypoparathyroidism and

association to Fahr's Syndrome: a case report

Patrick Giordanni Gomes Sampaio

, Hiago Diniz Maracajá

, Francielle Lopes de Araújo Batista

Virgínia Gabriela Nóbrega Figueiredo

, Nátalia Meg Adijuto de Melo

Neuroconance, Neurologia, Campina Grande, Paraíba, Brazil

Unifacisa, Medicina, Campina Grande, Paraíba, Brazil

Abstract

The authors present the case of a patient who presented an initial headache attack and seizure, who

developed intracranial hypertension secondary to hypoparathyroidism. Her complementary clinical

investigation was compatible with Fahr's syndrome.

Patrick Giordanni Gomes Sampaio

giordannipatrick@gmail.com

Edited by

Mario Fernando Prieto Peres

Received: September 20, 2020

Accepted: September 30, 2020

Keywords:

Pseudotumor Cerebri

Intracranial Hypertension

Hypoparathyroidism

Headache Disorders

Secondary

ASAA

Sampaio PGG, Maracajá HD, Batista FLA, Figueiredo, VGN, Melo NMA

Pseudotumor cerebri secondary to secondary hypoparathyroidism and association to Fahr´s yndrome: a case report

Introduction

eadaches are extremely common complications in emer-

gency departments, accounting for up to 16% of visits,

according to some studies

and are observed in almost all pa-

tients with idiopathic intracranial hypertension (IIH), which is

characterized by signs and symptoms that include, in addition

to headache, nausea, vomiting, diplopia, decreased visual

acuity, pulsatile tinnitus, among other possible manifestations.

However, the clinical presentation is highly variable, which

can delay diagnosis.

Idiopathic intracranial hypertension is predominantly found

in obese women and of childbearing age, with a worldwide

incidence of around 12-20 cases per 100,000 people in

this most affected group, but only 0.5-2 cases per 100,000

people in the general population.

Systemic disorders and some drugs have been associated

with increased intracranial pressure, such as thyroid disorders,

hyper- or hypothyroidism

and hypervitaminosis A, as well as

isotretinoin and tetracyclines, being in this occasion dened

as a condition of secondary intracranial hypertension.

Regarding Fahr's disease, a rare neurological disorder with

a prevalence of 1/1.000.000, it is known that it is character-

ized by abnormal deposition of calcium, of idiopathic origin,

in brain areas that control movement.

Below, we present the case of a patient who presented an

initial headache attack and seizure, who developed intra-

cranial hypertension secondary to hypoparathyroidism. Her

complementary clinical investigation was compatible with

Fahr's syndrome.

Case report

A 33-year-old woman, BMI of 40.17 kg/m², with grade 3

obesity, Caucasian, single, with Down syndrome. Presents a

history of ostoperatory hypothyroidism, whose thyroidectomy

was performed 10 years ago, due to nodular goiter. This

patient presented subacute nausea, visual turbidity, diarrhea

and pulsatile holocranial headache, which worsened in the

horizontal position and presented slight relief in the orthostat-

ic position. This clinical picture lasted for about three days

and evolved with complaints of loss of visual acuity and the

occurrence of two generalized tonic-clonic seizures, which

led to the emergency care in her city. At the time, the patient

was referred to a tertiary service for specialized evaluation.

Upon neurological examination, the patient presented on

the campimetry of confrontation, loss of peripheral visual

eld and preservation of the central eld, and visual acuity

of 20/200 in the right eye - measured by the Rosenbaum

card - and counted ngers on the left eye. In the fundoscopy,

bilateral papilledema was noticed. In the other neurological

systems, no abnormalities were detected. This patient was then

submitted to complementary exams, where hypocalcemia and

hypomagnesemia were noted, thus being compatible with

secondary, postoperatory hypoparathyroidism. On computer-

ized tomography examination of the skull, calcications were

identied in the basal ganglia, bilaterally, and these ndings

are characteristic of Fahr's syndrome. Due to the clinical nd-

ing of visual changes and headache, the patient underwent

lumbar puncture with spinal manometry; it is important to note

that she had no contraindications to the procedure. Following

are the exam data: opening pressure (OP) = 84 cm H

O and

closing pressure (CP) = 15 cm H

O. Proteins, glycorrhachia

and lactate without changes. No evidence of pleocytosis.

With these ndings, the proposed diagnostic hypothesis was

intracranial hypertension secondary to hypoparathyroidism,

presenting visual impairment and symptomatic convulsive

crisis, secondary to hypocalcemia.

The patient was submitted to the correction of metabolism

disorders, with replacement of calcium, phosphate and mag-

nesium, and additional tests were requested to rule out other

clinical conditions that promote intracranial hypertension.

Five days after these measurements, the patient was again

submitted to a lumbar puncture with spinal manometry, for

control, being as follows: OP = 54 cm H

O and CP = 12

cm H

O. Thus, lumboperitoneal shunt was considered, due

to the refractoriness of intracranial hypertension, visual loss

and side effects of medications (topiramate, acetozolamide

and furosemide) in high doses in an attempt to control the

production of cerebrospinal uid (CSF) - that is, to reduce the

elevation of intracranial pressure. After these measures, the

patient started clinical treatment directed at obesity and the

surgical procedure was scheduled, which happened without

complications. The patient presented a satisfactory clinical re-

sponse, controlling for metabolic disorders with endocrinology

and there was adequate control of intracranial pressure. Even

so, the patient presented with loss of peripheral visual eld

as a sequel, but much less when compared to the admission

examination.

ASAA

Sampaio PGG, Maracajá HD, Batista FLA, Figueiredo, VGN, Melo NMA

Pseudotumor cerebri secondary to secondary hypoparathyroidism and association to Fahr´s yndrome: a case report

Discussion

Headaches in a general context are classied into primary

and secondary. Although primary headaches are the most

common, there are other serious and potentially fatal condi-

tions in 5-15% of cases, these being secondary. Therefore, the

priority is to establish an accurate diagnosis and distinguish

between them which can be benign and severe situations

(subarachnoid hemorrhage, meningitis, intracranial hyper-

tension).

In IIH, headache is progressively more severe and

frequent.

In addition, it has a pulsatile characteristic, which

may be associated with photophobia and phonophobia, in

addition to worsening with decubitus and Valsalva maneuver.

Thus, according to the consensus of IHH

, the diagnostic

criteria are: papilledema, normal neurological examination

(except for VI cranial nerve palsy), imaging tests without struc-

tural lesions (without hydrocephalus, mass, structural lesion

or meningeal enhancement, cerebral and cervical venous

thrombosis). In the study of CSF, the biochemical assessment

and serology for fungi and tuberculosis are innocent. How-

ever, OP is ≥ 25 cm H

O. For cases in which papilledema

does not occur, the presence of high OP and another clinical

alteration are necessary, be it paralysis of the VI cranial nerve

(unilaterally or bilaterally) or alteration of the neuroimaging.

Assessing the case described here, we observed that the pa-

tient also meets diagnostic criteria for IIH, but with a known

underlying clinical cause-metabolic dysfunction-being thus

dened as a case of secondary intracranial hypertension.

Some patients with IIH complain of persistent headache after

normalization of CSF pressure, so it is necessary to evalu-

ate the possibilities of comorbidities, such as an underlying

primary headache.

In turn, it is possible to attribute the

appearance of this clinical condition to the hypofunction of

parathyroid glands, the latter resulting, in the vast majority,

from a surgical procedure, in the others, it arises due to ge-

netic, autoimmune or idiopathic etiologies.

7,8

As for hypothyroidism, some reports address patients who

met the criteria for the pseudotumor cerebri associated with

hypothyroidism. It was then assumed that hypothyroidism

primarily caused intracranial hypertension (IH) and with

the replacement of the thyroid hormone, without any other

measures to treat the pseudotumor, the condition would nor-

malize. However, the relationship between normalization of

thyroid function and improvement in neurological status was

not observed.

1,6,9-10

In addition, in another case study there

was a report of a female patient, with clinical hypothyroidism,

where persistent elevated CSF pressures were observed for

four months, even with the normalization of thyroid status.

Therefore, it is more consistent evidence with the assumption

that the two conditions have no causal relationship, with

obesity exacerbated by thyroid hormone deciency having

more importance, as a precipitating factor for pseudotumor.

It is likely that occasional reports of simultaneous hypothyroid-

ism and cerebri pseudotumor reect the incidence of these

diseases in a young female population.

Regarding obesity, it is one of the clinical contexts most often

associated with the development of pseudotumor cerebri,

affecting about 80% of patients, showing itself to be funda-

mental in the pathophysiology of this disorder.

Another important point is that hypocalcemia is a disorder

present, mostly, due to surgical hypoparathyroidism. The

rapid onset of hypocalcemia in the post-surgical setting can

be acute, a fact that requires immediate and aggressive

intervention. However, patients can evolve with chronic hy-

poparathyroidism, and are almost asymptomatic, even when

there is loss of metabolic compensation to trigger symptomatic

hypocalcemia. Despite this reality, patients with cerebral

calcications in the basal ganglia may evolve with IH, when

this condition is attributed to calcium metabolism.

So, in this context, Fahr's syndrome is dened by a triad:

association of symmetrical calcications of the central gray

nuclei, neuropsychiatric symptoms and hypofunction of the

parathyroid gland. In addition, hypocalcaemia caused by

hypoparathyroidism explains most of the clinical signs, which

may possibly arise as a result of this deciency, such as: cata-

racts, calcium malabsorption, neuromuscular hyperexcitability,

neurological and neuropsychic signs, psychiatric disorders

that can even lead to psychosis, in addition to several car-

diovascular disorders.

Conclusion

With all these ndings, and ruling out the possible differential

diagnoses, the diagnosis of pseudotumor cerebri secondary

to hypoparathyroidism due to total thyroidectomy, with a

radiological picture of Fahr's Syndrome, was closed.

Given the above, it appears that hypothyroidism itself had no

direct cause relationship with the development of the patient's

IH, which is a condition resulting from hypoparathyroidism,

which generated a metabolic dysfunction, reducing serum

calcium levels, and also calcications of the basal ganglia

bilaterally. This glandular dysfunction of parathyroid glands

was also responsible for neurological symptoms, resulting

in intracranial hypertension, manifested by progressive and

ASAA

Sampaio PGG, Maracajá HD, Batista FLA, Figueiredo, VGN, Melo NMA

Pseudotumor cerebri secondary to secondary hypoparathyroidism and association to Fahr´s yndrome: a case report

pulsatile holocranial headache, accompanied by nausea

and visual changes.

The conduct of Fahr's syndrome, in this case, was focused on

the treatment of hypoparathyroidism and which consists of

correcting metabolism disorders of phosphorus and calcium,

which naturally resulted in clinical and radiological improve-

ment, however, without denitive resolution of the condition.

Conflicts of Interest: The authors declare no conicts of interest.

Author's contributions: PGGS- Data Collection, Project Management,

Research, Writing; HDM-Project Management, Writing, Reviewing

and Editing; FLAB- Writing, Proofreading and Editing; VGNF- Wri-

ting; NMAM- Writing

Hiago Diniz Maracajá

https://orcid.org/0000-0002-5032-3436

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