Keywords
CIMDL - cocaine abuse - hydrocephalus - meningitis - skull base erosion
Introduction
Scotland ranks among the highest consumers of recreational drugs in Europe, with particularly
high usage of cocaine.[1] Cocaine is derived from the plant Erythroxylum coca, which was primarily used as a stimulant by South American communities in the 1800s.[2] In recent years, recreational misuse of cocaine has led to substantial socioeconomic
challenges and serious health-related issues.[3] One emerging condition associated with the insufflation of cocaine, which is becoming
increasingly common, is cocaine-induced midline destructive lesions (CIMDL).[4] The severity of osseocartilaginous destruction can be proportional to the chronicity
of cocaine use. The damage ranges from nasal mucosal loss to significant loss of midface
osseous support, which is noted to occur usually in a centrifugal manner.[5]
[6] However, chronic cocaine abuse leading to clival erosion, presenting as acute meningoencephalitis
with hydrocephalus, is a rare event.[6] We want to describe a patient who presented with acute hydrocephalus and meningitis
as a sequel to chronic cocaine abuse, highlighting significant morbidity and challenges
in the management of the same.
Illustrative Case
A 39-year-old woman presented with a 1-week history of fever and malaise. According
to her next of kin, she had developed a gait imbalance over the preceding 2 days.
Upon arrival at her local accident and emergency department, she exhibited altered
sensorium and a low Glasgow coma scale (GCS), necessitating emergency intubation.
Her preintubation GCS was E4V1M5, and she demonstrated left-sided hemiparesis.
Her medical history revealed chronic cocaine abuse via nasal insufflation. One year
prior, she was diagnosed by the ENT team with a posterior nasopharyngeal ulcer and
nasal septal perforation. A biopsy of the ulcer was benign, showing no evidence of
granulomatous inflammation. She had been under follow-up for these findings and was
advised to seek rehabilitation for her substance abuse. However, her compliance with
treatment and follow-up was suboptimal.
A computed tomography (CT) scan of her head revealed acute hydrocephalus with periventricular
seepage and brain edema ([Fig. 1]). Consequently, she was urgently referred to our tertiary care neurosurgery center
for advanced management. Laboratory investigations showed a markedly elevated white
blood cell (WBC) count of 31.4 × 109/L and a C-reactive protein level of 172 mg/L, indicating a high likelihood of an
infectious process. Blood viral markers, including HIV, were negative.
Fig. 1 CT scan of the head with contrast showing enhancing meninges, acute hydrocephalus,
dilatation of the temporal horns of lateral ventricle, dilatation of the aqueduct
at the posterior aspect of the midbrain, periventricular seepage, and effacement of
gyri and sulci suggesting brain edema. CT, computed tomography.
She was commenced on broad-spectrum antibiotics to cover for potential meningitis.
An emergency EVD was inserted as a temporary cerebrospinal fluid (CSF) diversion measure
to reduce intracranial pressure and facilitate a definitive diagnosis.
Her CSF was turbid appearing to the naked eye with a WBC of 307/cu.mm, polymorphs
of 75%, lymphocytes of 25%, glucose of 1.9 mmol/L, and protein of 2.8 g/L. The values
were in favor of acute bacterial meningitis, which was in line with her clinical deterioration.
Hence, broad-spectrum antibiotics were instituted. A more detailed evaluation of her
CT scan revealed that she had air in the sphenoid sinus, clival erosion, and partial
erosion of the anterior arch of C1 ([Figs. 2],[3],[4]). The attachment of the transverse atlantal ligament on C1 was intact, and there
was no atlantoaxial instability. All of these findings were indicative of CIMDL. Her
blood cultures grew methicillin-sensitive Staphylococcus aureus (MSSA). She tested negative for antineutrophil cytoplasmic antibody (ANCA), and we
did not test for blood levamisole levels. Transthoracic two-dimensional echocardiogram
revealed vegetations in the tricuspid valve, which is common among intravenous (IV)
drug abusers. She had a stormy course and a long-term intensive care unit stay. Her
subsequent interval CT scans showed a bilateral thalamic infarct and a right cerebellar
infarct ([Figs. 5] and [6]). The posterior circulation strokes were attributed to embolism from the cardiac
vegetations. Eventually, she required a ventriculoperitoneal shunt after completion
of an antibiotic course of 4 weeks, following which her CSF values were nonmeningitic.
She was discharged with a GCS of E4V5M6 and residual left hemiparesis (G3/5). She
is now being rehabilitated by our maxillofacial, otorhinolaryngology, and skull base
team for optimal multidisciplinary care.
Fig. 2 CT scan of the head (bone window)—midsagittal section demonstrating significant clival
erosion. CT, computed tomography.
Fig. 3 CT scan at the level of craniovertebral junction—showing lytic erosion of anterior
arch of C1, anterior to the attachment of transverse atlantal ligament. CT, computed
tomography.
Fig. 4 CT scan of the head (bone window)—coronal section at the level of sphenoid sinus
demonstrating erosion of floor of the sphenoid sinus and air in the sinus. CT, computed
tomography.
Fig. 5 CT scan of the head—bilateral thalamic infarcts, right side more than left, likely
because of posterior circulation embolic stroke. CT, computed tomography.
Fig. 6 CT scan of the head—right cerebellar infarct. CT, computed tomography.
Discussion
Cocaine is the second most commonly abused drug in Europe among young adults and the
middle age population according to the European drug report in 2023.[3] Nasal snorting of cocaine presents a wide spectrum of diseases manifesting from
nasal septum perforation most commonly to nasopharyngeal ulcers, midface osseocartilaginous
structures erosion, skull base destruction, central nervous system vasculitis, leukoencephalopathy,
and rarely meningitis.[5]
[7]
[8]
[9] Complications such as pituitary abscess due to sellar floor erosion and subsequent
diabetes insipidus have been described.[10]
[11]
[12] However, CIMDL synergistically potentiating systemic sepsis causing meningoencephalitis
with a sequel of communicating hydrocephalus has not been reported to date.
It is widely posited that many of the effects attributed to cocaine use are primarily
a consequence of vasculitis, which may mimic ANCA-associated vasculitis.[9]
[13] However, the presence or absence of ANCA in these patients may not always be consistent
with this clinical entity. The absence of granulomas on histopathological examination
either from the brain or the ulcers in the nasopharynx can differentiate CIMDL from
other vasculitis syndromes such as granulomatosis with polyangiitis or sarcoidosis.[5]
[14] Our patient did not have elevated ANCA or atypical ANCA levels. The histopathological
examination of the nasopharyngeal ulcer depicted the presence of inflammation and
vasculitis without evidence of granuloma formation.
Levamisole, an antihelmintic medication, is commonly employed as an additive in cocaine
to enhance its stimulant properties and prolong its effect.[15] However, it is associated with significant adverse effects such as toxic encephalopathy
which can complicate CIMDL. Nevertheless, the literature suggests that once the causative
agent is removed, the effects of toxic encephalopathy can typically be reversed. However,
recovery may be prolonged due to the extent of neurological damage sustained.[16]
Austrian syndrome is a similar condition characterized by the triad of endocarditis,
meningitis, and infection caused by Streptococcus pneumoniae.[17] In contrast, our patient was found to have MSSA, likely attributable to her history
of IV drug abuse. The interplay of drug abuse, endocarditis, and systemic sepsis culminated
in the development of fulminant meningitis, which subsequently led to communicating
hydrocephalus.
Conclusion
The overarching aim of this case report is to underscore the severe consequences of
cocaine abuse, particularly its potential to cause extensive skull base erosion. This
erosion can serve as a pathway for infections to spread to the brain, posing life-threatening
risks. Additionally, cocaine-induced erosion of the C1 arch may result in craniovertebral
junction instability. The debilitating effects of cocaine abuse leading to fulminant
meningitis, as demonstrated in this case, can sometimes necessitate permanent CSF
diversion.
This case emphasizes the critical importance of a multidisciplinary approach to care
involving skull base specialists, otolaryngologists, and maxillofacial teams to address
the complex challenges posed by such conditions.
