Case presentation
A 35-year-old Caucasian man, residing in an urban area, with right
laterality was admitted to our Neurology department in April 2022.
He was diagnosed with SM in October 2021 according to the latest
criteria from the WHO. Bone marrow biopsy showed multifocal, dense
infiltrates of mast cells (≥15 mast cells in aggregates) positive for
c-Kit CD 117. Serum basal tryptase measurements were positive. At the
time of diagnosis, complete blood count did not show anaemia,
thrombocytopenia, leukopenia or leucocytosis and abdominal ultrasound
was normal. Treatment with multikinase inhibitor Midostaurin 200mg/day
was initiated.
In April 2022 the patient was admitted to the Emergency Department for 3
subnitrate generalized tonic-clonic seizures associated with urine
incontinence, after a period of sleep deprivation and consumption of
neurostimulator drinks. His vital signs showed tachycardia with a heart
rate of 96 beats/minute, blood pressure of 85/60 mmHg, oxygen saturation
level of 85%, and temperature of 36 degrees Celsius. The patient was
unresponsive to first and second-level antiepileptic drugs, with a
tendency to vascular collapse. Considering the worsening of the clinical
condition, he was sedated with Thiopental and intubated, receiving
mechanical ventilation and high doses of inotropic medication due to
persistent hypotension. Laboratory values at admission showed elevated
liver enzymes, mild hyponatremia, and hypokalaemia, mild normocytic
normochromic anemia, and high serum creatine kinase levels. Head
computed tomography angiography showed no signs of cerebral hemorrhage,
stroke, or other abnormalities.
The patient developed a high temperature, 24 hours before admission, for
which a lumbar puncture was performed, an emergency MRI (Magnetic
Resonance Imaging) with gadolinium and empiric antimicrobial therapy
with Ceftriaxone 4 grams/day and Clindamycin 2,7 grams/day was
initiated. The results of the lumbar puncture were within normal limits
(negative results for multiplex panel) and the brain MRI revealed
pansinusitis and bilateral mastoiditis. Considering the MRI images, the
otorhinolaryngologist performed an otoscopy and established the
diagnosis of right suppurating otitis media, for which he drained the
secretions with a favourable result at the 48-hour reassessment. After 5
days of admission, it is decided to safely extubate the patient with a
slowly favourable evolution, the patient being later transferred to the
Neurology department.
The neurological examination showed the following: normal level of
alertness, pyramidal tract signs characterized by tetraparesis with a
score of 4/5 at lower limbs and 3/5 at upper limbs (on the
MRC—Modified Research Council scale). The psychological examination
revealed mild cognitive dysfunction (MMSE- Mini-Mental State Examination
24/30), executive dysfunction, and behavioural disinhibition.
Immediately after the patient was admitted to the Neurology department,
he became unstable and presented with decreased oxygen saturation and
hypotension. He was transferred to the Intensive Care Unit (ICU) and
after 3 days, he was transferred back to the Neurology department.
One hour after the patient was admitted to the Neurology department, he
presented with a sudden change in general condition, dyspnoea, severe
diffuse headache without loss of consciousness, decreased oxygen
saturation (up to 55% in ambient air), hypotension (70/40mmHg) and
tachycardia. During the episode of decompensation, the patient insisted
that we do not say anything to his mother about the worsening of his
clinical condition and wanted to draw up the will, but still he was not
afraid of death. Therefore, in association with the medical treatment
comprising of inotropic medication, oxygen therapy with increased flow
on a simple mask, corticotherapy and antihistamines, he also received a
homeopathic remedy: Carbo Vegetabilis 30 CH 5 granules every 5 minutes
(for 20 minutes). The patient’s general condition improved.
During hospitalization, the patient repeatedly expressed his wish to sue
the hematologist who prescribed Midostaurin. Upon talking to his father,
we found out that the patient has a highly reactive and compulsive
personality and a low tolerance for injustice. Taking this into account,
the patient was prescribed Causticum 30 CH seven granules administered
sublingually daily, for one month. During and after hospitalization, the
patient was treated with conventional therapy and a series of
individualized homeopathic remedies, as it is presented in Table 2.
Considering the low tolerance of the patient to the treatment with
Midostaurin (nausea, vomiting, abdominal pain, hepatocytolysis), upon
the recommendation of the hematologist, the therapy was stopped. He was
discharged with a diagnosis of severe SM with repetitive vasomotor
collapses and generalized tonic-clonic seizures.