Including cannabinoids in the treatment of painful schwannomatosis

A 47‐year‐old man, affected by Schwannomatosis, presented a very severe pain (10/10, NRS) with paroxysmal shooting episodes, allodynia, paresthesia, and dysesthesia; in parallel, the patient had lost weight (from 70 to 49 kg) and experienced fatigue and deep depression. The previous pain prescription, including opioids and antineutopathic drugs, was fully ineffective. We progressively substituted this therapy with 15 drops, 3 times/daily, of THC/CBD in a concentration ratio 5:1, equal to 15 mg of active substance each time, reaching improvement in pain intensity (6/10) and in several other aspects as mood and quality of life


Including cannabinoids in the treatment of painful schwannomatosis
Schwannomatosis is a rare form of neurofibromatosis affecting one in approximately 40,000 individuals. It is a clinical condition represented by the development of schwannomas, benign encapsulated tumors originating from Schwann cells. Schwannomas preferentially affect the spine (74%) and peripheral nerves (89%).
We describe the medical history of a 47-year-old man with Schwannomatosis who accessed our neurology department in November 2016.
The onset in 2011 featured signs and symptoms of neuropathic pain, such as paroxysmal shooting pain in the forearms, legs, and inguinal area, together with superficial mechanical allodynia, paresthesia, and dysesthesia in the same areas. Neurologic and imaging examinations (MRI, EMG, ENG, needle biopsy) revealed multiple neurinomas of unknown cause.
The patient described his pain as ranging from a tingling or needle pricking sensation to that of a classic jolting shock or as if someone were tightly pinching a nerve, making it impossible for him to extend his limbs.
Ten months later, a new series of diagnostic tests (contrast MRI, EMG, ENG, biopsy) lead to the diagnosis of Schwannomatosis with sensory and motor neuropathy.
In June 2013, 6 masses were surgically removed from the right femoral nerve, inguinal and right axillary regions. The nodules were located on the nerve sheaths like beads on a rosary having round, oval or rice grain shapes. After surgery, there was a pain intensity reduction from 8/10 to 6/10 on 0-10 numerical rating scale The pain therapy consisted of pregabalin 1,200 mg/qd, carbamazepine 400 mg, amitriptyline 70 mg, clonazepam 2,5 mg, tramadol 50 mg up to four times qd, but had a very poor efficacy.
The pain therapy unit was consulted and a new therapy schedule was initiated including a high-protein diet of 4,000 Kcal qd and motor rehabilitation. Pain treatment was changed. Pregabalin was interrupted and the following drugs were administered daily: oxycodone/ naloxone 30 + 15 mg; paracetamol 3,000 mg; vitamin B1 300 mg; vitamin D/colecalciferol 35 g; oil solution, as sublingual administration, containing tetra-hydro-cannabinol (THC) 19% = 5 g + cannabidiol (CBD) crystal 99% = 1 g (10 sublingual drops, equivalent to 10 mg of active substance, 3 times/daily, after increased until 15 drops equal to 15 mg, 3 times/daily for achieve a better state of well-being); duloxetine 120 mg; clonazepam drops (4,5 mg). This last treatment has been ongoing for about a year and it is still ongoing.
The patient had an overall improvement in pain scores (6/10), motor activity, quality of sleep, social relations and mood.
Progressively the fatigue was reduced and the patient could walk on his own.
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