Anesthetic management of deep brain stimulation in Hallervorden–Spatz syndrome: Surviving the “eye of the tiger” : Saudi Journal of Anaesthesia

Secondary Logo

Journal Logo

Case Report

Anesthetic management of deep brain stimulation in Hallervorden–Spatz syndrome

Surviving the “eye of the tiger”

Monteiro, Joseph Nascimento; Bedekar, Unmesh Pramod

Author Information
Saudi Journal of Anaesthesia 17(2):p 278-280, Apr–Jun 2023. | DOI: 10.4103/sja.sja_728_22
  • Open


Anesthetic management of dystonic patients with uncontrolled involuntary movements refractory to medical management is a challenge to neuroanaesthetists. According to some studies, the prevalence of Panthothenate Kinase Associated Neurodegeneration is 1 to 9/1,000,000. Report of Deep Brain Stimulation for Hallervorden–Spatz is extremely rare in literature. “Awake” bilateral electrode placement, with microelectrode recording (MER) and stimulation with a scalp nerve block, titrated conscious sedation with Monitored Anesthesia Care (MAC) is preferable. However, in those patients needing general anesthesia, a balanced anesthesia technique with careful selection and monitored titration of anesthetic drugs ensuring MERs for precise placement and stimulation of target nuclei along with adequate plane and depth of anesthesia and prevention of awareness are essentially the key factors in the anesthetic management. Surgery is the mainstay of the disease due to poor response to medical management. Multidisciplinary collaboration and cooperation among neurologists, neurosurgeons, neuroradiologists, and neuroanaesthesiologists are imperative to ensure good patient outcomes.

Case Reports

A 24-year-old male patient weighing 38 kg was posted for bilateral deep brain stimulation for dystonia. The patient started complaining of a change in handwriting for the past 7 years which was gradually worsening [Figure 1]. Later, he started having difficulty in swallowing, involuntary closure of the right eyelid, and difficulty swallowing and speaking for one year. There were dystonic movements of the tongue which were troublesome. The patient was on tablet trihexyphenidyl 2 mg to help with the tremors and on tablet clonazepam 0.5 mg as a benzodiazepine sedative. Motor power was 5/5 in all the limbs and the sensory system was normal with no deficits.

Figure 1:
MRI of Hallervorden–Spatz pre-placement [“Eye of the Tiger” appearance] and post-electrode placement

The MRI of the patient showed bilateral symmetric hyperintensities within hypo intensity in medial globus pallidus and accumulation of iron suggesting “eye of the tiger appearance.”[1] The site of deposition was the cause of dystonia.

After attaching the standard ASA monitors, the patient was sedated with an injection midazolam 1 mg IV and an injection fentanyl 50 ug. Antiemetic prophylaxis was ensured before the commencement of surgery. Injection propofol 50 mg IV was administered and an injection atracurium 35 mg was after ensuring that the patient was getting ventilated. After confirming the endotracheal tube, a 20 G arterial line was secured under anesthesia. A mouth gag was placed in the mouth to prevent the patient from biting the tube. The Bispectral Index strip was attached and monitoring was started.

The patient was taken for a CT scan on manual ventilation keeping him sedated. The exact site of electrode placement by superimposing CT scan images on the neuro-navigation system was determined in the operation theater.

Infusions of injection propofol at the rate of 50 ug/kg/h were started along with the infusion of injection dexmedetomidine at the rate of 0.5 mg/kg/min and injection ketamine at the rate of 0.5 mg/kg min in distinct IV access. No muscle relaxants were given after the induction dose. BIS and TOF (Train of four) monitoring was done throughout the case. Four twitches of TOF and a BIS value of 50 were maintained in the case. MAC (Minimum Alveolar Concentration) value was maintained between 0.2 and 0.3. Mechanical compression stockings were placed to reduce the risk of deep vein thrombosis.

After reaching the coordinates marked preoperatively, stimulation was started and the dystonia was relieved with the help of the attending neurologist. This time the patient was not on any muscle relaxants but adequate depth of anesthesia was ensured to prevent awareness in the patient. The electrode placement was done after ensuring the exact location. In the later part of the surgery, the patient was fully relaxed with continued TOF monitoring during the placement of electrodes and closure of the dura. MRI brain was done to confirm the placement, and pulse generator insertion was done after the MRI was confirmatory in general anesthesia itself.

There was no intraoperative hemodynamic instability in the patient and uneventful extubation was done after the patient was warm, normovolemic, and obeying commands. There was no respiratory depression or signs of upper airway obstruction in the patient in the postoperative period.


The patient was a known case of Hallervorden–Spatz disease. It is an autosomal recessive disorder characterized by dystonia and iron accumulation in the brain, with mutations in the gene encoding pantothenate kinase[2] (PANK2). Its prevalence is 1 to 9/1,000,000. Reports of deep brain stimulation for Hallervorden–Spatz are extremely rare in literature.

Clinically, it manifests as progressive movement abnormalities and dementia, and unfortunately, medical treatment is limited and dystonia is usually refractory. Hence, stereotactic surgery with placement and stimulation of deep brain stimulation electrodes is increasingly preferred.[3]

In the entire spectrum of childhood movement disorders, dystonia is the most common, most severe, and most challenging movement disorder. The management of such a movement disorder needs a multidisciplinary approach that includes a neurologist, neurosurgeon, neuroanesthesiologist, neuropsychologist, nursing staff, and physiotherapist to help in rehabilitation.[4]

Preoperative airway evaluation, eliciting a history of airway obstruction associated with dystonia, is imperative as being prepared for sudden airway obstruction both during an “awake” DBS electrode placement under scalp block and conscious sedation, as well as securing a definite airway during induction and careful extubation after the procedure is essential as was done in our cases due to cervical dystonia and potential airway compromise. Muscular rigidity including torticollis may cause a difficult airway management scenario. Extensive experience with basal ganglia disorders in general and dystonia, in particular, suggests that extrapyramidal signs such as chorea, athetosis, dystonia, rigidity, and tremor do not completely disappear after induction of general and may reappear on emergency anesthesia. Keegan et al. describe a case of an 11-year-old affected by Hallervorden–Spatz disease who underwent thalamotomy under general anesthesia and post-extubation had complete airway obstruction and required re-intubation followed by negative pressure pulmonary edema.[5]

These patients are usually on anticholinergics which are associated with central anticholinergic syndrome causing postoperative cognitive dysfunction[6] and delay post-surgical recovery.[7]

Performing any procedure may be challenging due to constant uncontrolled, brisk, and jerky involuntary movements. The involuntary movements are blunted under anesthesia and reappear on emergence.

Patients are usually on long-term benzodiazepines, so we prefer titrated doses of intravenous midazolam balancing the risk of airway obstruction with the benefit of anxiolysis. Antiemetic like ondansetron as prophylaxis which has no central cholinergic effect for emesis was given.

Induction agents such as thiopentone and propofol[8] have been used safely, however, propofol is known to affect microelectrode recordings and pallidal neuronal discharges[9]; hence, we avoid boluses of propofol.

Owing to immobility, hyperkalaemic cardiac arrest induced by succinylcholine is always a possibility.[10] There are several reports of autopsies revealing muscle wasting secondary to poor nutrition and diffuse axonal changes in the brain which may involve upper motor neuron lesions, thereby increasing the possibility of a hyperkalemic cardiac arrest.


An understanding of the pathophysiology and a rationale anesthetic plan in close coordination between the neuroanaesthesiologist and neurosurgeon in the perioperative period is essential to manage these cases with safe patient outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1. Hinkelbein J, Kalenka A, Alb M. Anesthesia for patients with pantothenate-kinase-associated neurodegeneration (Hallervorden -Spatz disease)-a literature review Acta Neuropsychiatr. 2006;18:168–72
2. Hayflick SJ, Westaway SK, Levinson B, Zhou B, Johnson MA, Ching KHL, et al Genetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome N Engl J Med. 2003;348:33–40
3. Hurtardo P, Salvador L, Carrero E, Rumia J, Fàbregas N. Anesthesia considerations for deep-brain stimulation in a patient with type-2 pantothenate kinase deficiency (Hallervorden-Spatz disease) Rev Esp Anestesiol Reanim. 2009;56:180–4
4. Monteiro JN, Thakore B, Jangra KRath GP. Anesthesia for pediatric deep brain stimulation surgery Fundamental Pediatric Neuroanesthesia. 2021 Springer Nature Singapore pte. Ltd doi: 10.1007/978-981-16-3376-8_26
5. Keegan MT, Flick RP, Matsumoto JY, Davis DH, Lanier WL. Anesthetic management for two stage computer assisted, stereotactic thalamotomy in a child with Hallervorden Spatz disease J Neurosurg Anesthesiol. 2000;12:107–11
6. Engelhard K, Werner C. Postoperative cognitive dysfunction Anaesthesist. 2005;54:588–94
7. Krause M, Fogel W, Kloss M, Rasche D, Volkmann J, Tronnier V. Pallidal stimulation for dystonia Neurosurgery. 2004;55:1361–8
8. Balas I, Kovacs N, Hollody K. Staged bilateral stereotactic pallidothalamotomy for life threatening dystonia in a child with Hallervorden-Spatz disease Mov Disord. 2006;21:82–5
9. Steigerwald F, Hinz L, Pinsker MO, Herzog J, Stiller UK, Kopper F, et al Effect of propofol anesthesia on pallidal neuronal discharges in generalized dystonia Neurosci Lett. 2005;386:156–9
10. Roy RC, McLain S, Wise A, Shaffner LD. Anesthetic management of a patient with HallervordenSpatz disease Anesthesiology. 1983;58:382–4

Anesthesia; deep brain stimulation; dystonia; Hallervorden–Spatz disease

© 2023 Saudi Journal of Anaesthesia | Published by Wolters Kluwer – Medknow