Dados do Trabalho

Título

BASAL GANGLIA ENCEPHALITIS WITH FULMINANT PARKINSONIAN FEATURES AND DYSAUTONOMIA IN A CHILD: LOOK NO FURTHER THAN LEVODOPA

Apresentação dos casos

A 5-year-old presented with an altered mental status, rigidity, and tremors. A few days earlier, he experienced a minor upper respiratory infection. He became confused six days later and developed a slow and monotonous tone of voice. A head CT was unremarkable and lumbar puncture showed 7 cells/mm3 (100% lymphocytes) and slightly increased protein on CSF. A treatment for possible herpes simplex virus encephalitis was initiated with acyclovir.
His condition deteriorated, with spasms, akinetic mutism, worsening consciousness, and severe dystonic seizures. Brain MRI revealed bilateral hyperintensities in the striatum and caudate, with minor gadolinium enhancement. PCR tests for HSV and other viruses were negative. He was treated with methylprednisolone (20mg/kg) for 5 days and Intravenous immunoglobulin (400mg/kg/day) for 5 days. Acyclovir was discontinued.
The patient exhibited painful dystonia with severe oromandibular spasms. Following immunotherapy, he could interact but continued with akinetic mutism. As his parkinsonian features worsened, he developed a spiking fever and a slow rise in creatine phosphokinase levels. A trial of levodopa/benserazide up to 20 mg/kg/day was administered, which controlled his dystonic seizures and significantly improved his rigidity. A repeat lumbar puncture showed negative neuronal surface antibodies and onconeural antibodies, and low serum titers of anti-GAD antibodies (1/64). Both, IgG and IgM antibodies against Mycoplasma pneumoniae, were positive.
Six weeks after symptom onset, the patient was discharged with a diagnosis of autoimmune/post-infectious basal ganglia encephalitis. He was fully ambulatory and speaking a few words.

Discussão

Basal ganglia encephalitis is a rare autoimmune or post-infectious [1] encephalitis primarily affecting the caudate and putamen [2]. Symptoms include severe parkinsonism, dystonia, and altered consciousness [3]. Reported causes include infectious agents like Mycoplasma pneumoniae [4]. However, the discovery of anti-D2R antibodies targeting the basal ganglia suggests an autoimmune etiology, potentially triggered by an infection [3,4].

Comentários finais

Given the rarity of this condition, large-scale studies may not provide definitive treatment guidelines. Nonetheless, this case demonstrates that high doses of levodopa can improve parkinsonism and resolve dystonic seizures and dysautonomia, even as immunotherapy may not fully halt the autoimmune process.

Referências

[1] Gowda, Vykuntaraju K.; Gupta, Priya; Shivappa, Sanjay K.1; Bhat, Maya2. Basal Ganglia Autoimmune Encephalitis Following Leptospirosis. Neurology India 70(5):p 2121-2124, Sep–Oct 2022. | DOI: 10.4103/0028-3886.359156.
[2] Kitazaki Y, Shirafuji N, Takaku N, Yamaguchi T, Enomoto S, Ikawa M, Yamamura O, Nakamoto Y, Hamano T. Autoimmune basal ganglia encephalitis associated with anti-recoverin antibodies: A case report. eNeurologicalSci. 2021 Nov 24;25:100382. doi: 10.1016/j.ensci.2021.100382. PMID: 34877416; PMCID: PMC8628202.
[3] Giri A, Andhale A, Acharya S, Kumar Singh R, Talwar D. Seronegative Autoimmune Basal Ganglia Encephalitis Presenting as Acute Parkinsonism and Refractory Faciobrachial Seizures: A Case Report. Cureus. 2022 Jan 17;14(1):e21351. doi: 10.7759/cureus.21351. PMID: 35186605; PMCID: PMC8849290.
[4] P65 – 2865: Basal ganglia inflammation and a severe dystonic-akinetic syndrome after mycoplasma pneumonia infection in a 6 years old boy. Gruber-Sedlmayr, U. et al. European Journal of Paediatric Neurology, Volume 19, S112.

Palavras Chave

basal ganglia encephalitis; childhood parkinsonism; mycoplasma encephalitis