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Síndrome de disfunção combinada hiperativa dos nervos cranianos
ORPHA:221078CID-10 · G52.7CID-11 · 8A85DOENÇA RARA
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A cocaína é um alcaloide tropânico e estimulante do sistema nervoso central, derivado principalmente das folhas de duas espécies de coca nativas da América do Sul: Erythroxylum coca e E. novogranatense. As folhas são processadas em pasta de coca, uma mistura bruta de alcaloides da coca, da qual a base de cocaína é isolada e, então, convertida em cloridrato de cocaína. Embora a síntese total seja possível, ela é complexa e não é utilizada para produção. Historicamente, a cocaína foi um medicamento tópico padrão usado como anestésico local com propriedades vasoconstritoras intrínsecas. No entanto, seu alto potencial de abuso, efeitos adversos e custo limitaram seu uso médico e levaram à sua substituição por medicamentos alternativos.

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SUS: Cobertura mínimaScore: 15%
CID-10: G52.7
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Últimos 10 anos8publicações
Pico20253 papers
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0 papers (10 anos)
#1

Concomitant trigeminal and glossopharyngeal neuralgia: Illustrative case and scoping review.

Surgical neurology international2025

Cranial neuralgias are characterized by sharp lancinating pain that occurs on specific regions served by cranial nerves. Glossopharyngeal neuralgia (GPN) and trigeminal neuralgia (TN) are disorders for which etiology, epidemiology, and pain regions differ. On the other hand, the treatment stays the same as microvascular decompression, which serves as a safe approach for these entities. We performed a specific search strategy using the following Boolean terms: (Combined hyperactive dysfunction syndrome) OR ("Trigeminal" AND "Glossopharyngeal" AND "neuralgia") AND ("Concomitant" OR "Combined" OR "Simultaneous" OR "Concurrent" OR "at once" OR synchronously"). Including PubMed and Scopus, we found 73 results, of which 10 were included in the study. Furthermore, we did a manual search for one relevant study. The studies were included only if cases with concomitant TN and GPN were present. Fifteen cases were retrieved. The mean age of presentation was 66. About 53.3% of cases were left-sided; the most common etiology was posterior inferior cerebellar artery compression (73.3%). Microvascular decompression is this pathology's most popular management approach (73.3%). Only two cases reported complications (13.3%). The concomitant appearance of TN and GPN is scarce. The microvascular decompression persists as the most popular approach. There are a few treatment modalities used apart from surgical decompression. More research on these areas should be done to generate interest in their application.

#2

Comparison of Prognosis After Microvascular Decompression in Combined and Single Hyperactive Dysfunction Syndrome of the Cranial Nerves.

World neurosurgery2025 Dec

Hyperactive dysfunction syndrome (HDS) of cranial nerves is caused by vascular compression of the cranial nerves and results in manifestation of specific symptoms of the involved cranial nerves. The purpose of this study was to compare the prognosis of combined and single HDS after microvascular decompression (MVD) surgery and to identify the contributing factors. From January 2001 to December 2022, a total of 5034 patients with HDS underwent 5055 MVD surgeries, of which 45 patients with combined HDS underwent 66 surgeries. Data concerning the subjects' demographic and disease-related characteristics and postoperative prognosis were collected by reviewing electronic medical records. Combined HDS had metachronous onset in 86.7% of patients and bilateral involvement in 66.7%. Hemifacial spasm (HFS) followed by trigeminal neuralgia (TN) was the most common type of combined HDS (28.9%). No significant differences were observed in postoperative prognosis between combined and single HDS for HFS, TN, and glossopharyngeal neuralgia. In combined HDS, no differences were observed in postoperative prognosis according to sex, age at surgery, symptom duration, comorbidities, and offending vessels for both HFS and TN. No significant differences in postoperative complications were identified between combined and single HDS. The prognosis after MVD surgery for combined HDS was similar to that for single HDS. MVD can be recommended as a safe and effective treatment option for patients with combined as well as single HDS.

#3

A New Form of Combined Hyperactive Dysfunction Syndrome: A Unique Case.

Cureus2025 Mar

Pain is an unpleasant sensory and emotional experience associated with actual or potential tissue damage. There are different types of pain. Neuropathic pain is caused by a lesion or pathological condition of the somatosensory nervous system. One of the most well-known neuropathic pains is trigeminal neuralgia (TN). However, occipital neuralgia (ON) and glossopharyngeal neuralgia (GPN) exist. These three rare pathologies occur in a very small part of the global population. Combined hyperactive dysfunction syndrome is a rare disorder characterized by overactivity in different cranial nerves, TN, hemifacial spasm, and GPN. In this paper, we present a very rare case of a young woman affected by an association of overactivity in the trigeminal, occipital, and glossopharyngeal nerves, leading to TN, ON, and GPN simultaneously.

#4

Glossopharyngeal neuralgia as initial symptom in combined hyperactive dysfunction syndrome: case report.

British journal of neurosurgery2023 Jun

Hyperactive dysfunction syndrome (HDS) is defined as symptoms arising from overactivities in cranial nerves, like trigeminal neuralgia (TN), hemifacial spasm (HFS), and glossopharyngeal neuralgia (GPN). A combination of these cranial nerve neuralgias, that might or might not occur in one or both sides, either synchronously, or metachronously is called combined hyperactive dysfunction syndrome (CHDS). We presented a 73 years-old male patient with CHDS presenting with GPN as the initial symptom, with total relief from GPN, TN, and HFS after microvascular decompression. Up to date, only nine patients have been reported in the literature with symptomatic. TN-HFS-GPN. Our case is the first case with GPN as the initial symptom. The combination of arterial and venous origin of the offending vessels makes the case picturesage.

#5

Combined hyperactive dysfunction syndrome of the cranial nerves complicated by essential hypertension: A case report.

Medicine2019 Aug

Combined hyperactive dysfunction syndrome (HDS) refers to a special type of HDS characterized by a combination of trigeminal neuralgia (TN), hemi facial spasm (HFS), and/or gloss pharyngeal neuralgia (GPN). Rostra ventrolateral medulla (RVLM) plays a crucial role in central cardiovascular regulation, and neurovascular compression of the RVLM has been identified as a contributor to essential hypertension. A 65-year-old female with a facial tic and pain located in the root of the tongue and throat on the same side; the systolic and diastolic blood pressure was approximately 170 and 100 mmHg. The patient was diagnosed with combined HDS (HFS-GPN) and essential hypertension. Brain magnetic resonance 3-dimensional time-of-flight imaging and digital subtraction angiography revealed vertebrobasilar artery compressed the left RVLM and contacted with the root entry zones of multiple cranial nerves. The patient was treated with microvascular decompression surgery OUTCOMES:: The symptoms were completely relieved, and blood pressure was well-controlled. The pathological association of hypertension and HDS should be highlighted, and microvascular decompression is an effective approach for relieving the hypertension.

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Concomitant trigeminal and glossopharyngeal neuralgia: Illustrative case and scoping review.
    Surgical neurology international· 2025· PMID 40469332mais citado
  2. Comparison of Prognosis After Microvascular Decompression in Combined and Single Hyperactive Dysfunction Syndrome of the Cranial Nerves.
    World neurosurgery· 2025· PMID 41077182mais citado
  3. A New Form of Combined Hyperactive Dysfunction Syndrome: A Unique Case.
    Cureus· 2025· PMID 40236341mais citado
  4. Glossopharyngeal neuralgia as initial symptom in combined hyperactive dysfunction syndrome: case report.
    British journal of neurosurgery· 2023· PMID 32915076mais citado
  5. Combined hyperactive dysfunction syndrome of the cranial nerves complicated by essential hypertension: A case report.
    Medicine· 2019· PMID 31415413mais citado
  6. Combined Hyperactive Dysfunction Syndrome of the Cranial Nerves: Analysis of 37 Cases and Literature Review.
    World Neurosurg· 2019· PMID 31158546recente
  7. Microvascular Decompression of the Optic Nerve for Paroxysmal Phosphenes and Visual Field Deficit.
    World Neurosurg· 2016· PMID 26459702recente
  8. [Painful tic convulsif and Botulinum toxin].
    Rev Neurol· 2006· PMID 16775798recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:221078(Orphanet)
  2. MONDO:0016371(MONDO)
  3. GARD:20541(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55786175(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

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Síndrome de disfunção combinada hiperativa dos nervos cranianos

ORPHA:221078 · MONDO:0016371
CID-10
G52.7 · Transtornos de múltiplos nervos cranianos
CID-11
MedGen
UMLS
C5190851
EuropePMC
Wikidata
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