해외 증례(Nepal) Autoimmune gastritis in a male adolescent with cerebellar involvement: A case report

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2022-11-06
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A/S: 17세 남자

P/I: 약 2.5개월 전부터 발생한 슬리퍼를 신기 힘들 정도의 both leg weakness, 이후 부축을 받지 않으면 걷지 못하였으며, 증상 발생 1개월부터 원인 모를 간헐적 발열도 있었고, 균형을 잃은 걸음걸이와 팔과 다리를 간헐적으로 떠는 증상이 지속됨. 지난 2개월 동안 원하는 단어를 떠올리거나 말하기 어려웠고, 가족이나 친구의 이름을 말하기 어렵고, 간단한 수학연산도 어려워 함. 

* excessive sweating (+), severe lethargy (+), easy fatigue (+), decreased touch and warm/cold sensation in lower limb(+)

* unsteady gait (+), slurred speech (+), vertigo (+), swinging movement of his trunk while standing and walking, even with support (+)

* back pain (-), stool & urinary incontinence/urinary retention/constipation (-), dizziness/double vision/decreased vision/decreased taste sensation/deviation of angle mouth/vomiting/headache (-)

* history of diarrhea/shortness of breath/sore throat/blurring of vision/photophobia (-)

* family history (-), past history of tuberculosis (-), diabetes mellitus (-), other significant comorbiditie (-)

* history of trauma (-), illicit drug use (-), use of herbal remedies (-), unprotected sexual intercourse (-), insect bite (-)

* non-vegetarian & consumed both red and white meat 

P/Ex.

* Orientation (normal)

* Glasgow Coma Scale (15), minimal mental state examination (MMSE) (20/30, problem: recall, attention and calculation)

* General exam (pallor, glossitis, stomatitis, lymphadenopathy: all negative), cranial nerve exam (normal)

* Motor: bulk, power, tone - normal (all limbs)

* Ankle flexors & extensors: 2/5 bilaterally, Knee flexors and extensors: 3/5, Hip foexors and extensors: 4+/5, upper limb: normal

* Deep tendon reflex (DTR): both ankle and knee (absent), both biceps tendon and supinators (decreased)

* Intentional tremor (+), dysmetria (운동실조) with staggering gait (+)

* nystagmus (-) dysdiadochokinesia (-) finger nose test (-) heel shin test (-) 

* Bilateral Babinski sign (present), Joing position (decreased), Vibration sense (bilaterally) (absent)

* Temperature & fine touch sensation: both lower limb (decreased), upper limb (intact)

* Abdominal, cardiovascular, respiratory exam: normal

* Ultrasound exam: focal fat sparing in the perihilar region (liver), spleen (normal)

* MRI check: symmetrical T2 hyper intensity along the medial aspect of the bilateral cerebellar hemisphere - demyelination (Figure 1) / spine MRI (normal: mild sub-acute combined degeneration of spinal cord)

* Nuerologist consult: Cobalamin deficiency로 인해 cerebellum, dorsal column and peripheral nerve를 침범한 ataxia로 진단 됨

Treatment: IM methylcobalamine 1000mcg (alternative days, 5 doses) -> vibration, position sense, touch & temperature sensations, cognitive symptoms 호전 -> IM methylcobalamine 1000mcg (weekly) 하기로 하고 퇴원 함. 퇴원 당시 일어서거나 몇 걸음 걸을 수 있었고, atatxia도 회복 되어 짐 -> 지속적인 치료 후 대부분의 운동증상, 보향, 관절 유지, 진동 감각, DTR 모두 서서히 회복되고 떨림도 줄어들었으며, support 없이 걸을 수 있게 됨 -> 3개월 후 cognition, MMSE (28/30) 회복됨 -> 평생동안 3개월 간격 지속 투여하기로 함. 이후 시행한 MRI (Figure 2: no altered signal intensity along the medial aspect of the bilateral cerebellar hemisphere)

* Anti-parietal cell antibody titer (1:40) / EGD는 거부하여 시행하지 못함 

Diagnosis: Autoimmune gastritis로 인해 발생한 vitamin B12 deficiency에 따른 ataxia

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... 내시경 검사 및 조직검사 등을 하지 못했던 것이 아쉬운 case 입니다. serum cobalamin level은 보통 200 pg/mL 을 결핍으로 보는데, 200~400 pg/mL 인 경우 실제 결핍일 수 있습니다. 이 환자는 입원 당시 매우 낮았습니다. 보통은 vegetarian 의 경우 있을 수 있는데, 이 환자에서 처럼 정상적인 diet를 하는 경우에 이러한 vitamin B12 결핍으로 신경학적 증상이 현저한 경우는 매우 드문 case 입니다. 청소년에서 AIG가 드물기도 하지만 이렇게 non-vegetarian의 경우에도 AIG와 동반되어 vitamin B12 결핍과 이로 인한 신경학적 징후가 동반될 수 있다는 것을 인지해야 할 것 같습니다. 


Table 1. Laboratory investigation findings of the indexed case. 


Figure 1. MRI scan of cerebellum showing hyper intensity along the medial aspect of the bilateral cerebellar hemisphere. (Left)

Figure 2. MRI follow up scan of cerebellum showing no altered signal intensity along the medial aspect of the bilateral cerebellar hemisphere. (Right)


<Abstract>

Introduction: Autoimmune gastritis is an immune mediated disorder characterized as anti-intrinsic factor and anti-parietal cell autoantibodies directed against intrinsic factor and parietal cells of the stomach respectively, leading to vitamin B12 deficiency. When the disease remains undiagnosed and untreated, it may lead to neurological complications and even fatal anemia. Case study: We exemplify a non-vegetarian male adolescent case with the neurological symptoms such as bilateral leg weakness, unsteady gait, slurred speech, vertigo, slowed movement, lethargy, and impaired joint sensation. None of his family members had such illness. His hemoglobin was normal with serum vitamin B12 level 105 pg/ mL and anti-intrinsic factor antibody titer positive. A presumed diagnosis of cobalamin deficiency with involvement of the cerebellum, dorsal column and peripheral nerves was made. His symptoms recovered gradually and later completely (after 6 months) after the intramuscular vitamin B12 therapy. Clinical discussion: The indexed rare adolescent case had auto immune gastritis showing neurological manifestation with more pronounced cerebellar features and vitamin B12 deficiency under the non-vegetarian diet consumption. Previous studies had reflected auto immune among adolescents but contrasted some of the clinical features. Conclusion: For the prompt and precise diagnosis of the autoimmune gastritis and to prevent further complications, some of the rare conditions such as deficiency with a non-vegetarian diet, neurological manifestation including cerebellar involvement without anemia should also be considered along with other relevant symptoms. The heightened awareness for timely surveillance and treatment will contribute in reduction of such unusual cases.

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대한자가면역성위염연구회

주소 : 경기도 용인시 기흥구 중부대로 579, 508-23호 (구갈동, 강남대프라자)

대표전화 : 070-8080-0453  이메일 : autogastritis@gmail.com 


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