Neurobechets disease
45 year old female patient ,who is a homemaker was apparently asympotamatic 2 years back when she developed headache that is intermittent ,throbbing type ,diffuse,holocranial and bilateral , not associated with nausea ,vomitings ,Photophobia,phonophobia ,no blurring of vision.
Since 6 months patient complained of bilateral knee joint pains and elbow joint pains ,no history of restriction of joint movements ,no early morning stiffness ,no Small joint involvement ,no lower backache , used to visit local doctor and got treated symptomatically
When these symptoms started increasing they went to hospital and got admitted for 4- 5 days .Where some routine investigations were done.
On 17/03/2020 she was referred to psychiatrist regarding history of multiple body pains with disturbed sleep ,auditory hallucinations and was diagnosed with severe depression and anxiety without psychosis
Stressor factor being death of her younger son (who was blind and stayed at home,no schooling.One day he suddenly developed whole body edema and was diagnosed to be having Renal failure 2 years back . He was then admitted in NIMS, underwent renal biopsy and liver biopsy . After that he was put on medications and Was on regular follow up. When they were about to start Dialysis before one week he expired at the age of 21 years). Following death of his son , patient became depressed for which she was put on antidepressants and anti anxiety drugs and she used them for 1 week.
So going by the history which was given by her elder son . All her symptoms started since 2 years when her younger son had health issues , after the death of her son , her symptoms worsened.
Later after 1 week /10 days Patient started swaying on either side with weakness of right upper and lower limb ,she found difficulty in doing her routine work during which her family took care and made her rest for most of the times,later she went to her mothers home as she was seeking some support ,on that day (17/04/2020) patient fell down and there was sudden loss of speech for 6-8 hours,for which they rushed her to near by hospital.There is no history of loss of consciousness,no vomitings,no headache ,no seizure episodes ,no loss of memory ,no deviation of mouth,no bowel and bladder incontinence,no weakness of limbs.
CT BRAIN was done and it showed Old calcified granuloma in left high parietal region
and was given some fluids and reassurance .Patient regained her speech spontaneously.
But patient attenders were not satisfied and they took her to another hospital where MRI BRAIN was done that showed ACUTE INFARCTS IN BILATERAL CAPSUOLOGANGLIONIC REGION AND LEFT CEREBELLAR HEMISPHERE ( there was diffusion restrictions on ADC )
She was admitted for 1 week and was put on Dual antiplatelets ,Statins ,and physiotherapy was done
Routine Investigtions done at that time showed no abnormalities (RFT,FASTING LIPID PROFILE,CBP,CUE,SEROLOGY,RBS)
Since 6 months patient complained of bilateral knee joint pains and elbow joint pains ,no history of restriction of joint movements ,no early morning stiffness ,no Small joint involvement ,no lower backache , used to visit local doctor and got treated symptomatically
When these symptoms started increasing they went to hospital and got admitted for 4- 5 days .Where some routine investigations were done.
On 17/03/2020 she was referred to psychiatrist regarding history of multiple body pains with disturbed sleep ,auditory hallucinations and was diagnosed with severe depression and anxiety without psychosis
Stressor factor being death of her younger son (who was blind and stayed at home,no schooling.One day he suddenly developed whole body edema and was diagnosed to be having Renal failure 2 years back . He was then admitted in NIMS, underwent renal biopsy and liver biopsy . After that he was put on medications and Was on regular follow up. When they were about to start Dialysis before one week he expired at the age of 21 years). Following death of his son , patient became depressed for which she was put on antidepressants and anti anxiety drugs and she used them for 1 week.
So going by the history which was given by her elder son . All her symptoms started since 2 years when her younger son had health issues , after the death of her son , her symptoms worsened.
Later after 1 week /10 days Patient started swaying on either side with weakness of right upper and lower limb ,she found difficulty in doing her routine work during which her family took care and made her rest for most of the times,later she went to her mothers home as she was seeking some support ,on that day (17/04/2020) patient fell down and there was sudden loss of speech for 6-8 hours,for which they rushed her to near by hospital.There is no history of loss of consciousness,no vomitings,no headache ,no seizure episodes ,no loss of memory ,no deviation of mouth,no bowel and bladder incontinence,no weakness of limbs.
CT BRAIN was done and it showed Old calcified granuloma in left high parietal region
and was given some fluids and reassurance .Patient regained her speech spontaneously.
She was admitted for 1 week and was put on Dual antiplatelets ,Statins ,and physiotherapy was done
Routine Investigtions done at that time showed no abnormalities (RFT,FASTING LIPID PROFILE,CBP,CUE,SEROLOGY,RBS)
TSH hormone levels were 0.81 IU and they started her on Thyronorm 25 mcg once daily .
EEG was normal
Later patient got discharged .On 17th May they went for review and was said to continue the same treatment .She was in home for some days before she visited us .patient during that period had similar complaints .And patient used to walk more toward right side .Patient never had complete relief or decrease in symptoms
Now when she presented to us with complaints of increased swaying,giddiness,intermittent Diplopia ,Increased weakness of right
- Giddiness- continuous ,patient experiences While sitting , more while walking and was comfortable on lying down . not associated with vomitings ,no tinnitus,no hearing difficulties ,no ear discharge
- Diplopia - intermittent ,horizontal and binocular Diplopia for which patient herself is closing her left eye so as to avoid Diplopia ,as she complaints of blurring of vision in left eye,there was no history of black spots,colored halos
- Weakness of right upper and lower limb - initially when the patient was diagnosed with CVA she had mild symptoms ,that gradually progressed over 1 month to current status .Initially she used to walk alone till the bathroom With some difficulty ,later patient found difficulty in walking without support and patient felt more giddiness while walking .Patient is able to mix food with her right hand ,able to hold glass and drink water
- No difference in smell perception
- No history of dysphagia ,no drooling of saliva ,no difficulty in chewing food .
- No history of difficulty in closing eyelids,no deviation of mouth ,no watering of eyes
- No hearing difficulties
- No history of cotton wool sensations
- Patient is able to perceive hot and cold perception
- No history of band like sensations ,no bowel and bladder disturbances,no history of breathing difficulty
- no history of trauma
- No history of buckling or stiffness
- No history of scanning speech
- No history of memory loss or disorientation
- No history of crying spells,no abnormal behaviour
- No history of abortion
- No history of oral or genital ulcerations
- No history of skin lesions
PAST HISTORY : Non Diabetic,Non Hypertensive
No history of Asthma,CAD,Epilepsy
No Addictions
She underwent hysterectomy 20 years back
On General physical examination:
- Patients head is turned towards right and left eye is closed
- There is deviation of mouth towards left side and loss of nasolabial fold on right side.
- No pallor,icterus,cyanosis,clubbing,lymphadenopathy,edema
- Vitals are stable
Drug history : since 2 months
Patient was on
Dual antiplatelets
Statins
Cerebroprotien
She used Antidepressants for 1 week and stopped .
Marital and OBS history:
Age of menarche : 15 years ,regular cycles ,no dysmenorrhoea
She was married at the age of 18 years ,3rd degree consanguinity
1 st child- spontaneous vaginal delivery - 25 yrs old
2 nd child-vaginal delivery, died at 1 month of age due to unknown reason
3rd child - died at the age of 21 due to kidney failure
Higher mental functions :
Patient is right handed ,conscious,coherent,coperative
Well oriented to time,place,person
Short term and long term memory intact
Emotional state - patient has low mood and often cries because of her sons demise and her weakness
Lobar functions-
Attentive
Recall intact
Speech-patient has spontaneous speech ,with intact comprehension,naming,repetition ,normal fluency
Able to draw and copy symbols
Cranial nerve examination :
All cranial nerves are intact except
3rd cranial nerve :on right side patient has mild restriction of elevation,depression,dextro elevation,depression
Eyeball is central in position
Pupils are normal in size,reacting to light
6th cranial nerve : there is mild restriction on abduction
7th cranial nerve : loss of Nasolabial fold on right side and deviation of mouth
Patient is able to hold air in cheeks,able to frown ,able to whistle
Motor system examination :
Muscle bulk is normal in all limbs,no wasting
No involuntary movements
Attitude of Right lower limb - extend at knee and eternally rotated
Right. Left
Muscle tone :
Upper limb. Normal. Normal
Lower limb. Hypotonia Normal
Muscle power :
Upper limb
Shoulder -4/5 5/5
Arm. -4/5. 5/5
Forearm. -4/5. 5/5
Hand. -4/5. 5/5
Finger grasp. 50 %. 100 %
Lower limb :
Hip girdle. 2/5. 5/5
Knee. 2/5. 5/5
Lower leg. 2/5. 5/5
Foot. 2/5. 5/5
Great toe. 2/5. 5/5
Sensory system :
Pain,temperature,light touch,crude touch is intact
Vibration perception is decreased bilaterally
Joint proprioception is intact
Reflex :
Jaw reflex +2
Biceps : +3 +3
Triceps: +3 +3
Supinator: +3 +3
Knee +3 +3
Ankle +3 +3
Plantars. Extensor. Extensor
Gait : ATAXIC
Rombergs and Tandem walking could not be elicited
Cerebellar signs :
No nystagmus
No scanning speech
No past pointing
No dysdaidokinesia
Cortical sensations :
Tactile sensation -intact
Two point discrimination -intact
Sterognosis -intact
Graphesthesia -intact
Meningeal signs :
No Neck rigidity
Kernings sign negative
Brudzinkis sign negative
Here are some videos of her CNS examination
Other system examination : no abnormality found
Provisional Diagnosis :
Progressive weakness of right upper and lower limb with intermittent headaches , large joint pains
Bilateral pyramidal signs
With left UMN type of Facial paresis
Right 3rd and 6th cranial nerve paresis
Cerebellar involvement
? Recurrent stroke
? Demyelinating disorders
? Basilar artery occlusion
With the given history and examination we evaluated her further
MRI BRAIN (Plain and contrast )with Angiogram was done that showed
T2 weighted Transverse section MRI BRAIN showing hyperintensity involvement of Midbrain sparing Red Nucleus .
Carotid Artery Doppler:
ECG and ECHO are Normal .
Treatment:
1.Iv methyl prednisolone 1gm /IV was started and continued for 5 days
2.Tab.Ecospirin Av(75/20 mg) /po/OD
3.Tab.Thyrnorm 25mcg/po/OD
4. Supportive treatment
After IV Methylprednisolone Patient improved symptomatically
And objectively her power improved in upper limbs to 4/5 and lower limbs - initially from 2/5 to 4/5 over period of 2-3 days.
LUMBAR PUNCTURE :
Lumbar puncture was done on 31/05/2020 ,and CSF analysis was sent .
High protien and lymphocyte predominant - suggestive of inflammatory process
Patient was discharged and continuously followed up.
Patient improved symptomatically and is able to walk alone and able to perform her own activities .
Review MRI was done to look for any new lesions.Mri showed regression of hyperintense lesions .
Discussion:
1.https://academic.oup.com/brain/article/122/11/2171/377380
1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC490512/
This shows only few percent of people show Neurologic symptoms prior to systemic manifestations, and most commonly they develop within 6months - 1 year ,( range 6 months -3years )
On the other hand, Ikedat7 stressed that the common neurologic features of neuro- Behqet's syndrome were motor impairment especially bilateral pyramidal signs and that the mental changes mainly consisted of loss of emotional control with relative sparing of intelligence and memory.
We performed a systematic review and meta-analysis of studies on neurosarcoidosis published between 1980 and 2016
We identified 29 articles describing 1088 patients diagnosed between 1965 and 2015. Neurosarcoidosis occurred in 5% of patients with systemic sarcoidosis. Mean age at presentation was 43 years and neurological symptoms were the first clinical manifestation of sarcoidosis in 52%. The most commonly reported feature of neurosarcoidosis was cranial neuropathy in 55%, with the facial and optic nerve most commonly affected, followed by headache in 32%. Pleiocytosis and elevated CSF protein were found in 58 and 63%. MRI of the brain showed abnormalities in 70%. Chest X-ray, chest CT, or gallium-67-scintigraphy showed findings consistent with sarcoidosis in 60%, 70% and 69%, respectively.
3. Isolated Neuro sarcoidosis:
In all patients, no extranueral sarcoidosis developed during a relatively long follow-up period (mean 58 mo). Compared with the systemic neurosarcoidosis cohort (60), isolated neurosarcoidosispatients had similar demographics and neurological manifestations with a few exceptions including a more common frequency of headache, hemiparesis, and radiculopathy, leptomeningeal involvement on brain MRI, increased cell count in cerebrospinal fluid, and a more favorable clinical outcome (P<0.05).
4: Isolated neurosarcoidosis presenting as meningitis
The exact etiology of neurosarcoidosis is unknown and multifactorial, involving genetic predisposition and individual and environmental factors
Exposure to mildew, musty odors, pesticides, and agricultural employment have been associated with the development of sarcoidosis
Clinical manifestations of neurosarcoidosis can be found in 5–20% of cases of systemic sarcoidosis, and these symptoms can be mild or severe 5 (Table 2). About half of patients with neurosarcoidosis can present with neurologic manifestations sooner than systemic sarcoidosis is apparent
6: https://www.hindawi.com/journals/pri/2012/871019/
Articular involvement is characterized by nonerosive and nondeforming arthritis which often presents with monoarticular pattern, although asymmetrical polyarthritis can occur. The articular involvement is usually transient in nature with episodes lasting from a few days to weeks
Parenchymal involvement including brainstem involvement, hemispheric manifestations, spinal cord lesions, and meningoencephalitis is seen in the majority of patients (%80)
Table 6: Summary of evidence-based algorithmic therapy for Neuro-Behc ̧ et’s disease.
1st line. Corticosteroids
2nd line Azathioprine, cyclophosphamide, Anti-TNF-α, IFN-α
3rd line Methotrexate, Anticoagulation
In parenchymal involvement, corticosteroids (100 mg/d or 1 gx 5 days as pulse treatment) should be the first choice. Azathioprine is usually com- bined with corticosteroids. In severe or unresponsive cases, cyclophosphamide can be given additionally [83]. Anti-TNF- α agents and IFN-α are other new effective alternative agents [19]. Methotrexate is another treatment alternative [67, 68].
7. https://www.sciencedirect.com/science/article/pii/B9780702040887001103
Nervous system involvement, known as “neuro-BS” (NBS), is seen in about 5–10% of all cases. Clinical and imaging evidence suggests that primary neurologic involvement in BS may be subclassified into two major forms: the first, which is seen in the majority of patients, may be characterized as a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement, mostly presenting with a subacute brainstem syndrome and hemiparesis (intra-axial NBS); the other, which has few symptoms and a better neurologic prognosis, may be caused by isolated cerebral venous sinus thrombosis and intracranial hypertension(extra-axial NBS), occurring in 10–20% of the cases.
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