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\begin{document}

\title{An unusual case of Dyke-Davidoff-Masson syndrome revealed by status
epilepticus in a Malian patient}



\author[1]{Samba  Djimdé}%
\author[2]{Abdoulaye Yalcouyé}%
\author[2]{Abdou Koita}%
\author[2]{Hassana Samir }%
\author[2]{Pofinet  Kebkiba}%
\author[2]{Awovi  Gueli}%
\author[2]{Alassane  Maiga}%
\author[3]{Adama  Sissoko}%
\author[3]{Guida  Landouré}%
\affil[1]{ Service de Neurologie, Centre Hospitalier Universitaire du Point G, Bamako, Mali}%
\affil[2]{Faculté de Médecine et d'Odontostomatologie, USTTB, Bamako, Mali}%
\affil[3]{Service de Neurologie, Centre Hospitalier Universitaire du Point G, Bamako, Mali}%


\vspace{-1em}



  
  \date{May 26, 2024}


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\selectlanguage{english}
\begin{abstract}
Duke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition
with unknown global prevalence. It typically manifests with body
asymmetry, drugs resistant epilepsy, mental retardation, cerebral
atrophy, skull bone thickening and hyperpneumatization of the frontal
sinuses. In this report, we present an unusual case of DDMS revealed by
status epilepticus.%
\end{abstract}%



\sloppy


\textbf{Introduction}

Duke-Davidoff-Masson syndrome (DDMS) is a rare neurological condition
with unknown global prevalence and incidence. This disease was first
described by three physicians, Cornelius G. Dyke, Leo M. Davidoff and
Clement M. Masson in 1933.\textsuperscript{1} DDMS is a
clinico-radiological recognized entity characterized by typical
manifestations including drugs resistant epilepsy, intellectual
disability, hemiparesis, skull bone thickening associated with cerebral
hemiatrophy and hyperpneumatization of paranasal and frontal sinuses on
neuroimaging.\textsuperscript{2} Some patients may present with
additional symptoms including cerebellar and basal ganglia atrophy, ear
malformations and neuropsychiatric disorders.\textsuperscript{3, 4}DDMS'
etiologies are broadly categorized into congenital and
acquired.\textsuperscript{5} Congenital causes of DDMS include
intrauterine vascular injury and cerebral hemispheric hypoperfusion and
acquired causes mostly derive from birth trauma, periventricular
leukomalacia, cerebral hemorrhage, cerebral infarction, cerebral
infection, radiation, postictal cerebral hemiatrophy, and prolonged
febrile seizures.\textsuperscript{6, 7} Almost a century after the first
description, the pathogenic mechanism remains unclear and is subject to
controversy. To date, less than 100 cases have been reported worldwide
with only four cases in the African population.\textsuperscript{8-11} We
report here the case of an 18-year-old adolescent who was admitted in
our neurology clinic for convulsive status epilepticus and in which
clinical and laboratory findings were in favor of DDMS, the first case
in Mali.

\textbf{Case report}

An 18-year-old male was admitted in our neurology clinic for recurring
tonic clonic seizures that started three days prior to his admission.
Medical history was consistent with a full-term infant, born from
healthy and non-consanguineous parents after an uneventful pregnancy.
However, delivery was done by caesarean section due to dystocia. Family
history was unremarkable. He had mental and motor acquisition delay
because he could not seat after one year of age and walked after the age
of two. Parents reported that he presented focal tonic clonic seizures
in the right half of his body at one year of age, at least once a day
that worsened over the time to more than ten episodes daily. He was
first treated with Valproic Acid, Phenobarbital and Carbamazepine at
different times which could poorly control the seizures, despite having
received the correct doses. He also presented frequent falls and
injuries due to seizures. Later, they noticed that the right side of his
body was becoming smaller and weaker than the left one. In addition, he
presented a progressive intellectual disability that led him to drop out
of school at the age of nine. On examination, the body temperature,
blood pressure, heart and breathing rates, and the oxygen saturation
were normal. Neurological examination found status epilepticus with
right-sided focal tonic clonic seizures followed by secondary
generalization. He had severe cognitive decline and couldn't count from
one to ten. Mini Mental State Examination (MMSE) was not possible. Body
asymmetry and hemiparesis was confirmed in addition to ankylosis in the
right wrist and elbow and a prominent forehead (Figure 1A\&B\&C).
Abdominal, cardiovascular, and pulmonary examination were unremarkable.
He did not present any cutaneous lesions other than scars caused by
seizure-related injuries. Blood chemistries including total blood cell
count, liver enzymes, serum creatinine and blood ions were normal. EEG
performed two weeks after admission found a diffuse slow background with
delta wave activities in hyperpnea and a continued focal to diffuse
pseudo periodic activities (Figure 2A\&B). Brain CT-scan revealed a
pronounced atrophy of left cerebral hemisphere with \emph{ex vacuo} of
the ipsilateral ventricle. Furthermore, a bilateral abnormal thickening
of the skull bone with an hyperpneumatization of the frontal sinuses
were seen (Figure 2C\&D). His clinical and imaging features were
consistent with DDMS. He was initially put on intravenous Levetiracetam
(1500mg/day), and continuous infusion of Clonazepam (2mg/day). The
disease course remarkably changed to fewer seizures (down to 10 a day).
Then, we added oral Valproic Acid (500mg twice a day) that drastically
improved the seizures with a maximum of two seizures daily and the
patient was discharged on this treatment. After the discharge, parents
reported that he presented three episodes of seizures in one month.

\textbf{Discussion}

Dyke, Davidoff and Masson recognized and reported the first cases of
this syndrome in 1933.\textsuperscript{1} Almost a century later, less
than 100 cases reported worldwide but its global incidence is still
unknown. DDMS falls in the category of epilepsy with brain structural
abnormalities of the International League Against Epilepsy
classification (ILAE).\textsuperscript{12} The clinical manifestations
encompass a focal and/or generalized drugs-resistant epilepsy,
hemiparesis or hemiplegia, facial or body asymmetry with atrophy and
mental retardation as seen in our patient.\textsuperscript{2, 3} Besides
these manifestations, rare cases may include cerebellar atrophy,
neuropsychiatric disorders and ear
malformations.\textsuperscript{3}However, the disease phenotype can vary
from one patient to another, and some may not present epilepsy, mental
retardation or body asymmetry.\textsuperscript{13} The patient was
referred for convulsive status epilepticus that led to the diagnosis as
seen in previous reports.\textsuperscript{14} DDMS is usually diagnosed
in childhood, mostly in the first decade, but cases of late diagnosis or
adults cases were also reported.\textsuperscript{2} Although the
syndrome is easily recognizable by clinical and brain imaging findings,
the diagnosis delay was eighteen years in our patient. This is likely
due to the limited access to specialists who could further investigate
with brain imaging as this was not performed before we saw the patient.
A similar case with a long diagnosis odyssey was reported in a patient
from Nigeria.\textsuperscript{11} Brain CT-scan or MRI have contributed
to facilitate the diagnosis by typically showing hemi cerebral atrophy,
ipsilateral thickening of the skull bone and ipsilateral hyper
pneumatization of the frontal and paranasal
sinuses.\textsuperscript{15}In our case, the hyper pneumatization of the
frontal and paranasal sinuses and the thickening of the skull bone were
bilateral. To the best of our knowledge, these brain imaging findings
have not been previously reported. This case could be another phenotypic
variant of DDMS that might be due to some specific genetic factors as
suggested by previous studies\textsuperscript{2} or to recurring brain.
Although our patient fulfilled the diagnosis criteria of DDMS, other
diseases such as Rasmussen's encephalitis (RE), hemiplegia-hemi
convulsion epilepsy (HHE) syndrome and Sturge Weber syndrome (SWS) are
also possible.\textsuperscript{15, 16} However, in RE and HHE there is
no skull bone thickening or a hyper pneumatization of the sinuses that
are pathognomonic radiological findings in DDMS as seen in the patient
presented here. In addition, in HHE syndrome, the seizures are in the
hemiplegia/hemiparesis side without secondary generalization. SWS is a
neurocutaneous syndrome characterized by facial birthmark also called
port-wine birthmark and cerebral vascular calcifications which were
absent in our patient. The management of DDMS is challenging, especially
in resource-limited settings due to the resistance to several
antiepileptic drugs (AED). The treatment is based on the use of a
combination of AEDs and the surgery with hemispherectomy is an option in
the case of refractory epilepsy.\textsuperscript{14}

\textbf{Conclusion:}

This is the first report of DDMS in a Malian patient. It highlights the
challenges for an early diagnosis and the treatment of this rare
condition. We suggest that in the presence of pharmaco-resistant
epilepsy in childhood and body deformities, further investigations
should be undertaken to establish evidence-based diagnosis for an
informed disease management strategy, particularly in low resources
setting where access to the surgery of epilepsy is highly limited or
unavailable.

\textbf{Author contributions}

\selectlanguage{ngerman}\textbf{Samba Ogomaly Djimdé} , data acquisition and
interpretation;\textbf{Abdoulaye Yalcouyé} , data analysis and
interpretation, and drafting the first version of the manuscript;
\textbf{Abdou Koita} , clinical management and data acquisition;
\textbf{Hassana Samir} , clinical management and data acquisition;
\textbf{Pofinet Kebkiba} , clinical management and data acquisition;
\textbf{Awovi Chrystelle Gueli} , clinical management and data
acquisition; \textbf{Alassane Baneye Maïga} , revision of the
manuscript; \textbf{Adama Seydou Sissoko} , revision of the manuscript;
\textbf{Guida Landouré} , editing and supervising of the study.

\textbf{Ethical approval}

None

\textbf{Consent}

Informed consent was obtained form the parents including publishing
photographs

\textbf{Data availability statement:} All the data included in this
study is available from the corresponding author upon reasonable request

\textbf{Conflict of interest:} authors declare no conflict of interest.

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\textbf{Figure legends}

\textbf{Figure 1:} Clinical findings in patient with
Duke-Davidoff-Masson syndrome. A): Images of the patient showing right
side hemi atrophy of the body more pronounced in upper limb. B,C):
ankylosis of the right wrist and elbow.

\textbf{Figure 2:} Laboratory findings in patient with
Duke-Davidoff-Masson syndrome. A,B): EEG showing slowed background with
delta wave activities in hyperpnea and a continued focal to diffuses
pseudo periodic activities (red squares). C,D): Brain CT-Scan in axial
cut showing a left hemisphere atrophy (blue arrow), bilateral calvarial
thickening (black arrow) and hyperpneumatization of the frontal sinuses
(red arrow).\selectlanguage{english}
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\includegraphics[width=0.70\columnwidth]{figures/Figure-2/Figure-2}

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